Polyclonal gammopathy related to renal bleeding in a peritoneal dialysis patient / 소아과

Polyclonal gammopathy represents the diffuse activation of B cells and is usually related to inflammation or immune-related diseases. However, the mechanisms leading to polyclonal gammopathy are essentially speculative. Generally, infectious, inflammatory, or various other reactive processes may be indicated by the presence of a broad-based peak or band in the gamma region on serum protein electrophoresis results. A 15-year-old girl, who had been receiving peritoneal dialysis, presented with polyclonal gammopathy and massive gross hematuria. Renal artery embolization was performed, after which the continuous bleeding subsided and albumin-globulin dissociation resolved. This is a rare case of polyclonal gammopathy related to renal bleeding.

A Case of Post-traumatic Necrobiotic Xanthogranuloma of the Orbit

PURPOSE: To investigate the role of nitric oxide (NO) on the migration of cultured human Tenon's capsule PURPOSE: Necrobiotic xanthogranuloma is a very rare disease characterized by the presence of yellow subcutaneous skin lesions and systemic paraproteinemia. We report a case of post traumatic necrobiotic xanthograuloma that presented with a preseptal mass. CASE SUMMARY: A 31-year-old man presented with ptosis and a mass on the left superior orbit that developed after trauma 3 weeks prior to admission. A computed tomography scan showed a contrast-enhancing preseptal mass, and surgical exploration was performed. CONCLUSIONS: Histopathological examination revealed a tumor that had all the major features of necrobiotic xanthogranuloma, including fat necrosis with granulomatous inflammation, giant multinucleated cells, foamy cells, and several lymphoid follicles with germinal centers. Hematologic evaluation revealed polyclonal gammopathy.

Discrimination of Monoclonal Gammopathy Using Immunoassay for Free Light Chains / 대한진단검사의학회지

BACKGROUND: Immunoglobulins exist in the serum, mostly in a union type of heavy and light chains. Free light chain types exist in an extremely small quantity and are useful in the diagnosis and follow up of multiple myeloma, but are also increased in autoimmune diseases such as SLE. The aim of this study was to evaluate the usefulness of the serum free light chain in discriminating between monoclonal and polyclonal gammopathy. METHODS: Between January and June of 2003, we identified 15 patients with monoclonal gammopathy and 12 patients with polyclonal gammopathy on serum protein electrophoresis (SPEP) and immunofixation electrophoresis (IFE). We measured the serum concentration of the free light chain using Beckman Coulter IMMAGE(TM) analyzer with FREELITE(TM) reagents and calculated the kappa/lambda (kappa/lambda) ratio. We also measured the free light chain of 35 healthy controls to establish a reference range. RESULTS: The reference ranges established in this study were 4.97-12.84 mg/L for kappa light chains, 6.71-18.09 mg/L for lambda light chains, and 0.46-1.01 for the kappa/lambda ratio. The free light chains were abnormal in all 27 but 2 patients with polyclonal gammopathy on SPEP. The kappa/lambda ratio was abnormal in 12 of the 15 patients with monoclonal gammopathy and in none of the 12 patients with polyclonal gammopathy. CONCLUSIONS: Our results suggest that the kappa/lambda ratio can be a useful tool to discriminate between monoclonal and polyclonal gammopathy, especially in the case of vague SPEP results, or when monoclonal gammopathy is suspected in SPEP.

A Case of Angioimmunoblastic Lymphadenopathy with Dysproteinemia (AILD) Like Peripheral T Cell Lymphoma / 대한소아혈액종양학회지

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.

A Case of Polymorphic Pemphigoid / 대한피부과학회지

We report herein a case of polymorphic pemphigoid in a 65-year-old woman, who had one-year history of generlized polymorphic eruption with intensely pruritic excoriated vesicobiullous lesions and residual pigmentation. Face and oral mucosa were spared. The skin biopsy specimen showed a subepidermal blister containing many eosinophils. Linear deposition of 1gG and C3 along the basement membrane was noticed on direct irnmunofluorescerice. Polyclonal garnmopathy with a tendency of beta-gamma bridging and increased IgG, IgA and kappachain, was frund on serum protein electrophoresis and immunoelectrophoresis. Skin lesions were well coritrolled by a combined therapy of prednisolone(20 mg/d), dapsone(100 mg/d) and cyclophosphamide(100 mg/d) for 4 months. Direct immunofluorescence performed 11 rnonths after the cnmpletion of the therapy was negative.