Gamma heavy chain disease (Franklin's disease) consists of a lymphoplasmacytic lymphoma with clonal production of an IgG heavy chain without light chains.
Clinical features:
(1) The disease usually affects older adults with median age of 60 years.
(2) Clinical symptoms include:
(2a) anorexia
(2b) weakness
(2c) weight loss
(2d) recurrent bacterial infections
(3) Clinical findings may include:
(3a) lymphadenopathy
(3b) hepatosplenomegaly
(3c) enlargement of Waldeyer's ring
Laboratory features:
(1) There is a monoclonal increase in IgG without light chains. The IgG consists of a truncated heavy chain that lacks binding sites for light chains.
(2) The patient may have an autoimmune hemolytic disease or autoimmune thrombocytopenia.
(3) The peripheral blood may show eosinophilia, plasma cells or a CLL-like lymphocytosis.
(4) Biopsy of bone marrow or lymph node shows a polymorphous lymphoplasmacytic proliferation.
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