BACKGROUND Post-transplant lymphoproliferative disorder(PTLD)is a rare but highly fatal complication occurring after allogeneic hematopoietic cell transplantation(allo-HCT)or solid organ transplantation(SOT).Unlike SO...BACKGROUND Post-transplant lymphoproliferative disorder(PTLD)is a rare but highly fatal complication occurring after allogeneic hematopoietic cell transplantation(allo-HCT)or solid organ transplantation(SOT).Unlike SOT,PTLD after allo-HCT usually originates from the donor and is rarely accompanied by a loss of donor chimerism.CASE SUMMARY We report a case of Epstein-Barr virus positive PTLD manifesting as diffuse large B-cell lymphoma(DLBCL)with significantly decreased T-cell chimerism early after allo-HCT.A 30-year-old patient with acute myeloid leukemia underwent unrelated allo-HCT after first complete remission.Nearly 3 mo after transplantation,the patient developed cervical lymph node enlargement and gastric lesions,both of which were pathologically suggestive of DLBCL.Meanwhile,the patient experienced a significant and persistent decrease in T-cell chimerism.A partial remission was achieved after chemotherapy with single agent rituximab and subsequent R-CHOP combined chemotherapy.CONCLUSION The loss of T-cell chimerism and the concomitant T-cell insufficiency may be the cause of PTLD in this patient.展开更多
Transplant recipients are vulnerable to a higher risk of malignancy after solid organ transplantation and allogeneic hematopoietic stem-cell transplant.Posttransplant lymphoproliferative disorders(PTLD)include a wide ...Transplant recipients are vulnerable to a higher risk of malignancy after solid organ transplantation and allogeneic hematopoietic stem-cell transplant.Posttransplant lymphoproliferative disorders(PTLD)include a wide spectrum of diseases ranging from benign proliferation of lymphoid tissues to frank malignancy with aggressive behavior.Two main risk factors of PTLD are:Firstly,the cumulative immunosuppressive burden,and secondly,the oncogenic impact of the Epstein-Barr virus.The latter is a key pathognomonic driver of PTLD evolution.Over the last two decades,a considerable progress has been made in diagnosis and therapy of PTLD.The treatment of PTLD includes reduction of immunosuppression,rituximab therapy,either isolated or in combination with other chemotherapeutic agents,adoptive therapy,surgical intervention,antiviral therapy and radiotherapy.In this review we shall discuss the prevalence,clinical clues,prophylactic measures as well as the current and future therapeutic strategies of this devastating disorder.展开更多
To investigate incidence and survival of post-transplant lymphoproliferative disorder (PTLD) patients after liver transplantation.METHODSA cross-sectional survey was conducted among patients who underwent liver transp...To investigate incidence and survival of post-transplant lymphoproliferative disorder (PTLD) patients after liver transplantation.METHODSA cross-sectional survey was conducted among patients who underwent liver transplantation at Shiraz Transplant Center (Shiraz, Iran) between August 2004 and March 2015. Clinical and laboratory data of patients were collected using a data gathering form.RESULTSThere were 40 cases of PTLD in the pediatric age group and 13 cases in the adult group. The incidence of PTLD was 6.25% in pediatric patients and 1.18% in adult liver transplant recipients. The post-PTLD survival of patients at 6 mo was 75.1% ± 6%, at 1 year was 68.9% ± 6.5% and at 5 years was 39.2% ± 14.2%. Higher serum tacrolimus level was associated with lower post-PTLD survival in pediatric patients (OR = 1.07, 95%CI: 1.006-1.15, P = 0.032). A serum tacrolimus level over 11.1 ng/mL was predictive of post PTLD survival (sensitivity = 90%, specificity = 52%, area under the curve = 0.738, P = 0.035).CONCLUSIONIncidence of PTLD in our liver transplant patients is comparable to other centers. Transplant physicians may consider adjustment of tacrolimus dose to maintain its serum level below this cutoff point.展开更多
Background:Post-transplant lymphoproliferative disorder(PTLD)is a lethal complication after pediatric liver transplantation,but information regarding risk factors for the development of PTLD remains unclear.This study...Background:Post-transplant lymphoproliferative disorder(PTLD)is a lethal complication after pediatric liver transplantation,but information regarding risk factors for the development of PTLD remains unclear.This study was to identify characteristics and risk factors of PTLD.Methods:A total of 705 pediatric patients who underwent liver transplantation between January 2017 and October 2018 were studied.Impact of clinical characteristics and Epstein-Barr virus(EBV)infection on the development of PTLD was evaluated.In addition,ImmuKnow assay was adopted in partial patients to analyze the immune status.Results:Twenty-five(3.5%)patients suffered from PLTD with a median time of 6 months(3–14 months)after transplantation.Extremely high tacrolimus(TAC)level was found in 2 fatal cases at PTLD onset.EBV infection was found in 468(66.4%)patients.A higher peak EBV DNA loads(>9590 copies/mL)within 3 months was a significant indicator for the onset of PTLD.In addition,the ImmuKnow assay demonstrated that overall immune response was significantly lower in patients with EBV infection and PTLD(P<0.0001).The cumulative incidence of PTLD was also higher in patients with lower ATP value(≤187 ng/mL,P<0.05).Conclusions:A careful monitoring of EBV DNA loads and tacrolimus concentration might be supportive in prevention of PTLD in pediatric patients after liver transplantation.In addition,application of the ImmuKnow assay may provide guidance in reducing immunosuppressive agents in treatment of PTLD.展开更多
Hepatitis C virus （HCV） is a global health problem affecting 3% of the world＇s population （about 180 million） and a cause of both hepatic and extrahepatic diseases. B-cell lymphoproUferative disorders, whose prot...Hepatitis C virus （HCV） is a global health problem affecting 3% of the world＇s population （about 180 million） and a cause of both hepatic and extrahepatic diseases. B-cell lymphoproUferative disorders, whose prototype is mixed cryoglobulinemia, represent the most closely related as well as the most investigated HCV- related extrahepatic disorder. The association between extrahepatic （lymphoma） as well as hepatic malignancies （hepatocellular carcinoma） has justified the inclusion of HCV among human cancer viruses. HCV-associated manifestations also include porphyria cutanea tarda, lichen planus, nephropathies, thyreopathies, sicca syndrome, idiopathic pulmonary fibrosis, diabetes, chronic polyarthritis, sexual dysfunctions, cardiopathy/ atherosclerosis, and psychopathological disorders. A pathogenetic link between HCV virus and some lymphoproliferative disorders was confirmed by their responsiveness to antiviral therapy, which is now considered the first choice treatment. The aim of the present paper is to provide an overview of extrahepatic manifestations of HCV infection with particular attention to B-cell lymphoproliferative disorders. Available pathogenetic hypotheses and suggestions about the most appropriate, currently available, therapeutic approaches will also be discussed.展开更多
