The Varying Clinical Picture of Pure-White Cell Aplasia

Abstract

Pure white cell aplasia (PWCA) is an uncommon cause of neutropenia. The diagnosis is based largely on morphologic criteria. In the bone marrow, granulopoiesis is either completely absent or very much reduced, while erythropoiesis and megakaryocytopoiesis are intact [1]. Clinically, patients with PWCA present with severe neutropenia and recurrent infections. The peripheral smear and cell count show only severe neutropenia. PWCA may be drug-induced [2,3] and cases have also been reported in association with autoimmune diseases [4] and thymomas [5]. Immunologic mechanisms have been demonstrated in some PWCA cases where an inhibiting antibody was found (6). Treatment with thymectomy or drug discontinuation may be clinically useful. Other cases have required immunosuppressive therapies including steroids [6], intravenous immunoglobulin (IVIG) [7], cyclosporine (CsA) [8], or rituximab with CsA [9]. Bone marrow transplantation has also been utilized with some success [10]. We review the literature of PWCA and present two of our cases as well as their responses to treatment.