Acquired idiopathic pure white cell aplasia: A rare cause of agranulocytosis

Abstract

Pure white cell aplasia (PWCA) is an exceedingly rare hematological disease of unknown origin exemplified by agranulocytosis, virtually lack of all granulocytic spectrums (from myeloblast to neutrophils) in the bone marrow with normal erythroid and megakaryocytic lineages. It has been associated with underlying autoimmune disorders, thymomas, chronic lymphocytic leukemia and drug induced or may be idiopathic. Here we report the case of a patient with this rare finding, who was admitted to our hospital with septicemia having agranulocytosis, multiple oral candidiasis, perianal abscess and right arm cellulitis. Bone marrow examination revealed absolute absence of all myeloid precursors cells. All diagnostic workup for secondary underlying cause were found to be negative and diagnosed as idiopathic PWCA. Immunosuppression with methylprednisolone pulse therapy was failed to induce neutrophils recovery. PWCA is a benign disorder, but have serious consequences due to threatening febrile neutropenia, disseminated fungal infection and septicemia. Needs urgent management depending upon underlying etiology.

Keywords

• Agranulocytosis
• idiopathic
• pure white cell aplasia