BACKGROUND: M protein, as a marker for monoclonal gammopathy, has been evaluated by protein electrophoresis (PEP) and immunofixation electrophoresis (IFE). Recently a highly sensitive, automated immunonephelometric assay for measurement of free light chains (FLCs) in serum and urine has been developed for the identification and monitoring of patients with monoclonal gammopathy. METHODS: We measured the concentration of kappa and lambda FLCs in 120 healthy individuals aged 20 to 80 years to establish the reference range of kappa and lambda FLCs and kappa/lambda FLC ratio and in 61 serum and 14 urine samples from patients with multiple myeloma (MM) to examine the correlation between the amount of M protein indirectly calculated on PEP and the direct measurement of FLCs. RESULTS: The concentrations of kappa and lambda FLCs and the kappa/lambda FLC ratio in healthy individuals were not significantly related to age or sex. The 95 percentile reference ranges for kappa FLC, lambda FLC, and kappa/lambda FLC ratio were 8.5-23.7 mg/L, 9.5-23.5 mg/L, and 0.67-1.38, respectively. On the PEP performed with MM specimens, 18 cases did not show the evidence of M protein. But, they revealed abnormal FLC concentrations on FLC assay and a significant correlation was found between the amount of M protein and the concentration of kappa and lambda FLC. However, inconsistent results such as the concentra-tion of kappa+lambda FLCs being more than the total protein in urine or M protein in serum were found in 5 of the 14 urine and 1 of the 61 serum samples of MM patients. CONCLUSIONS: FLC assay showed a good correlation with PEP and was more sensitive and accurate than PEP. Therefore, FLC assay is useful for diagnosing and monitoring monoclonal gammopathy at an early stage of the disease and during a remission state after chemotherapy or peripheral blood stem cell transplantation.
Drug Therapy ; Electrophoresis ; Humans ; Multiple Myeloma* ; Paraproteinemias ; Peripheral Blood Stem Cell Transplantation ; Reference Values*

Extramedullary plasmacytoma is a rare presentation of plasma cell dyscrasia. Most such tumors arise on the upper aerodigestive tract and renal plasmacytoma is very rare. The patient was 44 years old female presented with a 3 month-history of palpable mass in the right flank. There was a past history of complete remission after a chemotherapy for multiple myeloma (6 cycles of VAD chemotherapy) for the two years following the first diagnosis. After surgical resection, histologic and immunofluorescence studies of resected specimens revealed that the renal parenchyma was destroyed by sheets of mature plasma cells producing monoclonal protein (IgG-lambda) and by deposits of amorphous eosinophilic substance stained with anti-lambda antisera. Treatment with chemotherapy of Hyper-CVAD and local irradiation was done. The patient has been disease-free for 3 months after treatment. We report a case of relapsed renal plasmacytoma after complete remission of multiple myeloma.
Adult ; Diagnosis ; Drug Therapy ; Eosinophils ; Female ; Fluorescent Antibody Technique ; Humans ; Immune Sera ; Multiple Myeloma* ; Paraproteinemias ; Plasma Cells ; Plasmacytoma*

Primary systemic amyloidosis is associated with plasma cell dyscrasia, such as multiple myeloma. The amyloid fibrils in the disease are composed of amyloid light (AL) protein that is derived from immunoglobulin L chain. Cutaneous manifestations are presented in about 20~40% of patients with mainly petechia, purpura on flexural area of the body and waxy, translucent papulonodules. We report a case of primary systemic amyloidosis associated with multiple myeloma occurring in a 76-year old woman who had suffered from ecchymotic purpura on periorbital and flexural area with hemorrhagic bulla for one year. She also had macroglossia with grouped papulonodules on her tongue, and peripheral neuropathy. The final diagnosis was confirmed by positive PAS, Congo-red stain and specific serum/urine electrophoresis. She had been treated with several cycles of chemotherapy; however, she expired from various complications of the disease, such as congestive heart failure, renal failure, and secondary bacterial infection.
Aged ; Amyloid ; Amyloidosis* ; Bacterial Infections ; Diagnosis ; Drug Therapy ; Electrophoresis ; Female ; Heart Failure ; Humans ; Immunoglobulins ; Macroglossia ; Multiple Myeloma* ; Paraproteinemias ; Peripheral Nervous System Diseases ; Purpura ; Renal Insufficiency ; Tongue

