A Case of Common Variable Immunodeficiency Associated with Marked Granulocytopenia

Abstract

A case of common variable immunodeficiency (CVID) associated with drug-induced marked granulocytopenia is described.
A 17-year-old boy referred to our hospital for further examination on agammaglobulinemia. He had a history of recurrent infections since 9 years of age. Family history was non-contributory. Laboratory studies on admission disclosed marked hypogammaglobulinemia of all classes and depressed antibody titers against bacteria, virus and blood groups, but normal responses in vivo skin test and in vitro lymphocyte stimulation with mitogens, normal numbers of peripheral T and B-cells, normal morphology and in vitro function of neutrophils, normal complement activities, and intrinsic defect of B-cells, which were demonstrated by using coculture study of T and B-cells for secreting immunoglobulins in vitro. A diagnosis of CVID was made. Gammaglobulin was given intravenously and two days after antibiotics treatment with sulbenicillin sodium (SB-PC) and gentamicin (GM) a marked leukopenia with 7% of immature neutrophils was found. For 6 days thereafter neutrophils completely disappeared from peripheral blood, and drug-induced granulocytopenia was suspected. Bone marrow showed maturation arrest of granulopoiesis. SB-PC and GM were stopped. Over the next 3 days leukocytecounts gradually returned to normal with transient leukemoid reaction and without any bacterial infections. Antiteukocyte agglutinating antibodies could not be demonstrated.