Introduction:Primary systemic amyloidosis is characterized by clonal plasma cell disorder,and its signs and symptoms are various and complex,damage to the skin and mucous membrane is often more likely to attract atten...Introduction:Primary systemic amyloidosis is characterized by clonal plasma cell disorder,and its signs and symptoms are various and complex,damage to the skin and mucous membrane is often more likely to attract attention.Here we reported a case of a 61-year-old male patient who presented with topical mucocutaneous lesion,as well unusual skin vegetations.Case presentation:A 61-year-old man was hospitalized due to repeated burning sensation on his back,multiple ecchymosis,and skin vegetations.Through a series of examinations(mainly including skin histopathology,bone marrow cytology,bone marrow flow cytometry,immunofixation electrophoresis),Primary systemic amyloidosis was diagnosed,but multiple myeloma could not be diagnosed.Subsequently,he received chemotherapy.In the half-year follow-up,there was no significant change in his symptoms and signs.Discussion:In this case,in addition to the typical skin damage of primary amyloidosis,the multiple skin vegetations in the buttocks,abdomen,and arms are particularly noteworthy.According to the histopathology and Immunohistochemistry of the skin vegetation,we infer that the formation mechanism of these skin vegetation is lymphatic obstruction caused by amyloid,which leads to lymphatic dilatation,lymph leakage,and dermal edema.Conclusion:Primary systemic amyloidosis is a rare disease,which is often difficult to diagnose.We should be alert to those atypical skin features so as not to delay diagnosis.展开更多
Background Primary systemic light chain amyloidosis （AL） is a rare plasma cell disease,our purpose was to analyze the immunophenotypic characteristics of the plasma cells in bone marrow in AL patients,and explore wh...Background Primary systemic light chain amyloidosis （AL） is a rare plasma cell disease,our purpose was to analyze the immunophenotypic characteristics of the plasma cells in bone marrow in AL patients,and explore whether the detection of abnormal plasma cell clones in bone marrow by flow cytometry （FCM） could be used as an important indicator of AL diagnosis.Methods Fresh bone marrow samples were collected from 51 AL,21 multiple myeloma （MM）,and 5 Waldenstr（o）m＇s macroglobulinemia （WM） patients.The immunophenotype of bone marrow cells were analyzed and compared by FCM using a panel of antibodies including CD45,CD38,CD138,CD117,CD56,and CD19.Results In AL,light chain restriction could be identified in 31 cases （60.9%）,in which the λ light chain restriction was found in 24 cases （77.4%）.In MM,κ light chain restriction was found in 13 cases （61.9%）,and λ light chain restriction in eight cases.CD45 on abnormal plasma cells was negative to weakly positive in both AL and MM,but was positive to strongly positive in WM.In the bone marrow plasma cells of the 51 AL,78.4% were CD56＋,68.6% were CD117＋,and 88.2% were CD19-.While in the 21 MM cases,66.7% were CD56＋,38.1% were CD117＋,and 90.4% were CD19-.The plasmacytoid lymphocytes in the five WM patients were CD19＋ and CD56-,CD117-.Conclusion Detection of abnormal plasma cell clones in bone marrow by FCM is valuable for the diagnosis of AL.展开更多
Primary systemic amyloidosis is a relatively rare disease, caused when abnormal extracellular deposition of fibrillary protein builds up in a variety of target organs, such as heart, kidneys, lungs liver, and so forth...Primary systemic amyloidosis is a relatively rare disease, caused when abnormal extracellular deposition of fibrillary protein builds up in a variety of target organs, such as heart, kidneys, lungs liver, and so forth. The symptoms of the disease are usually vague, while many kinds of auxiliary or laboratory examinations especially pathologic biopsy can provide a clue for the diagnosis. Here we described a case who had purpura-like lesions in the initial stage, followed by progressive malfunctions in the kidneys, the heart, the lungs, as wel~ as the liver. The final diagnosis was primary systemic amyloidosis determined by skin pathologic biopsy. And the disease led to a fatal outcome within three months after the diagnosis.展开更多
文摘Introduction:Primary systemic amyloidosis is characterized by clonal plasma cell disorder,and its signs and symptoms are various and complex,damage to the skin and mucous membrane is often more likely to attract attention.Here we reported a case of a 61-year-old male patient who presented with topical mucocutaneous lesion,as well unusual skin vegetations.Case presentation:A 61-year-old man was hospitalized due to repeated burning sensation on his back,multiple ecchymosis,and skin vegetations.Through a series of examinations(mainly including skin histopathology,bone marrow cytology,bone marrow flow cytometry,immunofixation electrophoresis),Primary systemic amyloidosis was diagnosed,but multiple myeloma could not be diagnosed.Subsequently,he received chemotherapy.In the half-year follow-up,there was no significant change in his symptoms and signs.Discussion:In this case,in addition to the typical skin damage of primary amyloidosis,the multiple skin vegetations in the buttocks,abdomen,and arms are particularly noteworthy.According to the histopathology and Immunohistochemistry of the skin vegetation,we infer that the formation mechanism of these skin vegetation is lymphatic obstruction caused by amyloid,which leads to lymphatic dilatation,lymph leakage,and dermal edema.Conclusion:Primary systemic amyloidosis is a rare disease,which is often difficult to diagnose.We should be alert to those atypical skin features so as not to delay diagnosis.
基金This work was funded by the National Natural Science Foundation of China （No.81370612） and the Youth Fund Project of the National Natural Science Foundation of China （No.81102211）.
文摘Background Primary systemic light chain amyloidosis （AL） is a rare plasma cell disease,our purpose was to analyze the immunophenotypic characteristics of the plasma cells in bone marrow in AL patients,and explore whether the detection of abnormal plasma cell clones in bone marrow by flow cytometry （FCM） could be used as an important indicator of AL diagnosis.Methods Fresh bone marrow samples were collected from 51 AL,21 multiple myeloma （MM）,and 5 Waldenstr（o）m＇s macroglobulinemia （WM） patients.The immunophenotype of bone marrow cells were analyzed and compared by FCM using a panel of antibodies including CD45,CD38,CD138,CD117,CD56,and CD19.Results In AL,light chain restriction could be identified in 31 cases （60.9%）,in which the λ light chain restriction was found in 24 cases （77.4%）.In MM,κ light chain restriction was found in 13 cases （61.9%）,and λ light chain restriction in eight cases.CD45 on abnormal plasma cells was negative to weakly positive in both AL and MM,but was positive to strongly positive in WM.In the bone marrow plasma cells of the 51 AL,78.4% were CD56＋,68.6% were CD117＋,and 88.2% were CD19-.While in the 21 MM cases,66.7% were CD56＋,38.1% were CD117＋,and 90.4% were CD19-.The plasmacytoid lymphocytes in the five WM patients were CD19＋ and CD56-,CD117-.Conclusion Detection of abnormal plasma cell clones in bone marrow by FCM is valuable for the diagnosis of AL.
文摘Primary systemic amyloidosis is a relatively rare disease, caused when abnormal extracellular deposition of fibrillary protein builds up in a variety of target organs, such as heart, kidneys, lungs liver, and so forth. The symptoms of the disease are usually vague, while many kinds of auxiliary or laboratory examinations especially pathologic biopsy can provide a clue for the diagnosis. Here we described a case who had purpura-like lesions in the initial stage, followed by progressive malfunctions in the kidneys, the heart, the lungs, as wel~ as the liver. The final diagnosis was primary systemic amyloidosis determined by skin pathologic biopsy. And the disease led to a fatal outcome within three months after the diagnosis.