Malignant melanoma is an uncommon tumor in Korea. To the best of our knowledge, we could not find malignant melanoma with multiple myeloma in Korean literature. A 57-year-old male patient had a 4×5 cm sized, irregular bordered, dark brownish plaque on the left sole, which has extended gradually since about 1 year ago and showed an occasional bleeding tendency. Laboratory examinations revealed a low hemoglobin level, rouleaux formation on peripheral blood, monoclonal gammopathy of IgG-kappa type and Bence-Jones proteinuria. Bone marrow aspiration findings showed markedly increased immature plasma cells suggesting multiple myeloma. Histopathologic findings of the skin biopsy from the left sole revealed proliferation of atypical melanocytes. We performed a surgical excision with a skin graft for malignant melanoma and chemotherapy (melphalan, vincristine and prednisolone) for multiple myeloma.
Biopsy ; Bone Marrow ; Drug Therapy ; Hemorrhage ; Humans ; Korea ; Male ; Melanocytes ; Melanoma* ; Middle Aged ; Multiple Myeloma* ; Paraproteinemias ; Plasma Cells ; Proteinuria ; Skin ; Transplants ; Vincristine

BACKGROUND: POEMS syndrome is a rare paraneoplastic syndrome associated with plasma cell dyscrasia. High-dose chemotherapy followed by autologous stem cell transplantation has shown encouraging efficacy in the treatment of patients with POEMS syndrome. However, there are minimal reports on clinical outcomes after autologous stem cell transplantation for patients with advanced disease and very poor performance status. METHODS: We retrospectively evaluated 9 advanced POEMS syndrome patients, who had an Eastern Cooperative Oncology Group performance status score of 3 or 4, and were treated with high-dose melphalan therapy followed by autologous stem cell transplantation from 2004 to 2011. RESULTS: Eight patients achieved initial hematologic response, 4 of whom had complete responses. At a median follow-up of 44 months (range, 8-94 months), 7 patients were alive, with 3-year overall survival rate of 77.8%. There were no hematologic relapses in the survivors. One patient died of disease progression; the other died of pneumonia despite a hematologic response 3 months after autologous stem cell transplantation. All survivors achieved improvement in general performance status and in clinical response. CONCLUSION: High-dose melphalan followed by autologous stem cell transplantation can be considered a valid treatment option even for patients with advanced POEMS syndrome.
Disease Progression ; Drug Therapy ; Follow-Up Studies ; Humans ; Melphalan* ; Paraneoplastic Syndromes ; Paraproteinemias ; Pneumonia ; POEMS Syndrome* ; Recurrence ; Retrospective Studies ; Stem Cell Transplantation* ; Stem Cells* ; Survival Rate ; Survivors

Angioimmunoblastic lymphadenopathy with dysproteinemia(AILD), first described at early seventies, is a clinico-pathologic syndrome which has common features of lymphadenopathy, hepatosplenomegaly, fever, rash and dysproteinemia. It is a lymphoproliferative disease, differs from malignant lymphoma by the high frequency of constitutional symptoms and by the presence of generalized lymph node enlargement, hepatosplenomegaly, cutaneous menifestations and polyclonal hypergammaglobulinemia at the time of clinical onset. Peripheral T cell lymphomas(PTCL) are morphologically and immunologically heterogeneous group of lymphoproliferative disorders that are composed of postthymic lymphocytes. There are group of cases showing features similar to AILD, and these cases are classified as AILD like PTCL. We report a rare case of AILD like PTCL in an 8 year old boy, who visited Seoul National University Hospital with fever, hepatosplenomegaly and cervical lymphadenopathy, and diagnosed as AILD like PTCL by lymph node biopsy. He did not respond to conventional chemotherapy and died 1 year after clinical onset during chemotherapy, showing feature of multiple brain infarction of unknown etiology.
Biopsy ; Brain Infarction ; Child ; Drug Therapy ; Exanthema ; Fever ; Humans ; Hypergammaglobulinemia ; Immunoblastic Lymphadenopathy* ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoma ; Lymphoma, T-Cell, Peripheral* ; Lymphoproliferative Disorders ; Male ; Paraproteinemias ; Seoul

