Immunoblastic lymphoma involving the bone marrow in a patient with alpha chain disease. Clinical and immunoelectron...

A patient is reported who had disseminated immunoblastic proliferation that emerged during the course of alpha chain disease. This proliferation was characterized by overt marrow invasion together with osseous and neurologic manifestations. On immunoelectron microscopic study, the malignant immunoblasts displayed varying degrees of cytoplasmic maturation and constituted a morphologic spectrum of alpha-chain-synthesizing cells, ranging from immature blasts without endoplasmic reticulum development to relatively mature plasmablasts; alpha chain was not expressed at the surface of these cells. The general features of the overt malignant stage of alpha chain disease are reviewed in reference to this unusual case. The implications of the cellular findings are discussed with regard to the maturation stage of malignant immunoblasts.