BACKGROUND Cumulative evidence suggests that the aberrant immune responses in acquired aplastic anemia(AA) are sustained by active chronic infections in genetically susceptible individuals. Recently, the constant sour...BACKGROUND Cumulative evidence suggests that the aberrant immune responses in acquired aplastic anemia(AA) are sustained by active chronic infections in genetically susceptible individuals. Recently, the constant source to trigger and sustain the pathophysiology has been proposed to come from the altered gut microbiota and chronic intestinal inflammation. In this case, our serendipitous finding provides convincing evidence that the persistently dysregulated autoimmunity may be generated, at least in a significant proposition of AA patients, by the altered gut microbiota and compromised intestinal epithelium.CASE SUMMARY A 30-year-old Chinese male patient with refractory severe AA experienced a 3-month-long febrile episode, and his fever was refractory to many kinds of injected broad-spectrum antibiotics. When presenting with abdominal cramps, he was prescribed oral mannitol and gentamycin to get rid of the gut infection. This treatment resulted in a quick resolution of the fever. Unanticipatedly, it also produced an excellent hematological response. He had undergone three episodes of recurrence within the one-year treatment, with each recurrence occurring 7-8 wk from the gastrointestinal inflammation eliminating preparations. However,subsequent treatments were able to produce subsequent remissions and consecutive treatments were successful in achieving durative hematological improvements, strongly indicating an etiological association between chronic gut inflammation and the development of AA. Interestingly, comorbid diseases superimposed on this patient(namely, psychiatric disorders, hypertension,insulin resistance, and renal dysfunction) were ameliorated together with the hematological improvements.CONCLUSION Chronic gut inflammation may be responsible for AA pathogenesis. The comorbidities and AA may share a common etiological association.展开更多
BACKGROUND Severe hyperlipemia(SHLE)has an impact on the results of many kinds of laboratory tests.Complete blood count(CBC)examination by automated blood cell counter(ABCC)is a quick and convenient measurement for sc...BACKGROUND Severe hyperlipemia(SHLE)has an impact on the results of many kinds of laboratory tests.Complete blood count(CBC)examination by automated blood cell counter(ABCC)is a quick and convenient measurement for screening abnormalities of blood cells that are triggered by various pathogenic insults in disease diagnosis and for monitoring changes in the treatment of existing hematological conditions.However,CBC results are frequently affected by many intrinsic and extrinsic factors from blood samples,such as in the setting of hypergammaglobulinemia and certain anticoagulants.SHLE could also affect CBC results.CASE SUMMARY A 33-year-old Chinese male presented with painful foot numbness and abdominal pain.He was initially misdiagnosed as having a myeloproliferative neoplasm(MPN)because of the marked abnormalities in CBC examination by the ABCC.Morphological evaluation of the bone marrow smears and biopsy showed no evidence of MPN.Gene mutations in Breakpoint cluster regions-Abelson murine leukemia viral oncogene homologue 1(BCR-ABL1),Janus kinase 2(JAK2),calreticulin(CALR),myeloproliferative leukemia virus(MPL),and colony-stimulating factor 3 receptor(CSF3R)were negative.Having noticed the thick chylomicron layer on blood samples and the dramatically fluctuating CBC results,we speculated that the fat droplets formed by shaking the blood samples in the setting of SHLE were mistakenly identified as blood cells due to the limited parameters of ABCC.Therefore,we removed a large part of the chylomicron layer and then reexamined the CBC,and the CBC results,as we expected,differed significantly from that of the sample before the chylomicron layer was removed.These significant differences had been validated by the subsequently repeated laboratory tests by measuring dual blood samples that the chylomicron layer was removed in one sample and was not in another,and comparing the CBC results.Computerized tomography reexamination of the upper abdomen revealed an exudative lesion surrounding his pancreas.After intensive consultation,definitive diagnosis was made as recurrent pancreatitis,hyperlipemia and pseudoerythrocytosis.CONCLUSION SHLE may become a potential cause of misdiagnosis of hyperlipemia-related diseases as MPNs and the resultant mistreatment.It may also lead to the misinterpretation of transfusion indications in patients with hematological disorders who critically need blood transfusion for supportive treatment.展开更多