Post-transplant lymphoproliferative disorder(PTLD) is an aggressive complication of solid organ and hematopoietic stem cell transplantation that arises in up to 20% of transplant recipients. Infection or reactivation ...Post-transplant lymphoproliferative disorder(PTLD) is an aggressive complication of solid organ and hematopoietic stem cell transplantation that arises in up to 20% of transplant recipients. Infection or reactivation of the Epstein-Barr virus(EBV), a ubiquitous human herpesvirus, in combination with chronic immunosuppression are considered as the main predisposing factors, however insight in PTLD biology is fragmentary. The study of PTLD is complicated by its morphological heterogeneity and the lack of prospective trials, which also impede treatment optimization. Furthermore, the broad spectrum of underlying disorders and the graft type represent important confounding factors. PTLD encompasses different malignant subtypes that resemble histologically similar lymphomas in the general population. Post-transplant diffuse large B-cell lymphoma(PT-DLBCL), Burkitt lymphoma(PTBL) and plasmablastic lymphoma(PT-PBL) occur most frequently. However, in many studies various EBV+ and EBV- PTLD subtypes are pooled, complicating the interpretation of the results. In this review, studies of the gene expression pattern, the microenvironment and the genetic profile of PT-DLBCL, PT-BL and PT-PBL are summarized to better understand the mechanisms underlying post-transplantation lymphomagenesis. Based on the available findings we propose stratification of PTLD according to the histological subtype and the EBV status to facilitate the interpretation of future studies and the establishment of clinical trials.展开更多
AIM： To explore the role of IgG4 serology in identifying common orbital lymphoproliferative disorders, METHODS： Eighty -one patients with orbital lymphoproliferative diseases were treated in the Department of Ocular...AIM： To explore the role of IgG4 serology in identifying common orbital lymphoproliferative disorders, METHODS： Eighty -one patients with orbital lymphoproliferative diseases were treated in the Department of Ocular Oncology, Beijing Tongren Hospital, Capital Medical University between September 2010 and December 2012. Serum IgG4 levels were measured in 46 cases of idiopathic orbital inflammatory pseudotumor （IOIP）, 17 benign lymphoepithelial lesion （BLEL）, 12 cases of orbital mucosa-associated lymphoid tissue （MALT）, and 6 cases of diffuse large B-cell lymphoma （DLBL） using immuno-scatter turbidmetry （ISTM）.RESULTS： The frequency of elevated IgG4 levels in patients with IOIP, BLEL, MALT, and DLBL was 30.43% （14/46）, 76.47% （13/17）, 8.33% （1/12）, and 0.00 （0/6）, respectively. Among the patients with elevated serum IgG4 levels, all IgG-IOIP patients were male, and 92.31% of the IgG4-BLEL patients were female （12/13）. The mean serum IgG4 level of IgG4-1OIP patients was lower than that of individuals with IgG4-BLEL, but the variation in serum IgG4 levels was larger in IgG4-1OIP than IgG4- BLEL patients. Only one case of IgG4-MALT with elevated serum IgG4 levels had a medical history 〉10y, which was significantly longer than the MALT patients with normal serum IgG4 levels. There was no significant elevation of serum IgG4 levels in patients with DLBL. CONCLUSION： Detecting serum IgG4 levels plays an important role in the differential diagnosis of orbital lymphoproliferative diseases.展开更多
Epstein-Barr virus(EBV)T/NK-cell lymphoproliferative diseases are characterized by clonal expansion of EBV-infected T or NK cells,including chronic active EBV infection of T/NK-cell type(CAEBVT/NK),EBV-associated hemo...Epstein-Barr virus(EBV)T/NK-cell lymphoproliferative diseases are characterized by clonal expansion of EBV-infected T or NK cells,including chronic active EBV infection of T/NK-cell type(CAEBVT/NK),EBV-associated hemophagocytic lymphohistiocytosis(EBV HLH),extranodal NK/T-cell lymphoma of nasal type(ENKTL),and aggressive NK-cell leukemia(ANKL).However,the role of inherited genetic variants to EBV+T/NK-LPDs susceptibility is still unknown.A total of 171 nonimmunosuppressed patients with EBV T/NK-LPDs and 104 healthy donors were retrospectively collected and a targeted sequencing study covering 15 genes associated with lymphocyte cytotoxicity was performed.The 94 gene variants,mostly located in UNCI 3D,LYST,ITK,and PRF1 genes were detected,and mutations covered 28/50(56.00%)of CAEBV-T/NK,31/51(60.78%)of EBV HLH,13/28(46.42%)of ENKTL,and 13/48(27.09%)of ANKL.Most mutations represented monoallelic and missense.Three-year overall survival rate of patients with CAEBV-T/NK and EBV+HLH was significantly lower in patients with germline mutations than in those without germline mutations(P=0.0284,P=0.0137).Our study provided novel insights into understanding a spectrum of nonimmunosuppressed EBV*T/NK-LPDs with respect to genetic defects associated with lymphocyte cytotoxicity and reminded us that the gene sequencing may be an auxiliary test for diagnosis and risk stratification of EBV+T/NK-LPDs.展开更多
AIM To examine the risk of late-onset post-transplant lymphoproliferative disorder(PTLD) in the presence of persisting high Epstein-Barr virus(EBV) in EBV na?ve pediatric heart transplant(HT) recipients. METHODS A ret...AIM To examine the risk of late-onset post-transplant lymphoproliferative disorder(PTLD) in the presence of persisting high Epstein-Barr virus(EBV) in EBV na?ve pediatric heart transplant(HT) recipients. METHODS A retrospective review of the medical records of the 145 pediatric HT recipients who had serial EBV viral load monitoring at our center was performed. We defined EBV naive patients whose EBV serology either IgM or IgG in the blood were negative at the time of HT and excluded passive transmission from mother to child in subjects less than 6 mo of age. RESULTS PTLD was diagnosed in 8 out of 145 patients(5.5%); 6/91(6.5%) in those who were EBV seropositive and 2/54(3.7%) in the EBV na?ve group at the time of HT(P = 0.71). We found 32/145(22%) patients with persistently high EBV load during continuing follow-up; 20/91(22%) in EBV seropositive group vs 12/54(22%) in EBV na?ve group(P = 0.97). There was no significant association between pre-HT serostatus and EBV load after transplant(P > 0.05). In the EBV seropositive group, PTLD was diagnosed in 15%(3/20) of patients with high EBV vs 4.2%(3/71) of patients with low or undetectable EBV load(P = 0.14) whereas in EBV na?ve patients 8.3%(1/12) of those withhigh EBV load and 2.3%(1/42) with low or undetectable EBV load(P = 0.41). There was a highly significant association between occurrence of PTLD in those with high EBV load and duration of follow up(4.3 ± 3.9 years) after HT by Cochran-Armitage test for the entire cohort(P = 0.005). At least one episode of acute rejection occurred in 72%(23/32) of patients with high EBV vs 36%(41/113) patients with low or undetectable EBV after HT(P < 0.05). CONCLUSION There is an association between persistently high EBV load during post-HT follow up and the occurrence of late-onset PTLD in pediatric HT recipients irrespective of serostatus at the time of transplant. The occurrence of allograft rejection increased in patients with high EBV load presumably due to reduction in immunosuppression.展开更多
