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

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 thymectomyor 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.

CD8+ Mycosis Fungoides in Peripheral Blood; A Rare Finding

Cutaneous T cell lymphomas are mature lymphomas of T lymphocyte presenting with skin lesions and/or systemic manifestations. Majority of these cases show CD4+ phenotype and are classified as Mycosis Fungoides (MF)/Sezary syndrome (SS) spectrum.Here we present a case of 74-year-old male patient, having no known comorbid, presented in Dr. Ziauddin Hospital, Karachi OPD with complains of generalized skin lesions, itching for 2 years, generalized weakness for 4 months and no lymphadenopathy or visceromegaly. CBC showed absolute lymphocytosis and absolute eosinophilia. Some lymphocytes exhibited cerebriform nuclei. CT scan neck, chest and abdomen showed bilateral enlarged superficial inguinal lymph nodes and multiple enlarged bilateral axillary lymph nodes. Skin biopsy was inconclusive. Immunophenotyping of peripheral blood was then performed which showed an aberrant T cell population showing positivity for CD3, CD8, CD2, CD25 and negative for CD4, CD45, CD5, CD30, CD56 with variable expression of CD7. Case was finalized as CD8+ Mycosis Fungoides with peripheral blood involvement.These findings are very rare and highlight the importance of integrated approach to clinical course, morphological findings and other ancillary tests to be used in correlation with each other. These findings also highlight the diversity present in T cell malignancies in terms of immunophenotype.