Heavy chain disease: clinical case and literature review

Карнабеда О.А., Stupakova Z.V.

Summary. Heavy chain diseases (HCD) are group of chronic B-cell lymphoproliferative disorders that are characterized by production of the pathological monoclonal heavy chain of immunoglobulin (α, μ or γ), incapable to binding the corresponding light chains due to genetic mutations. The clinical manifestations of HCD depend on the isotype of the produced heavy chain and range from an asym­ptomatic course to aggressive lymphoma. In the article we disscussed the issues of pathogenesis, clinical picture and differential diagnostics of different types of HCD and described a clinical case of diagnosis of α-HCD.
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