BACKGROUND Immunoglobulin light chain(AL)amyloidosis is a rare disease characterized by deposition of ALs essentially in any organ or tissue,with cardiac involvement being very frequent(61%).Early diagnosis is of high...BACKGROUND Immunoglobulin light chain(AL)amyloidosis is a rare disease characterized by deposition of ALs essentially in any organ or tissue,with cardiac involvement being very frequent(61%).Early diagnosis is of high importance because early initiation of treatment in AL amyloidosis may improve outcomes.Despite the administration of immunotherapeutic agents,in particular bortezomib and daratumumab,which have improved the outcomes of AL amyloidosis,antiplasma cell therapy remains suboptimal for some patients.CASE SUMMARY We report the case of a 55-year-old man presenting with heart failure who was diagnosed with cardiac AL amyloidosis by an endomyocardial biopsy.He experienced a short-term hematological remission with no organ response after being administered a bortezomib-daratumumab containing regimen.The treatment was switched to pomolidomide due to pulmonary involvement and progressive pleural effusion,in which flow cytometry analysis showed abnormal plasma cells.After two cycles of this regimen,the pleural effusion was controlled effectively with no recurrence.CONCLUSION This case emphasizes the crucial role of endomyocardial biopsy in early diagnosis of cardiac amyloidosis and suggests that pomolidomide may be an effective treatment for patients with AL amyloidosis that is relapsed/refractory to both bortezomib and daratumumab.展开更多
BACKGROUND Multiple myeloma(MM)can be accompanied by amyloidosis,which occurs in a small number of patients and is characterized by deposition of light chains in the joints,leading to multiple myeloma-associated amylo...BACKGROUND Multiple myeloma(MM)can be accompanied by amyloidosis,which occurs in a small number of patients and is characterized by deposition of light chains in the joints,leading to multiple myeloma-associated amyloid arthropathy(MAA).As a rare complication of MM,clinical manifestations of MAA are often similar to those of rheumatoid arthritis,and the two are easily confused.CASE SUMMARY In recent years,our center treated two patients of MM with amyloid arthropathy as the first manifestation,both of whom presented with polyarthritis.After treatment for MM,both patients achieved complete remission.However,subsequently,the two patients underwent hip arthroplasty for femoral neck fractures.Congo red staining and immunofluorescence of the joint tissues confirmed MAA after surgery.Eventually,one of the patients died of MM recurrence,while the other survived.CONCLUSION MAA should be regarded as an initial symptom of MM and should be taken seriously.展开更多
Objective： Our aim was to investigate clinical and laboratory characteristics of osteoarthritic patients who had amyloid deposition in their knee joints. Methods： Synovial membranes were obtained from 36 patients wi...Objective： Our aim was to investigate clinical and laboratory characteristics of osteoarthritic patients who had amyloid deposition in their knee joints. Methods： Synovial membranes were obtained from 36 patients with knee osteoarthritis （OA） who underwent joint replacement surgery. From this sample, the diagnosis of amyloid was deter- mined by Congo red staining, which demonstrated apple-green birefringence under a polarized microscope. All syn- ovial membranes were immunohistochemically characterized for the expressions of amyloid immunoglobulin light chain （AL-K and AL-,k）, serum amyloid-A （SAA）, amyloidogenic transthyretin （ATTR）, and amyloidogenic 152- microglobulin （A152M）. Matrix-assisted laser desorption-ionizaton/time of flight mass spectrometry （MALDI-TOF MS） was used to analyze transthyretin （TTR） isoforms in the serum of each patient. Results： Nine cases （25%） were found to be amyloid-positive. Immunohistochemicaliy, eight cases （88.9%） had ATTR deposition, and one sample （11.1%） was shown to be AL-K-positive. MALDI-TOF MS identified that the TTR in the serum of the patients was unmodified wild-type TTR, TTR-Cys-S-S-Cys, and TTR-Cys-S-S-CysGly. The age at surgery and the disease duration were sig- nificantly higher in the ATTR-positive group than in the ATTR-negative group. Knee score and function score were significantly lower in the ATTR-positive group than in the ATTR-negative group. Conclusions： Amyloid deposition in synovial membranes of OA patients was found to be ATTR and AL-K. TTR in the serum of the patients was unmodified wild-type TTR together with two isoforms. The high age at surgery, long disease duration, and a deteriorated knee function were associated with ATTR amyloid deposition in the osteoarthritic knee joints.展开更多