We previously reported a serendipitous finding from a patient with refractory severe aplastic anemia who had gotten an unexpected hematological response to treatment with gut-cleansing preparations(GCPs).This patient ...We previously reported a serendipitous finding from a patient with refractory severe aplastic anemia who had gotten an unexpected hematological response to treatment with gut-cleansing preparations(GCPs).This patient experienced three recurrences over the ensuing one year of intermittent GCP treatments,with each recurrence occurring 7-8 wk from a GCP.After his third recurrence,he was prescribed successive treatment with rifampicin,berberine,and monthly administered GCP for 4 mo,and he developed an erythroid proliferative neoplasma and an overwhelming enteropathy,and eventually died of septic shock.Laboratory investigations had validated the resolution of myelosuppression and the appearance of malignant clonal hematopoiesis.From the treatment process and laboratory investigations,it is reasonably inferred that the engagement of gut inflammation is critically required in sustaining the overall pathophysiology of acquired aplastic anemia probably by creating a chronic inflammatory state.Incorporation of rifampicin,berberine,and monthly GCP into cyclosporine can enhance the immunosuppressive effect.In a subgroup of acquired aplastic anemia patients whose pathogenesis is associated with genotoxic exposure,the suppressed normal hematopoiesis may result from the bystander insult that is mediated by the soluble inflammatory cytokines generated in response to the immunogenic products of damaged hematopoietic cells in the context of chronic inflammatory state and may offer a protective antineoplastic mechanism against malignant proliferation.展开更多
Acquired aplastic anemia(AAA)is an auto-immune disease(AID)resulting from aberrant T-cell-mediated and antigen-driven immune responses to hematopoietic stem and progenitor cells(HSPCs)in genetically susceptible indivi...Acquired aplastic anemia(AAA)is an auto-immune disease(AID)resulting from aberrant T-cell-mediated and antigen-driven immune responses to hematopoietic stem and progenitor cells(HSPCs)in genetically susceptible individuals,leading to the significantly enhanced suppression and apoptosis of HSPCs.Active systemic and local inflammation is responsible for the overall pathophysiology.展开更多
As a morphological sub-type of multiple myeloma(MM),anaplastic myeloma(AM),which includes anaplastic multiple myeloma and anaplastic plasmacytoma,is a rare disease that aggressively progresses.It is reported to be ver...As a morphological sub-type of multiple myeloma(MM),anaplastic myeloma(AM),which includes anaplastic multiple myeloma and anaplastic plasmacytoma,is a rare disease that aggressively progresses.It is reported to be very resistant to chemotherapy and exhibits a poor prognosis.1 Although AM was.detected in 2.6%of patients in a large series of cases,there have only been some case reports since AM was first reported in 1983.31 Therefore,AM lacks detailed descriptions and strict definitions.To further analyze the clinical and pathological features of AM patients and to provide some suggestions for diagnosis and treatment,we retrospectively analyzed four AM patients who were admitted to the Affliated Hospital of Qingdao University.展开更多
文摘BACKGROUND Cumulative evidence suggests that the aberrant immune responses in acquired aplastic anemia(AA) are sustained by active chronic infections in genetically susceptible individuals. Recently, the constant source to trigger and sustain the pathophysiology has been proposed to come from the altered gut microbiota and chronic intestinal inflammation. In this case, our serendipitous finding provides convincing evidence that the persistently dysregulated autoimmunity may be generated, at least in a significant proposition of AA patients, by the altered gut microbiota and compromised intestinal epithelium.CASE SUMMARY A 30-year-old Chinese male patient with refractory severe AA experienced a 3-month-long febrile episode, and his fever was refractory to many kinds of injected broad-spectrum antibiotics. When presenting with abdominal cramps, he was prescribed oral mannitol and gentamycin to get rid of the gut infection. This treatment resulted in a quick resolution of the fever. Unanticipatedly, it also produced an excellent hematological response. He had undergone three episodes of recurrence within the one-year treatment, with each recurrence occurring 7-8 wk from the gastrointestinal inflammation eliminating preparations. However,subsequent treatments were able to produce subsequent remissions and consecutive treatments were successful in achieving durative hematological improvements, strongly indicating an etiological association between chronic gut inflammation and the development of AA. Interestingly, comorbid diseases superimposed on this patient(namely, psychiatric disorders, hypertension,insulin resistance, and renal dysfunction) were ameliorated together with the hematological improvements.CONCLUSION Chronic gut inflammation may be responsible for AA pathogenesis. The comorbidities and AA may share a common etiological association.