BACKGROUND Post-transplant lymphoproliferative disorder(PTLD) is a fatal complication of solid organ transplantation or allogenic hematopoietic stem cell transplantation that is associated with immunosuppressive thera...BACKGROUND Post-transplant lymphoproliferative disorder(PTLD) is a fatal complication of solid organ transplantation or allogenic hematopoietic stem cell transplantation that is associated with immunosuppressive therapy. Potential manifestations are diverse, ranging from reactive lymphoid hyperplasia to high-grade lymphoma.PTLD is usually of B-cell origin and associated with Epstein-Barr virus(EBV)infection. Herein, we describe a case of PTLD involving the peritoneal omentum.There has been only case of PTLD as a diffuse large B-cell lymphoma(DLBCL) in the peritoneum.CASE SUMMARY The patient was a 62-year-old man who had been receiving immunosuppressive therapy with tacrolimus since undergoing a liver transplant 15 years prior. He reported that he had experienced abdominal discomfort and anorexia 1 month prior to the current admission. Abdominal pelvic computed tomography(CT)revealed peritoneal and omental mass-like lesions without bowel obstruction.Ultrasonography-guided biopsy was performed, and he was histologically diagnosed with EBV-negative DLBCL. Positron emission tomography(PET)-CT depicted peritoneum and omentum involvement only, without any lymphadenopathy or organ masses, including in the gastrointestinal tract. Six cycles of chemotherapy with a "R-CHOP" regimen(rituximab-cyclophosphamide, doxorubicin, vincristine, prednisolone) were administered,and PET-CT performed thereafter indicated complete remission.CONCLUSION This is the first report of isolated peritoneal lymphomatosis defined as PTLD in a liver transplant recipient.展开更多
BACKGROUND Post-transplant lymphoproliferative disorder(PTLD) is a rare severe complication after renal transplantation, with an incidence of approximately 0.3%-2.0% in patients undergoing renal transplantation. The c...BACKGROUND Post-transplant lymphoproliferative disorder(PTLD) is a rare severe complication after renal transplantation, with an incidence of approximately 0.3%-2.0% in patients undergoing renal transplantation. The clinical manifestations of PTLD are often nonspecific, leading to tremendous challenges in the clinical diagnosis and treatment of PTLD.CASE SUMMARY We report two Epstein-Barr virus(EBV)-positive PTLD cases whose main clinical manifestations were digestive tract symptoms. Both of them admitted to our hospital because of extranodal infiltration symptoms and we did not suspect of PTLD until the pathology confirmation. Luckily, they responded well to the treatment of rituximab. We also discuss the virological monitoring, clinical characteristics, diagnosis, and treatment of PTLD.CONCLUSION PTLD is a deceptive disease and difficult to diagnose. Once patients are confirmed with PTLD, immune suppressant dosage should be immediately reduced and rituximab should be used as first-line therapy.展开更多
BACKGROUND Patients with a history of solid organ transplantation(SOT)or hematopoietic stem cell transplantation(HSCT)are at an increased risk of developing post-transplant lymphoproliferative disorder(PTLD).The gastr...BACKGROUND Patients with a history of solid organ transplantation(SOT)or hematopoietic stem cell transplantation(HSCT)are at an increased risk of developing post-transplant lymphoproliferative disorder(PTLD).The gastrointestinal(GI)tract is commonly affected as it has an abundance of B and T cells.AIM To determine typical GI-manifestations,risk factors for developing PTLD,and management.METHODS Major databases were searched until November 2021.RESULTS Non-case report studies that described GI manifestations of PTLD,risk factors for developing PTLD,and management of PTLD were included.Nine articles written within the last 20 years were included in the review.All articles found that patients with a history of SOT,regardless of transplanted organ,have a propensity to develop GI-PTLD.CONCLUSION GI tract manifestations may be nonspecific;therefore,consideration of risk factors is crucial for identifying GI-PTLD.Like other lymphoma variants,PTLD is very aggressive making early diagnosis key to prognosis.Initial treatment is reduction of immunosuppression which is effective in more than 50%of cases;however,additional therapy including rituximab,chemotherapy,and surgery may also be required.展开更多
Pretherapy sperm cryopreservation in young men is currently included in good clinical practice guidelines for cancer patients. The aim of this paper is to outline the effects of different oncological treatments on sem...Pretherapy sperm cryopreservation in young men is currently included in good clinical practice guidelines for cancer patients. The aim of this paper is to outline the effects of different oncological treatments on semen quality in patients with testicular neoplasia or lymphoprol iferative disorders, based on an 8-year experience of the Cryopreservation Centre of a large public hospital. Two hundred and sixty-one patients with testicular neoplasia and 219 patients with lymphoproliferative disorders who underwent chemotherapy and/or radiotherapy and pretherapy semen cryopreservation were evaluated. Sperm and hormonal parameters （follicle-stimulating hormone （FSH）, luteinizing hormone （LH）, testosterone, inhibin B levels） were assessed prior to and 6, 12, 18, 24 and 36 months after the end of cancer treatment. At the time of sperm collection, baseline FSH level and sperm concentration were impaired to a greater extent in patients with malignant testicular neoplasias than in patients with lymphoproliferative disorders. Toxic effects on spermatogenesis were still evident at 6 and 12 months after the end of cancer therapies, while an improvement of seminal parameters was observed after 18 months. In conclusion, an overall increase in sperm concentration was recorded about 18 months after the end of cancer treatments in the majority of patients, even if it was not possible to predict the evolution of each single case ＇a priori＇. For this reason, pretherapy semen cryopreservation should be considered in all young cancer patients.展开更多
