A new case of gamma-heavy chain disease.
The first case of gamma-heavy chain disease described in Spain is here reported. The patient, a 36-year-old woman, presented fever, enlarged regional lymph nodes, and hepatosplenomegaly, without bone marrow abnormalities but with lymphopenia. Serum electrophoresis did not disclose any M-component. The abnormal gamma-chain protein had a alpha2-globulin mobility and was immunochemically related to the Fc fragment. It belonged to the IgG 4 subclass, its molecular weight was about 60,000. Proteinuria was minimal but the electrophoresis of concentrated urine showed a homogeneous peak of alpha2-globulin mobility constituted by the gamma-chain fragment. Biopsy of an axillary lymph node disclosed features of immunoblastic sarcoma. The course was malignant, resulting in death in 8 months.
D000328:Adult; D005260:Female; D006362:Heavy Chain Disease / Q000276:immunology; D006529:Hepatomegaly / Q000276:immunology; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000032:analysis; D007142:Immunoglobulin gamma-Chains / Q000032:analysis; D008206:Lymphatic Diseases / Q000276:immunology; D008231:Lymphopenia / Q000276:immunology; D008970:Molecular Weight; D013163:Splenomegaly / Q000276:immunology
D007143:Immunoglobulin Heavy Chains; D007142:Immunoglobulin gamma-Chains