BACKGROUND Glomerulopathy with fibrillary deposits is not uncommon in routine nephropathology practice,with amyloidosis and fibrillary glomerulonephritis being the two most frequently encountered entities.Renal amyloi...BACKGROUND Glomerulopathy with fibrillary deposits is not uncommon in routine nephropathology practice,with amyloidosis and fibrillary glomerulonephritis being the two most frequently encountered entities.Renal amyloid heavy and light chain(AHL)is relatively uncommon and its biopsy diagnosis is usually limited to cases that show strong equivalent staining for a single immunoglobulin(Ig)heavy chain and a single light chain,further supported by mass spectrometry(MS)and serum studies for monoclonal protein.But polyclonal light chain staining can pose a challenge.CASE SUMMARY Herein we present a challenging case of renal AHL with polyclonal and polytypic Ig gamma(IgG)staining pattern by immunofluorescence.The patient is a 62-yearold Caucasian male who presented to an outside institution with a serum creatinine of up to 8.1 mg/dL and nephrotic range proteinuria.Despite the finding of a polyclonal and polytypic staining pattern on immunofluorescence,ultrastructural study of the renal biopsy demonstrated the presence of fibrils with a mean diameter of 10 nm.Congo red was positive while DNAJB9 was negative.MS suggested a diagnosis of amyloid AHL type with IgG and lambda,but kappa light chains were also present supporting the immunofluorescence staining results.Serum immunofixation studies demonstrated IgG lambda monoclonal spike.The patient was started on chemotherapy.The chronic renal injury however was quite advanced and he ended up needing dialysis shortly after.CONCLUSION Tissue diagnosis of AHL amyloid can be tricky.Thorough confirmation using other available diagnostic techniques is recommended in such cases.展开更多
We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT level...We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT levels with normal transaminases and bilirubin. On imaging she had diffuse enlargement of liver with heterogeneous contrast uptake in liver. Her viral marker and autoimmune markers were negative. Liver biopsy depicted massive deposition of amyloid in peri-sinusoidal spaces which revealed apple green birefringence on polarizing microscopy after Congo red staining. Cardiac and renal evaluation was unremarkable. Abdominal fat pad and rectum biopsy was negative for amyloid deposit. There was no evidence of primary amyloidosis as bone marrow examination was normal. Serum and urine immunofixation electrophoresis were normal. Immunoperoxidase staining for serum amyloid associated protein for secondary amyloidosis was negative from liver biopsy. We present this rare case of primary hepatic amyloidosis and review the literature regarding varied presentations of hepatic involvement in amyloidosis.展开更多
An improved method for showing Gc band by sulfosalicylic acidafter IEF is reported. This method provides us an effective mean to identify theGc subtypes and its variants within 4 h without using anti-serum for immunof...An improved method for showing Gc band by sulfosalicylic acidafter IEF is reported. This method provides us an effective mean to identify theGc subtypes and its variants within 4 h without using anti-serum for immunofix-ation. And new variants Gc<sup>1c3</sup> and Gc<sup>2c7</sup> are discovered in Chinese people, andtheir frequencies are 0. 0008 and 0. 0004 respectively.展开更多
BACKGROUND Immunoglobulin D(IgD)multiple myeloma(MM)is a rare subtype of MM and commonly occurs in younger subjects but at a later stage of the International Staging System(ISS)when admitted.As a special type of IgD m...BACKGROUND Immunoglobulin D(IgD)multiple myeloma(MM)is a rare subtype of MM and commonly occurs in younger subjects but at a later stage of the International Staging System(ISS)when admitted.As a special type of IgD myeloma,IgD-λ/λbiclonal MM is rarer.Its serum protein electrophoresis and serum immunofixation electrophoresis(IFE)might find no anomalies even if the bone marrow(BM)examination is performed.Thus,it is easy to miss the diagnosis.CASE SUMMARY A 62-year-old man diagnosed as IgD-λ/λmyeloma(ISS stage III)was admitted with fatigue and weight loss.The physical examination suggested an anemic face,a few moist rales at the left lung base,and