BACKGROUND Post-transplant lymphoproliferative disease(PTLD)is a heterogeneous group of diseases that develop after solid organ and hematopoietic stem cells transplantation related to intensive immunosuppression regim...BACKGROUND Post-transplant lymphoproliferative disease(PTLD)is a heterogeneous group of diseases that develop after solid organ and hematopoietic stem cells transplantation related to intensive immunosuppression regimen,T-cell depletion and Epstein-Barr virus infection.Despite the improvement in the management of PTLD,the prognosis remains poor.Here we report the management of two transplanted patients with PTLD and infections during immunochemotherapy(ICTH).CASE SUMMARY Of 65-year-old woman 11 years after kidney transplantation(first case)presented with diffuse large B-cell lymphoma(DLBCL)CS III and started ICHT according to R-CHOP protocol.Despite the secondary prevention of neutropenic fever,the patient developed grade 4 neutropenia with urinary and pulmonary tract infections after the fifth cycle.ICTH was continued in reduced doses up to 7 cycles followed by involved-field radiation therapy of the residual disease.The second case presents a 49-year-old man,8 years after liver transplantation due to cirrhosis in the course of chronic hepatitis B,who started ICTH for DLBCL Burkitt-like CS IV.The patient received four cycles of ICTH according to RCODOX/R-IVAC protocol,with reduced doses.In both cases initially undertaken reduction of immunosuppression was ineffective to prevent infectious complications.Despite one incomplete ICHT treatment due to recurrent infections,both our patients remain in complete remission.CONCLUSION Reduction of immunosuppression and the doses of chemotherapeutics may be insufficient to prevent infectious complications during ICTH in PTLD patients.展开更多
BACKGROUND: Posttransplantation lymphoproliferative disorder (PTLD) involving the central nervous system (CNS) is a rare and serious complication associated with solid organ transplantation. We treated a case of PTLD ...BACKGROUND: Posttransplantation lymphoproliferative disorder (PTLD) involving the central nervous system (CNS) is a rare and serious complication associated with solid organ transplantation. We treated a case of PTLD with CNS involvement in a liver transplant recipient and reviewed the literature. METHOD: The clinicopathological features of a 53-year-old man were retrospectively analyzed. RESULTS: Metastasis of the hepatoma was preoperatively considered on the basis of clinical findings. Craniotomy was performed and PTLD was diagnosed pathologically. The patient was treated with antiviral agents, radiation therapy, and chemotherapy; the immunosuppressive medication was reduced. The patient is still alive after follow-up for 14 months. CONCLUSIONS: Definitive diagnosis of PTLD is only established on the basis of histopathologic evaluation of the tissue. Although there are several ways to manage PTLD with CNS involvement, the prognosis is still poor.展开更多
To identify the knowledge of rare lymphoproliferative disorder, the clinical and biological features of three kinds of lymphoproliferative disorders with cytoplasmic projections were compared The clinical manifestat...To identify the knowledge of rare lymphoproliferative disorder, the clinical and biological features of three kinds of lymphoproliferative disorders with cytoplasmic projections were compared The clinical manifestations, ultrastructure and immunophenotype were analyzed The results showed that hairy cell leukemia (HCL), splenic lymphoma with villous lymphocyte (SLVL) and hairy cell leukemia-variant (HCL-V) had some common characters including splenomegaly, peripheral blood and bone marrow infiltration by villous lymphocyte and B lymphocyte immunophenotype; but these three disorders had specific features respectively It was concluded that overall analysis of clinical and laboratory features might be contributive to the differential diagnosis of these three disorders展开更多
BACKGROUND Kaposi sarcoma and post-transplant lymphoproliferative disorder have been occasionally reported in post-liver transplant patients.However,the simultaneous occurrence of these two diseases in the same lymph ...BACKGROUND Kaposi sarcoma and post-transplant lymphoproliferative disorder have been occasionally reported in post-liver transplant patients.However,the simultaneous occurrence of these two diseases in the same lymph nodes is very rare.CASE SUMMARY We report the case of a 19-mo-old boy,who presented with intermittent fever and enlarged cervical lymph nodes after liver transplantation.Six cervical lymph nodes were biopsied,and the histopathological examinations revealed multifocal hyperplasia of spindle cells around small blood vessels,extravasated erythrocytes,and heavy infiltration of plasma cells in the cortex and medulla of the lymph nodes.The immunohistochemical analyses of spindle cells revealed positive expression of CD34,CD31,erythroblast transformation-specific-related gene,friend leukemia integration 1,and human herpesvirus-8.The lymphoproliferative lesions expressed CD38,CD138,and multiple myeloma 1.Epstein-Barr encoded RNA in situ hybridization demonstrated Epstein-Barr virus-positive lymphoid cells.Finally,we diagnosed the coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder(plasmacytic hyperplasia)in the same lymph nodes.Treatment strategy included anti-CD20 monoclonal antibody(rituximab)and discontinuation of the immunosuppressant therapies.Lymph node biopsies during follow-up examinations revealed lymphoid hyperplasia.CONCLUSION The rare coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder in the same lymph nodes post-liver transplantation possibly associates with immunodeficiency and Epstein-Barr virus and human herpesvirus-8 coinfection.展开更多
BACKGROUND Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract(ITLPDGI),a primary tumor forming in the gastrointestinal(GI)tract,represents a rarely diagnosed clonal T-cell disease with a protra...BACKGROUND Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract(ITLPDGI),a primary tumor forming in the gastrointestinal(GI)tract,represents a rarely diagnosed clonal T-cell disease with a protracted clinical course.CASE SUMMARY This report presented a 45-year-old male patient with a 6-year history of anal fistula and a more than 10-year history of recurrent diarrhea who was not correctly diagnosed until the occurrence of complications such as intestinal perforation.Postsurgical histopathological analysis,combined with hematoxylineosin staining,immunohistochemistry and TCRβ/γ clonal gene rearrangement test,confirmed the diagnosis of CD8+ITLPD-GI.CONCLUSION Individuals with this scarce lymphoma frequently show non-specific symptoms that are hard to recognize.So far,indolent CD8+ITLPD-GI has not been comprehensively examined.The current mini-review focused on evaluating indolent CD8+ITLPD-GI cases based on existing literature and discussing future directions for improved differential diagnosis,detection of genetic and epigenetic alterations,and therapeutic target identification.展开更多
