Pure White Cell Aplasia Associated with Secondary Hemophagocytosis: Experience from the Indus Hospital

Abstract

Pure white cell aplasia (PWCA), also known as agranulocytosis without granulopoiesis, is an uncommon condition that causes neutropenia. If linked with thymomas or medication-induced PWCA, surgical thymectomy or drug cessation may be therapeutically helpful. Here we present the case of an adult 18-year-old male came at The Indus Hospital and Health Network (IHHN) , Karachi in-patient department (IPD) with a complaint of colicky abdominal pain and loose stools for 10 days that were watery in consistency and large in amount, 6-7 episodes per day, for which he was taking flagyl. 5 days later, the patient developed high grade fever (102°F) with on and off chills that regressed on antipyretic. The patient also had decreased appetite and 2-3 episodes of vomiting. He had a history of dengue fever and Posterior Inferior Cerebellar Artery (PICA).Laboratory work-up revealed that he had been suffering from severe neutropenia. His bone marrow biopsy indicated absolute neutropenia with normocellular bone marrow demonstrating virtually total lack of myeloid progenitors, with maintenance of erythroid and megakaryocytic lineages, findings strongly indicative of PWCA with secondary hemophagocytosis.
 Findings of the case highlights the importance of an unusual clinical condition PWCA with hemophagocytosis. The aggressive clinical course of the idiopathic variant necessitates early immunosuppressive treatment.