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A New Case of Gamma-Heavy Chain Disease

A New Case of Gamma-Heavy Chain Disease The first case of γ-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 γ-chain protein had an α<sub>2</sub>-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 γ-globulin mobility constituted by the γ-chain fragment. Biopsy of an axillary lymph node disclosed features of immunoblastic sarcoma. The course was malignant, resulting in death in 8 months. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Acta Haematologica Karger

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Publisher
Karger
Copyright
© 1978 S. Karger AG, Basel
ISSN
0001-5792
eISSN
1421-9662
DOI
10.1159/000207781
Publisher site
See Article on Publisher Site

Abstract

The first case of γ-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 γ-chain protein had an α<sub>2</sub>-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 γ-globulin mobility constituted by the γ-chain fragment. Biopsy of an axillary lymph node disclosed features of immunoblastic sarcoma. The course was malignant, resulting in death in 8 months.

Journal

Acta HaematologicaKarger

Published: Jan 1, 1978

Keywords: Immunoglobulins; Immunoblastic sarcoma; Malignant lymphoma; Monoclonal gammopathy; Franklin’s disease; γ-Heavy chain disease; γ-Heavy chain disease proteins

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