BACKGROUND Post-transplant lymphoproliferative disease(PTLD)is the most common malignant tumor that occurs after kidney transplantation in children,and is associated with Epstein-Barr virus(EBV).CASE SUMMARY We report...BACKGROUND Post-transplant lymphoproliferative disease(PTLD)is the most common malignant tumor that occurs after kidney transplantation in children,and is associated with Epstein-Barr virus(EBV).CASE SUMMARY We report a case of PTLD that occurred in a 17-year-old female patient at 5 mo post-transplant.The first symptom was abdominal pain accompanied by fever,nausea,and vomiting.EBV-associated monomorphic PTLD with multiple abdominal nodules was diagnosed by pathology,clinical manifestations,imaging results,and the presence of EB-DNA.After successful treatment with rituximab,the abdominal nodules in the spleen and liver disappeared.CONCLUSION Early pathological biopsy to confirm the diagnosis is critical to treatment and prognosis.Reducing immunosuppression and rituximab therapy are effective methods for treating PTLD,but need to be initiated as early as possible.展开更多
Lymph node aspirates of 17 cases with enlarged superficial or isceral lymph nodes were detected for immunoglobulin heavy chain gene rearrangement (IgHRA) and T cell rcceptor γ gene rearrangement (TCRγRA) bypolymeras...Lymph node aspirates of 17 cases with enlarged superficial or isceral lymph nodes were detected for immunoglobulin heavy chain gene rearrangement (IgHRA) and T cell rcceptor γ gene rearrangement (TCRγRA) bypolymerase chain reaction (PCR). Combining with clinical data, pathologic diagnosis and immunophenotapy, we analyse the results as follows: 5 cases with nonlymphoid cancers and 3 cases with reactive lymphadenopathy do not present two kinds of clone gene rearrangements.5 out of 7 cases with NHL show clone gene rearrangements (IgH 3 cases, TCRY 2cases),two kinds of monoclonal band(100-120bp for IgHRA and 170-230bp for TCRγRA) were observed after electrophoresis of amplified DNA products. One case whose clinical sitcaion accorded with features of lymphoma was diagnosed as granulomatous lymphadenitis by pathologist, after gene rearaangement clone TCRγRA was detected,a correct diaguosis as NHL was made then. The significance of detecting the two kinds of gene rearrangement for clinical application and the limitations in diagnosis of lymphoproliferative disorders was discussed.展开更多
文摘BACKGROUND Post-transplant lymphoproliferative disorder(PTLD)is a rare but highly fatal complication occurring after allogeneic hematopoietic cell transplantation(allo-HCT)or solid organ transplantation(SOT).Unlike SOT,PTLD after allo-HCT usually originates from the donor and is rarely accompanied by a loss of donor chimerism.CASE SUMMARY We report a case of Epstein-Barr virus positive PTLD manifesting as diffuse large B-cell lymphoma(DLBCL)with significantly decreased T-cell chimerism early after allo-HCT.A 30-year-old patient with acute myeloid leukemia underwent unrelated allo-HCT after first complete remission.Nearly 3 mo after transplantation,the patient developed cervical lymph node enlargement and gastric lesions,both of which were pathologically suggestive of DLBCL.Meanwhile,the patient experienced a significant and persistent decrease in T-cell chimerism.A partial remission was achieved after chemotherapy with single agent rituximab and subsequent R-CHOP combined chemotherapy.CONCLUSION The loss of T-cell chimerism and the concomitant T-cell insufficiency may be the cause of PTLD in this patient.
文摘Transplant recipients are vulnerable to a higher risk of malignancy after solid organ transplantation and allogeneic hematopoietic stem-cell transplant.Posttransplant lymphoproliferative disorders(PTLD)include a wide spectrum of diseases ranging from benign proliferation of lymphoid tissues to frank malignancy with aggressive behavior.Two main risk factors of PTLD are:Firstly,the cumulative immunosuppressive burden,and secondly,the oncogenic impact of the Epstein-Barr virus.The latter is a key pathognomonic driver of PTLD evolution.Over the last two decades,a considerable progress has been made in diagnosis and therapy of PTLD.The treatment of PTLD includes reduction of immunosuppression,rituximab therapy,either isolated or in combination with other chemotherapeutic agents,adoptive therapy,surgical intervention,antiviral therapy and radiotherapy.In this review we shall discuss the prevalence,clinical clues,prophylactic measures as well as the current and future therapeutic strategies of this devastating disorder.
文摘To investigate incidence and survival of post-transplant lymphoproliferative disorder (PTLD) patients after liver transplantation.METHODSA cross-sectional survey was conducted among patients who underwent liver transplantation at Shiraz Transplant Center (Shiraz, Iran) between August 2004 and March 2015. Clinical and laboratory data of patients were collected using a data gathering form.RESULTSThere were 40 cases of PTLD in the pediatric age group and 13 cases in the adult group. The incidence of PTLD was 6.25% in pediatric patients and 1.18% in adult liver transplant recipients. The post-PTLD survival of patients at 6 mo was 75.1% ± 6%, at 1 year was 68.9% ± 6.5% and at 5 years was 39.2% ± 14.2%. Higher serum tacrolimus level was associated with lower post-PTLD survival in pediatric patients (OR = 1.07, 95%CI: 1.006-1.15, P = 0.032). A serum tacrolimus level over 11.1 ng/mL was predictive of post PTLD survival (sensitivity = 90%, specificity = 52%, area under the curve = 0.738, P = 0.035).CONCLUSIONIncidence of PTLD in our liver transplant patients is comparable to other centers. Transplant physicians may consider adjustment of tacrolimus dose to maintain its serum level below this cutoff point.
基金supported by grants from Shanghai Munici-pal Hospital Three-year-Project for Clinical Skills’ Promotion and Innovation(16CR1003A)Shanghai Jiaotong University School of Medicine(DLY201606)National Natural Science Foundation of China(81670602)
文摘Background:Post-transplant lymphoproliferative disorder(PTLD)is a lethal complication after pediatric liver transplantation,but information regarding risk factors for the development of PTLD remains unclear.This study was to identify characteristics and risk factors of PTLD.Methods:A total of 705 pediatric patients who underwent liver transplantation between January 2017 and October 2018 were studied.Impact of clinical characteristics and Epstein-Barr virus(EBV)infection on the development of PTLD was evaluated.In addition,ImmuKnow assay was adopted in partial patients to analyze the immune status.Results:Twenty-five(3.5%)patients suffered from PLTD with a median time of 6 months(3–14 months)after transplantation.Extremely high tacrolimus(TAC)level was found in 2 fatal cases at PTLD onset.EBV infection was found in 468(66.4%)patients.A higher peak EBV DNA loads(>9590 copies/mL)within 3 months was a significant indicator for the onset of PTLD.In addition,the ImmuKnow assay demonstrated that overall immune response was significantly lower in patients with EBV infection and PTLD(P<0.0001).The cumulative incidence of PTLD was also higher in patients with lower ATP value(≤187 ng/mL,P<0.05).Conclusions:A careful monitoring of EBV DNA loads and tacrolimus concentration might be supportive in prevention of PTLD in pediatric patients after liver transplantation.In addition,application of the ImmuKnow assay may provide guidance in reducing immunosuppressive agents in treatment of PTLD.