文摘BACKGROUND Severe hyperlipemia(SHLE)has an impact on the results of many kinds of laboratory tests.Complete blood count(CBC)examination by automated blood cell counter(ABCC)is a quick and convenient measurement for screening abnormalities of blood cells that are triggered by various pathogenic insults in disease diagnosis and for monitoring changes in the treatment of existing hematological conditions.However,CBC results are frequently affected by many intrinsic and extrinsic factors from blood samples,such as in the setting of hypergammaglobulinemia and certain anticoagulants.SHLE could also affect CBC results.CASE SUMMARY A 33-year-old Chinese male presented with painful foot numbness and abdominal pain.He was initially misdiagnosed as having a myeloproliferative neoplasm(MPN)because of the marked abnormalities in CBC examination by the ABCC.Morphological evaluation of the bone marrow smears and biopsy showed no evidence of MPN.Gene mutations in Breakpoint cluster regions-Abelson murine leukemia viral oncogene homologue 1(BCR-ABL1),Janus kinase 2(JAK2),calreticulin(CALR),myeloproliferative leukemia virus(MPL),and colony-stimulating factor 3 receptor(CSF3R)were negative.Having noticed the thick chylomicron layer on blood samples and the dramatically fluctuating CBC results,we speculated that the fat droplets formed by shaking the blood samples in the setting of SHLE were mistakenly identified as blood cells due to the limited parameters of ABCC.Therefore,we removed a large part of the chylomicron layer and then reexamined the CBC,and the CBC results,as we expected,differed significantly from that of the sample before the chylomicron layer was removed.These significant differences had been validated by the subsequently repeated laboratory tests by measuring dual blood samples that the chylomicron layer was removed in one sample and was not in another,and comparing the CBC results.Computerized tomography reexamination of the upper abdomen revealed an exudative lesion surrounding his pancreas.After intensive consultation,definitive diagnosis was made as recurrent pancreatitis,hyperlipemia and pseudoerythrocytosis.CONCLUSION SHLE may become a potential cause of misdiagnosis of hyperlipemia-related diseases as MPNs and the resultant mistreatment.It may also lead to the misinterpretation of transfusion indications in patients with hematological disorders who critically need blood transfusion for supportive treatment.
文摘We previously reported a serendipitous finding from a patient with refractory severe aplastic anemia who had gotten an unexpected hematological response to treatment with gut-cleansing preparations(GCPs).This patient experienced three recurrences over the ensuing one year of intermittent GCP treatments,with each recurrence occurring 7-8 wk from a GCP.After his third recurrence,he was prescribed successive treatment with rifampicin,berberine,and monthly administered GCP for 4 mo,and he developed an erythroid proliferative neoplasma and an overwhelming enteropathy,and eventually died of septic shock.Laboratory investigations had validated the resolution of myelosuppression and the appearance of malignant clonal hematopoiesis.From the treatment process and laboratory investigations,it is reasonably inferred that the engagement of gut inflammation is critically required in sustaining the overall pathophysiology of acquired aplastic anemia probably by creating a chronic inflammatory state.Incorporation of rifampicin,berberine,and monthly GCP into cyclosporine can enhance the immunosuppressive effect.In a subgroup of acquired aplastic anemia patients whose pathogenesis is associated with genotoxic exposure,the suppressed normal hematopoiesis may result from the bystander insult that is mediated by the soluble inflammatory cytokines generated in response to the immunogenic products of damaged hematopoietic cells in the context of chronic inflammatory state and may offer a protective antineoplastic mechanism against malignant proliferation.
文摘Acquired aplastic anemia(AAA)is an auto-immune disease(AID)resulting from aberrant T-cell-mediated and antigen-driven immune responses to hematopoietic stem and progenitor cells(HSPCs)in genetically susceptible individuals,leading to the significantly enhanced suppression and apoptosis of HSPCs.Active systemic and local inflammation is responsible for the overall pathophysiology.
基金The study was supported by grants from the National Natural Science Foundation of China(No.81400166)the Key Research and Development Program of Shandong Province(No.2018GSF118157).
文摘As a morphological sub-type of multiple myeloma(MM),anaplastic myeloma(AM),which includes anaplastic multiple myeloma and anaplastic plasmacytoma,is a rare disease that aggressively progresses.It is reported to be very resistant to chemotherapy and exhibits a poor prognosis.1 Although AM was.detected in 2.6%of patients in a large series of cases,there have only been some case reports since AM was first reported in 1983.31 Therefore,AM lacks detailed descriptions and strict definitions.To further analyze the clinical and pathological features of AM patients and to provide some suggestions for diagnosis and treatment,we retrospectively analyzed four AM patients who were admitted to the Affliated Hospital of Qingdao University.