文摘BACKGROUND Immunoglobulin light chain(AL)amyloidosis is a rare disease characterized by deposition of ALs essentially in any organ or tissue,with cardiac involvement being very frequent(61%).Early diagnosis is of high importance because early initiation of treatment in AL amyloidosis may improve outcomes.Despite the administration of immunotherapeutic agents,in particular bortezomib and daratumumab,which have improved the outcomes of AL amyloidosis,antiplasma cell therapy remains suboptimal for some patients.CASE SUMMARY We report the case of a 55-year-old man presenting with heart failure who was diagnosed with cardiac AL amyloidosis by an endomyocardial biopsy.He experienced a short-term hematological remission with no organ response after being administered a bortezomib-daratumumab containing regimen.The treatment was switched to pomolidomide due to pulmonary involvement and progressive pleural effusion,in which flow cytometry analysis showed abnormal plasma cells.After two cycles of this regimen,the pleural effusion was controlled effectively with no recurrence.CONCLUSION This case emphasizes the crucial role of endomyocardial biopsy in early diagnosis of cardiac amyloidosis and suggests that pomolidomide may be an effective treatment for patients with AL amyloidosis that is relapsed/refractory to both bortezomib and daratumumab.
文摘BACKGROUND Multiple myeloma(MM)can be accompanied by amyloidosis,which occurs in a small number of patients and is characterized by deposition of light chains in the joints,leading to multiple myeloma-associated amyloid arthropathy(MAA).As a rare complication of MM,clinical manifestations of MAA are often similar to those of rheumatoid arthritis,and the two are easily confused.CASE SUMMARY In recent years,our center treated two patients of MM with amyloid arthropathy as the first manifestation,both of whom presented with polyarthritis.After treatment for MM,both patients achieved complete remission.However,subsequently,the two patients underwent hip arthroplasty for femoral neck fractures.Congo red staining and immunofluorescence of the joint tissues confirmed MAA after surgery.Eventually,one of the patients died of MM recurrence,while the other survived.CONCLUSION MAA should be regarded as an initial symptom of MM and should be taken seriously.
文摘Objective： Our aim was to investigate clinical and laboratory characteristics of osteoarthritic patients who had amyloid deposition in their knee joints. Methods： Synovial membranes were obtained from 36 patients with knee osteoarthritis （OA） who underwent joint replacement surgery. From this sample, the diagnosis of amyloid was deter- mined by Congo red staining, which demonstrated apple-green birefringence under a polarized microscope. All syn- ovial membranes were immunohistochemically characterized for the expressions of amyloid immunoglobulin light chain （AL-K and AL-,k）, serum amyloid-A （SAA）, amyloidogenic transthyretin （ATTR）, and amyloidogenic 152- microglobulin （A152M）. Matrix-assisted laser desorption-ionizaton/time of flight mass spectrometry （MALDI-TOF MS） was used to analyze transthyretin （TTR） isoforms in the serum of each patient. Results： Nine cases （25%） were found to be amyloid-positive. Immunohistochemicaliy, eight cases （88.9%） had ATTR deposition, and one sample （11.1%） was shown to be AL-K-positive. MALDI-TOF MS identified that the TTR in the serum of the patients was unmodified wild-type TTR, TTR-Cys-S-S-Cys, and TTR-Cys-S-S-CysGly. The age at surgery and the disease duration were sig- nificantly higher in the ATTR-positive group than in the ATTR-negative group. Knee score and function score were significantly lower in the ATTR-positive group than in the ATTR-negative group. Conclusions： Amyloid deposition in synovial membranes of OA patients was found to be ATTR and AL-K. TTR in the serum of the patients was unmodified wild-type TTR together with two isoforms. The high age at surgery, long disease duration, and a deteriorated knee function were associated with ATTR amyloid deposition in the osteoarthritic knee joints.