文摘Hepatitis C virus （HCV） is a global health problem affecting 3% of the world＇s population （about 180 million） and a cause of both hepatic and extrahepatic diseases. B-cell lymphoproUferative disorders, whose prototype is mixed cryoglobulinemia, represent the most closely related as well as the most investigated HCV- related extrahepatic disorder. The association between extrahepatic （lymphoma） as well as hepatic malignancies （hepatocellular carcinoma） has justified the inclusion of HCV among human cancer viruses. HCV-associated manifestations also include porphyria cutanea tarda, lichen planus, nephropathies, thyreopathies, sicca syndrome, idiopathic pulmonary fibrosis, diabetes, chronic polyarthritis, sexual dysfunctions, cardiopathy/ atherosclerosis, and psychopathological disorders. A pathogenetic link between HCV virus and some lymphoproliferative disorders was confirmed by their responsiveness to antiviral therapy, which is now considered the first choice treatment. The aim of the present paper is to provide an overview of extrahepatic manifestations of HCV infection with particular attention to B-cell lymphoproliferative disorders. Available pathogenetic hypotheses and suggestions about the most appropriate, currently available, therapeutic approaches will also be discussed.
文摘Post-transplant lymphoproliferative disorder(PTLD) is an aggressive complication of solid organ and hematopoietic stem cell transplantation that arises in up to 20% of transplant recipients. Infection or reactivation of the Epstein-Barr virus(EBV), a ubiquitous human herpesvirus, in combination with chronic immunosuppression are considered as the main predisposing factors, however insight in PTLD biology is fragmentary. The study of PTLD is complicated by its morphological heterogeneity and the lack of prospective trials, which also impede treatment optimization. Furthermore, the broad spectrum of underlying disorders and the graft type represent important confounding factors. PTLD encompasses different malignant subtypes that resemble histologically similar lymphomas in the general population. Post-transplant diffuse large B-cell lymphoma(PT-DLBCL), Burkitt lymphoma(PTBL) and plasmablastic lymphoma(PT-PBL) occur most frequently. However, in many studies various EBV+ and EBV- PTLD subtypes are pooled, complicating the interpretation of the results. In this review, studies of the gene expression pattern, the microenvironment and the genetic profile of PT-DLBCL, PT-BL and PT-PBL are summarized to better understand the mechanisms underlying post-transplantation lymphomagenesis. Based on the available findings we propose stratification of PTLD according to the histological subtype and the EBV status to facilitate the interpretation of future studies and the establishment of clinical trials.
基金Supported by National Natural Science Foundation of China (No.81170875 No.81371052)
文摘AIM： To explore the role of IgG4 serology in identifying common orbital lymphoproliferative disorders, METHODS： Eighty -one patients with orbital lymphoproliferative diseases were treated in the Department of Ocular Oncology, Beijing Tongren Hospital, Capital Medical University between September 2010 and December 2012. Serum IgG4 levels were measured in 46 cases of idiopathic orbital inflammatory pseudotumor （IOIP）, 17 benign lymphoepithelial lesion （BLEL）, 12 cases of orbital mucosa-associated lymphoid tissue （MALT）, and 6 cases of diffuse large B-cell lymphoma （DLBL） using immuno-scatter turbidmetry （ISTM）.RESULTS： The frequency of elevated IgG4 levels in patients with IOIP, BLEL, MALT, and DLBL was 30.43% （14/46）, 76.47% （13/17）, 8.33% （1/12）, and 0.00 （0/6）, respectively. Among the patients with elevated serum IgG4 levels, all IgG-IOIP patients were male, and 92.31% of the IgG4-BLEL patients were female （12/13）. The mean serum IgG4 level of IgG4-1OIP patients was lower than that of individuals with IgG4-BLEL, but the variation in serum IgG4 levels was larger in IgG4-1OIP than IgG4- BLEL patients. Only one case of IgG4-MALT with elevated serum IgG4 levels had a medical history 〉10y, which was significantly longer than the MALT patients with normal serum IgG4 levels. There was no significant elevation of serum IgG4 levels in patients with DLBL. CONCLUSION： Detecting serum IgG4 levels plays an important role in the differential diagnosis of orbital lymphoproliferative diseases.
基金the National Natural Science Foundation of China(No.81770211)。
文摘Epstein-Barr virus(EBV)T/NK-cell lymphoproliferative diseases are characterized by clonal expansion of EBV-infected T or NK cells,including chronic active EBV infection of T/NK-cell type(CAEBVT/NK),EBV-associated hemophagocytic lymphohistiocytosis(EBV HLH),extranodal NK/T-cell lymphoma of nasal type(ENKTL),and aggressive NK-cell leukemia(ANKL).However,the role of inherited genetic variants to EBV+T/NK-LPDs susceptibility is still unknown.A total of 171 nonimmunosuppressed patients with EBV T/NK-LPDs and 104 healthy donors were retrospectively collected and a targeted sequencing study covering 15 genes associated with lymphocyte cytotoxicity was performed.The 94 gene variants,mostly located in UNCI 3D,LYST,ITK,and PRF1 genes were detected,and mutations covered 28/50(56.00%)of CAEBV-T/NK,31/51(60.78%)of EBV HLH,13/28(46.42%)of ENKTL,and 13/48(27.09%)of ANKL.Most mutations represented monoallelic and missense.Three-year overall survival rate of patients with CAEBV-T/NK and EBV+HLH was significantly lower in patients with germline mutations than in those without germline mutations(P=0.0284,P=0.0137).Our study provided novel insights into understanding a spectrum of nonimmunosuppressed EBV*T/NK-LPDs with respect to genetic defects associated with lymphocyte cytotoxicity and reminded us that the gene sequencing may be an auxiliary test for diagnosis and risk stratification of EBV+T/NK-LPDs.
