gamma heavy chain disease

gamma heavy chain disease

Franklin's disease A disorder of older ♂, ranging from fulminant–ie, death in wks to prolonged–lasting 20 yrs; death is the norm in the 1^st yr, often due to infection Clinical Presents as a lymphoproliferation with fever, fatigue, anemia, angioimmunoblastic lymphadenopathy, hepatosplenomegaly, uvular and palatal edema, eosinophilic infiltrates, leukopenia, autoimmune disease, TB, lymphoma Lab ↑ IgG₁; most excrete < 1 g/day of paraprotein, rarely up to 20g/day Treatment Cyclophosphamide, vincristine, prednisone