Lymphoma with Autoimmune Neutropenia and Hepatic Sinusoidal Infiltration: A Syndrome

A relation between lymphoma and autoimmune neutropenia, unlike autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura, has not previously been well documented. We report a patient with a disorder presenting as autoimmune agranulocytosis, splenomegaly, and infiltration of the hepatic sinusoids by lymphocytes. Antineutrophil antibodies were present. Over a 2 1/2-year period, the illness progressed to an aggressive, poorly differentiated lymphocytic lymphoma with terminal liver failure and fibrosis. Peripheral blood lymphocyte markers identified the tumor as a proliferation of T-cells of the helper class. A review of previous literature disclosed other reports of similar patients who had neutropenia, a lymphoproliferative illness, and hepatic disease. Our case is representative of a previously unrecognized syndrome characterized by autoimmune neutropenia in the setting of a lymphoproliferative disorder of T cells, with a predilection for liver involvement.