文摘AIM To examine the risk of late-onset post-transplant lymphoproliferative disorder(PTLD) in the presence of persisting high Epstein-Barr virus(EBV) in EBV na?ve pediatric heart transplant(HT) recipients. METHODS A retrospective review of the medical records of the 145 pediatric HT recipients who had serial EBV viral load monitoring at our center was performed. We defined EBV naive patients whose EBV serology either IgM or IgG in the blood were negative at the time of HT and excluded passive transmission from mother to child in subjects less than 6 mo of age. RESULTS PTLD was diagnosed in 8 out of 145 patients(5.5%); 6/91(6.5%) in those who were EBV seropositive and 2/54(3.7%) in the EBV na?ve group at the time of HT(P = 0.71). We found 32/145(22%) patients with persistently high EBV load during continuing follow-up; 20/91(22%) in EBV seropositive group vs 12/54(22%) in EBV na?ve group(P = 0.97). There was no significant association between pre-HT serostatus and EBV load after transplant(P > 0.05). In the EBV seropositive group, PTLD was diagnosed in 15%(3/20) of patients with high EBV vs 4.2%(3/71) of patients with low or undetectable EBV load(P = 0.14) whereas in EBV na?ve patients 8.3%(1/12) of those withhigh EBV load and 2.3%(1/42) with low or undetectable EBV load(P = 0.41). There was a highly significant association between occurrence of PTLD in those with high EBV load and duration of follow up(4.3 ± 3.9 years) after HT by Cochran-Armitage test for the entire cohort(P = 0.005). At least one episode of acute rejection occurred in 72%(23/32) of patients with high EBV vs 36%(41/113) patients with low or undetectable EBV after HT(P < 0.05). CONCLUSION There is an association between persistently high EBV load during post-HT follow up and the occurrence of late-onset PTLD in pediatric HT recipients irrespective of serostatus at the time of transplant. The occurrence of allograft rejection increased in patients with high EBV load presumably due to reduction in immunosuppression.
基金Supported by the National Research Foundation of Korea,No.NRF-2017R1C1B5076793
文摘BACKGROUND Post-transplant lymphoproliferative disorder(PTLD) is a fatal complication of solid organ transplantation or allogenic hematopoietic stem cell transplantation that is associated with immunosuppressive therapy. Potential manifestations are diverse, ranging from reactive lymphoid hyperplasia to high-grade lymphoma.PTLD is usually of B-cell origin and associated with Epstein-Barr virus(EBV)infection. Herein, we describe a case of PTLD involving the peritoneal omentum.There has been only case of PTLD as a diffuse large B-cell lymphoma(DLBCL) in the peritoneum.CASE SUMMARY The patient was a 62-year-old man who had been receiving immunosuppressive therapy with tacrolimus since undergoing a liver transplant 15 years prior. He reported that he had experienced abdominal discomfort and anorexia 1 month prior to the current admission. Abdominal pelvic computed tomography(CT)revealed peritoneal and omental mass-like lesions without bowel obstruction.Ultrasonography-guided biopsy was performed, and he was histologically diagnosed with EBV-negative DLBCL. Positron emission tomography(PET)-CT depicted peritoneum and omentum involvement only, without any lymphadenopathy or organ masses, including in the gastrointestinal tract. Six cycles of chemotherapy with a "R-CHOP" regimen(rituximab-cyclophosphamide, doxorubicin, vincristine, prednisolone) were administered,and PET-CT performed thereafter indicated complete remission.CONCLUSION This is the first report of isolated peritoneal lymphomatosis defined as PTLD in a liver transplant recipient.
基金Supported by the National Natural Science Foundation of China,No.81771720
文摘BACKGROUND Post-transplant lymphoproliferative disorder(PTLD) is a rare severe complication after renal transplantation, with an incidence of approximately 0.3%-2.0% in patients undergoing renal transplantation. The clinical manifestations of PTLD are often nonspecific, leading to tremendous challenges in the clinical diagnosis and treatment of PTLD.CASE SUMMARY We report two Epstein-Barr virus(EBV)-positive PTLD cases whose main clinical manifestations were digestive tract symptoms. Both of them admitted to our hospital because of extranodal infiltration symptoms and we did not suspect of PTLD until the pathology confirmation. Luckily, they responded well to the treatment of rituximab. We also discuss the virological monitoring, clinical characteristics, diagnosis, and treatment of PTLD.CONCLUSION PTLD is a deceptive disease and difficult to diagnose. Once patients are confirmed with PTLD, immune suppressant dosage should be immediately reduced and rituximab should be used as first-line therapy.
文摘BACKGROUND Patients with a history of solid organ transplantation(SOT)or hematopoietic stem cell transplantation(HSCT)are at an increased risk of developing post-transplant lymphoproliferative disorder(PTLD).The gastrointestinal(GI)tract is commonly affected as it has an abundance of B and T cells.AIM To determine typical GI-manifestations,risk factors for developing PTLD,and management.METHODS Major databases were searched until November 2021.RESULTS Non-case report studies that described GI manifestations of PTLD,risk factors for developing PTLD,and management of PTLD were included.Nine articles written within the last 20 years were included in the review.All articles found that patients with a history of SOT,regardless of transplanted organ,have a propensity to develop GI-PTLD.CONCLUSION GI tract manifestations may be nonspecific;therefore,consideration of risk factors is crucial for identifying GI-PTLD.Like other lymphoma variants,PTLD is very aggressive making early diagnosis key to prognosis.Initial treatment is reduction of immunosuppression which is effective in more than 50%of cases;however,additional therapy including rituximab,chemotherapy,and surgery may also be required.
文摘Pretherapy sperm cryopreservation in young men is currently included in good clinical practice guidelines for cancer patients. The aim of this paper is to outline the effects of different oncological treatments on semen quality in patients with testicular neoplasia or lymphoprol iferative disorders, based on an 8-year experience of the Cryopreservation Centre of a large public hospital. Two hundred and sixty-one patients with testicular neoplasia and 219 patients with lymphoproliferative disorders who underwent chemotherapy and/or radiotherapy and pretherapy semen cryopreservation were evaluated. Sperm and hormonal parameters （follicle-stimulating hormone （FSH）, luteinizing hormone （LH）, testosterone, inhibin B levels） were assessed prior to and 6, 12, 18, 24 and 36 months after the end of cancer treatment. At the time of sperm collection, baseline FSH level and sperm concentration were impaired to a greater extent in patients with malignant testicular neoplasias than in patients with lymphoproliferative disorders. Toxic effects on spermatogenesis were still evident at 6 and 12 months after the end of cancer therapies, while an improvement of seminal parameters was observed after 18 months. In conclusion, an overall increase in sperm concentration was recorded about 18 months after the end of cancer treatments in the majority of patients, even if it was not possible to predict the evolution of each single case ＇a priori＇. For this reason, pretherapy semen cryopreservation should be considered in all young cancer patients.
文摘BACKGROUND Post-transplant lymphoproliferative disease(PTLD)is a heterogeneous group of diseases that develop after solid organ and hematopoietic stem cells transplantation related to intensive immunosuppression regimen,T-cell depletion and Epstein-Barr virus infection.Despite the improvement in the management of PTLD,the prognosis remains poor.Here we report the management of two transplanted patients with PTLD and infections during immunochemotherapy(ICTH).CASE SUMMARY Of 65-year-old woman 11 years after kidney transplantation(first case)presented with diffuse large B-cell lymphoma(DLBCL)CS III and started ICHT according to R-CHOP protocol.Despite the secondary prevention of neutropenic fever,the patient developed grade 4 neutropenia with urinary and pulmonary tract infections after the fifth cycle.ICTH was continued in reduced doses up to 7 cycles followed by involved-field radiation therapy of the residual disease.The second case presents a 49-year-old man,8 years after liver transplantation due to cirrhosis in the course of chronic hepatitis B,who started ICTH for DLBCL Burkitt-like CS IV.The patient received four cycles of ICTH according to RCODOX/R-IVAC protocol,with reduced doses.In both cases initially undertaken reduction of immunosuppression was ineffective to prevent infectious complications.Despite one incomplete ICHT treatment due to recurrent infections,both our patients remain in complete remission.CONCLUSION Reduction of immunosuppression and the doses of chemotherapeutics may be insufficient to prevent infectious complications during ICTH in PTLD patients.