Chronic lymphocytic leukemia (CLL) and multiple myeloma (MM) are both lymphoproliferative disease occurring in different stages of B cell oncogeny. An increased incidence of secondary malignancies in patients with CLL is well recognized, however, the coexistence of both disorders in the same patient was very rare. Furthermore, clonal relationship between these diseases has not been clearly established. We report the occurrence of MM during the course of CLL. A 68-year-old patient was presented with general weakness and bone marrow aspiration showed a hypercellular marrow with 80% mature lymphocytes. At 5 months after diagnosis of CLL, bone marrow of the patient showed increased immature plasma cells. Serum protein electrophoresis showed monoclonal gammopathy and serum immunoelectrophoresis IgG kappa type monoclonality. The patient received six cycles of VAD (vincristine, adriamycin, dexamethasone) chemotherapy, but died of pneumonia and sepsis.
Aged ; Bone Marrow ; Diagnosis ; Doxorubicin ; Drug Therapy ; Electrophoresis ; Humans ; Immunoelectrophoresis ; Immunoglobulin G ; Incidence ; Leukemia, Lymphocytic, Chronic, B-Cell* ; Lymphocytes ; Multiple Myeloma* ; Paraproteinemias ; Plasma Cells ; Pneumonia ; Sepsis

We report a case of 74 year old man presented with generalized lymphadenopathy with hepatosplenomegaly. Lymph node biopsy revealed angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) and most areas of lymph node were replaced with large atypical lymphocytes which were revealed to be T cells by immunohistochemistry. All together, the patient was diagnosed as AILD-like T cell lymphoma with bone marrow involvement. The patient presented monoclonal gammopathy (IgG lambda) without any evidence of plasma cell dyscrasia or secondary amyloidosis. After three cycles of combination chemotherapy including cyclophosphamide, prednisone, vincristine and doxorubicin, the patient achieved partial remission with decreased level of serum and urine monoclonal protein.
Aged ; Amyloidosis ; Biopsy ; Bone Marrow ; Cyclophosphamide ; Doxorubicin ; Drug Therapy, Combination ; Humans ; Immunoblastic Lymphadenopathy ; Immunohistochemistry ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoma, T-Cell* ; Paraproteinemias* ; Prednisone ; T-Lymphocytes ; Vincristine

The coexistence of non-Hodgkin's lymphoma and multiple myeloma is very rare and the coexistence of nasal NK/T-cell lymphoma and multiple myeloma has never been reported in the literature. Recently, a 63-year-old woman was presented with small amount of hematemesis. Bone marrow showed increased immature plasma cell (72.5%). Serum protein electrophoresis showed monoclonal gammopathy (6.5g/dL). Serum immunoelectrophoresis showed IgG and kappa type monoclonality. Microscopic examination of left nasopharynx and inferior turbinate revealed nasal NK/T-cell lymphoma. Six cycles of CHOP (cyclophosphamide, daunomycin, vincristine, prednisone) chemotherapy induced the complete remission of both tumors, but died of cerebral involvement of NK/T-cell lymphoma in 12 months after the diagnosis.
Bone Marrow ; Daunorubicin ; Diagnosis ; Drug Therapy ; Electrophoresis ; Female ; Hematemesis ; Humans ; Immunoelectrophoresis ; Immunoglobulin G ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Middle Aged ; Multiple Myeloma* ; Nasopharynx ; Paraproteinemias ; Plasma Cells ; Turbinates ; Vincristine

An 8-year-old male Austrian Pinscher and a 14-year-old male Golden Retriever were presented for evaluation due to unexplainable high fructosamine values despite euglycemia and epistaxis in combination with polydipsia/polyuria, respectively. Blood analysis revealed severe hyperglobulinemia, hypoalbuminemia and markedly elevated fructosamine concentrations in both dogs. Multiple myeloma with IgA-monoclonal gammopathy was diagnosed by serum and urine electrophoresis including immunodetection with an anti-dog IgA antibody and bone marrow aspirations. Diabetes mellitus was excluded by repeated plasma and urine glucose measurements. Fructosamine values were positively correlated with globulin, but negatively correlated with albumin concentrations. These cases suggest that, as in human patients, monoclonal IgA gammopathy should be considered as a possible differential diagnosis for dogs with high fructosamine concentrations.
Animals ; Blood Proteins/analysis ; Dog Diseases/*blood/drug therapy ; Dogs ; Fructosamine/*blood ; Immunoglobulin A/*metabolism ; Male ; Melphalan/therapeutic use ; Multiple Myeloma/complications/drug therapy/*veterinary ; Myeloablative Agonists/therapeutic use ; Paraproteinemias/blood/complications/drug therapy/*veterinary