mild concave edema in both lower extremities.Laboratory examinations showed the elevated creatinine levels,β2-microglobulin,lactic dehydrogenase,and erythrocyte sedimentation rate,while the decreased neutrophils,granulocytes,and hemoglobin.In the serum protein electrophoresis,there appeared two inconspicuous M-spikes.Serum IFE indicated an over-representation of lambda light chain and yielded two monoclonal bands inλregion,but only one corresponding heavy chain band in the antisera to IgD region.The BM histology and BM cytology both supported the diagnosis of IgD-λ/λmyeloma.CONCLUSION This case highlights the differential clinical manifestations and laboratory findings of IgD-λ/λmyeloma to help minimize the chance of misdiagnosis.展开更多
Monoclonal gammopathy of undetermined significance (MGUS) is characterized by increased production of an immunoglobuling (Ig) from a clone of plasma cells and is a pre-malignant disorders in subjects older than 50 yea...Monoclonal gammopathy of undetermined significance (MGUS) is characterized by increased production of an immunoglobuling (Ig) from a clone of plasma cells and is a pre-malignant disorders in subjects older than 50 years. The prevalence of MGUS in Caucasian population is still not determined. MGUS is characterized by the presence of a monoclonal-protein(M-protein) (IgG and IgA) lower than 30 g/L, bone marrow plasma cell percentage lower than 10%, and absence of clinical signs related to multiple myeloma (MM). MGUS can be responsible for damage to organs through the production of toxic M proteins that may have autoantibody activity or deposit pathologically in the organ tissues. Many techniques are available for the characterization of M-proteins. These techniques can involve different expenses, skills, labor time, and sensitivity in detecting monoclonal proteins also at low-level. Detection of M-proteins needs of assays based on high-resolution electrophoresis and im-munofixation (or immunosubtraction). We show suggestive clinical cases where the subjects involved had not an apparent disease but they showed an interesting pattern in electrophoresis. All cases were investigated by capillary’s electrophoresis and immunofixation to confirm or not the clinical suspect, and then if the immunofixation is not exhaustive, additionally immunosubstraction is done. However in some cases, the interpretation of the peaks is not so easy. Clinical and scientific data provided evidences that immunofixaction technique can fail the identification of monoclonal components. In that cases, we opted for the immunosubtraction method as a third level test, in that cases when immunofixation failed the identification of a monoclonal protein.展开更多
文摘BACKGROUND Glomerulopathy with fibrillary deposits is not uncommon in routine nephropathology practice,with amyloidosis and fibrillary glomerulonephritis being the two most frequently encountered entities.Renal amyloid heavy and light chain(AHL)is relatively uncommon and its biopsy diagnosis is usually limited to cases that show strong equivalent staining for a single immunoglobulin(Ig)heavy chain and a single light chain,further supported by mass spectrometry(MS)and serum studies for monoclonal protein.But polyclonal light chain staining can pose a challenge.CASE SUMMARY Herein we present a challenging case of renal AHL with polyclonal and polytypic Ig gamma(IgG)staining pattern by immunofluorescence.The patient is a 62-yearold Caucasian male who presented to an outside institution with a serum creatinine of up to 8.1 mg/dL and nephrotic range proteinuria.Despite the finding of a polyclonal and polytypic staining pattern on immunofluorescence,ultrastructural study of the renal biopsy demonstrated the presence of fibrils with a mean diameter of 10 nm.Congo red was positive while DNAJB9 was negative.MS suggested a diagnosis of amyloid AHL type with IgG and lambda,but kappa light chains were also present supporting the immunofluorescence staining results.Serum immunofixation studies demonstrated IgG lambda monoclonal spike.The patient was started on chemotherapy.The chronic renal injury however was quite advanced and he ended up needing dialysis shortly after.CONCLUSION Tissue diagnosis of AHL amyloid can be tricky.Thorough confirmation using other available diagnostic techniques is recommended in such cases.