文摘BACKGROUND: Posttransplantation lymphoproliferative disorder (PTLD) involving the central nervous system (CNS) is a rare and serious complication associated with solid organ transplantation. We treated a case of PTLD with CNS involvement in a liver transplant recipient and reviewed the literature. METHOD: The clinicopathological features of a 53-year-old man were retrospectively analyzed. RESULTS: Metastasis of the hepatoma was preoperatively considered on the basis of clinical findings. Craniotomy was performed and PTLD was diagnosed pathologically. The patient was treated with antiviral agents, radiation therapy, and chemotherapy; the immunosuppressive medication was reduced. The patient is still alive after follow-up for 14 months. CONCLUSIONS: Definitive diagnosis of PTLD is only established on the basis of histopathologic evaluation of the tissue. Although there are several ways to manage PTLD with CNS involvement, the prognosis is still poor.
文摘To identify the knowledge of rare lymphoproliferative disorder, the clinical and biological features of three kinds of lymphoproliferative disorders with cytoplasmic projections were compared The clinical manifestations, ultrastructure and immunophenotype were analyzed The results showed that hairy cell leukemia (HCL), splenic lymphoma with villous lymphocyte (SLVL) and hairy cell leukemia-variant (HCL-V) had some common characters including splenomegaly, peripheral blood and bone marrow infiltration by villous lymphocyte and B lymphocyte immunophenotype; but these three disorders had specific features respectively It was concluded that overall analysis of clinical and laboratory features might be contributive to the differential diagnosis of these three disorders
基金Supported by the Digestive Medical Coordinated Development Center of Beijing Hospitals Authority,No.XXX0102。
文摘BACKGROUND Kaposi sarcoma and post-transplant lymphoproliferative disorder have been occasionally reported in post-liver transplant patients.However,the simultaneous occurrence of these two diseases in the same lymph nodes is very rare.CASE SUMMARY We report the case of a 19-mo-old boy,who presented with intermittent fever and enlarged cervical lymph nodes after liver transplantation.Six cervical lymph nodes were biopsied,and the histopathological examinations revealed multifocal hyperplasia of spindle cells around small blood vessels,extravasated erythrocytes,and heavy infiltration of plasma cells in the cortex and medulla of the lymph nodes.The immunohistochemical analyses of spindle cells revealed positive expression of CD34,CD31,erythroblast transformation-specific-related gene,friend leukemia integration 1,and human herpesvirus-8.The lymphoproliferative lesions expressed CD38,CD138,and multiple myeloma 1.Epstein-Barr encoded RNA in situ hybridization demonstrated Epstein-Barr virus-positive lymphoid cells.Finally,we diagnosed the coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder(plasmacytic hyperplasia)in the same lymph nodes.Treatment strategy included anti-CD20 monoclonal antibody(rituximab)and discontinuation of the immunosuppressant therapies.Lymph node biopsies during follow-up examinations revealed lymphoid hyperplasia.CONCLUSION The rare coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder in the same lymph nodes post-liver transplantation possibly associates with immunodeficiency and Epstein-Barr virus and human herpesvirus-8 coinfection.
文摘BACKGROUND Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract(ITLPDGI),a primary tumor forming in the gastrointestinal(GI)tract,represents a rarely diagnosed clonal T-cell disease with a protracted clinical course.CASE SUMMARY This report presented a 45-year-old male patient with a 6-year history of anal fistula and a more than 10-year history of recurrent diarrhea who was not correctly diagnosed until the occurrence of complications such as intestinal perforation.Postsurgical histopathological analysis,combined with hematoxylineosin staining,immunohistochemistry and TCRβ/γ clonal gene rearrangement test,confirmed the diagnosis of CD8+ITLPD-GI.CONCLUSION Individuals with this scarce lymphoma frequently show non-specific symptoms that are hard to recognize.So far,indolent CD8+ITLPD-GI has not been comprehensively examined.The current mini-review focused on evaluating indolent CD8+ITLPD-GI cases based on existing literature and discussing future directions for improved differential diagnosis,detection of genetic and epigenetic alterations,and therapeutic target identification.
文摘BACKGROUND Post-transplant lymphoproliferative disease(PTLD)is the most common malignant tumor that occurs after kidney transplantation in children,and is associated with Epstein-Barr virus(EBV).CASE SUMMARY We report a case of PTLD that occurred in a 17-year-old female patient at 5 mo post-transplant.The first symptom was abdominal pain accompanied by fever,nausea,and vomiting.EBV-associated monomorphic PTLD with multiple abdominal nodules was diagnosed by pathology,clinical manifestations,imaging results,and the presence of EB-DNA.After successful treatment with rituximab,the abdominal nodules in the spleen and liver disappeared.CONCLUSION Early pathological biopsy to confirm the diagnosis is critical to treatment and prognosis.Reducing immunosuppression and rituximab therapy are effective methods for treating PTLD,but need to be initiated as early as possible.
文摘Lymph node aspirates of 17 cases with enlarged superficial or isceral lymph nodes were detected for immunoglobulin heavy chain gene rearrangement (IgHRA) and T cell rcceptor γ gene rearrangement (TCRγRA) bypolymerase chain reaction (PCR). Combining with clinical data, pathologic diagnosis and immunophenotapy, we analyse the results as follows: 5 cases with nonlymphoid cancers and 3 cases with reactive lymphadenopathy do not present two kinds of clone gene rearrangements.5 out of 7 cases with NHL show clone gene rearrangements (IgH 3 cases, TCRY 2cases),two kinds of monoclonal band(100-120bp for IgHRA and 170-230bp for TCRγRA) were observed after electrophoresis of amplified DNA products. One case whose clinical sitcaion accorded with features of lymphoma was diagnosed as granulomatous lymphadenitis by pathologist, after gene rearaangement clone TCRγRA was detected,a correct diaguosis as NHL was made then. The significance of detecting the two kinds of gene rearrangement for clinical application and the limitations in diagnosis of lymphoproliferative disorders was discussed.