文摘We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT levels with normal transaminases and bilirubin. On imaging she had diffuse enlargement of liver with heterogeneous contrast uptake in liver. Her viral marker and autoimmune markers were negative. Liver biopsy depicted massive deposition of amyloid in peri-sinusoidal spaces which revealed apple green birefringence on polarizing microscopy after Congo red staining. Cardiac and renal evaluation was unremarkable. Abdominal fat pad and rectum biopsy was negative for amyloid deposit. There was no evidence of primary amyloidosis as bone marrow examination was normal. Serum and urine immunofixation electrophoresis were normal. Immunoperoxidase staining for serum amyloid associated protein for secondary amyloidosis was negative from liver biopsy. We present this rare case of primary hepatic amyloidosis and review the literature regarding varied presentations of hepatic involvement in amyloidosis.
文摘An improved method for showing Gc band by sulfosalicylic acidafter IEF is reported. This method provides us an effective mean to identify theGc subtypes and its variants within 4 h without using anti-serum for immunofix-ation. And new variants Gc<sup>1c3</sup> and Gc<sup>2c7</sup> are discovered in Chinese people, andtheir frequencies are 0. 0008 and 0. 0004 respectively.
基金National Natural Science Foundation of China,No.81500430 and No.U1304802Science and Technology Planning Project of Henan Province,No.192102310045,No.182102310544,No.182102310566,and No.182102310573Henan Medical Science and Technology Tackling Project,No.2018020320.
文摘BACKGROUND Immunoglobulin D(IgD)multiple myeloma(MM)is a rare subtype of MM and commonly occurs in younger subjects but at a later stage of the International Staging System(ISS)when admitted.As a special type of IgD myeloma,IgD-λ/λbiclonal MM is rarer.Its serum protein electrophoresis and serum immunofixation electrophoresis(IFE)might find no anomalies even if the bone marrow(BM)examination is performed.Thus,it is easy to miss the diagnosis.CASE SUMMARY A 62-year-old man diagnosed as IgD-λ/λmyeloma(ISS stage III)was admitted with fatigue and weight loss.The physical examination suggested an anemic face,a few moist rales at the left lung base,and mild concave edema in both lower extremities.Laboratory examinations showed the elevated creatinine levels,β2-microglobulin,lactic dehydrogenase,and erythrocyte sedimentation rate,while the decreased neutrophils,granulocytes,and hemoglobin.In the serum protein electrophoresis,there appeared two inconspicuous M-spikes.Serum IFE indicated an over-representation of lambda light chain and yielded two monoclonal bands inλregion,but only one corresponding heavy chain band in the antisera to IgD region.The BM histology and BM cytology both supported the diagnosis of IgD-λ/λmyeloma.CONCLUSION This case highlights the differential clinical manifestations and laboratory findings of IgD-λ/λmyeloma to help minimize the chance of misdiagnosis.
文摘Monoclonal gammopathy of undetermined significance (MGUS) is characterized by increased production of an immunoglobuling (Ig) from a clone of plasma cells and is a pre-malignant disorders in subjects older than 50 years. The prevalence of MGUS in Caucasian population is still not determined. MGUS is characterized by the presence of a monoclonal-protein(M-protein) (IgG and IgA) lower than 30 g/L, bone marrow plasma cell percentage lower than 10%, and absence of clinical signs related to multiple myeloma (MM). MGUS can be responsible for damage to organs through the production of toxic M proteins that may have autoantibody activity or deposit pathologically in the organ tissues. Many techniques are available for the characterization of M-proteins. These techniques can involve different expenses, skills, labor time, and sensitivity in detecting monoclonal proteins also at low-level. Detection of M-proteins needs of assays based on high-resolution electrophoresis and im-munofixation (or immunosubtraction). We show suggestive clinical cases where the subjects involved had not an apparent disease but they showed an interesting pattern in electrophoresis. All cases were investigated by capillary’s electrophoresis and immunofixation to confirm or not the clinical suspect, and then if the immunofixation is not exhaustive, additionally immunosubstraction is done. However in some cases, the interpretation of the peaks is not so easy. Clinical and scientific data provided evidences that immunofixaction technique can fail the identification of monoclonal components. In that cases, we opted for the immunosubtraction method as a third level test, in that cases when immunofixation failed the identification of a monoclonal protein.
