Extra pulmonary tuberculosis presenting as fever with massive splenomegaly and pancytopenia

Abstract

Disseminated tuberculosis is an important differential diagnosis for fever of unknown origin (FUO) and it can present with hepatosplenomegaly and lymphadenopathy and may have meningitis and with hematological abnormalities including pancytopenia or a leukemoid reaction. We report the case of a 13-year old male who presented with fever, weight loss, pallor and massive splenomegaly with pancytopenia, in whom a bone marrow trephine biopsy showed caseating granulomata, who responded well to antituberculous treatment and has remained healthy on follow up after nine years.

An Unusual Presentation of Disseminated Tuberculosis in South-West Nigeria: A Case Report

“…The clinical presentation is sometimes nonspecific, and this could constitute a diagnostic quagmire. Disseminated TB is a known cause of fever of unknown origin [6,[8][9][10], as also reflected in this patient who had history of fever for about 5 months. Symptoms and signs are variable and may relate to the organ systems affected [9].…”

“…The presence of massive splenomegaly together with the haematologic picture of pancytopenia might have also promoted the consideration of haematologic malignancy like a lymphoma at the initial review of the patient. Haematologic findings in DTB include different types of anemia, leukemoid reaction, pancytopenia, disseminated intravascular coagulation [8][9][10]. A rare, life-threatening condition known as hemophagocytic syndrome could occur and this comprises anemia (100%), neutropenia (65%), thrombocytopenia (91%), pancytopenia (74%) with morphological evidence of macrophage phagocytosis of red cells, granulocyte and platelets [8].…”

“…[8,14]. Explanations for pancytopenia in DTB include hypersplenism, histiocytic hyperplasia, maturation arrest, or infiltration of the bone marrow by caseating or noncaseating granulomas resulting in reversible or irreversible fibrosis [8][9][10].…”

“…The clinical presentation of DTB is variable and could follow a subtle, insidious course and this together with delayed presentation of some of the patients make prompt diagnosis a challenge [5,6,8]. Patients may present with fever of unknown origin, weight loss, anorexia, hepatosplenomegaly and elevated erythrocyte sedimentation rate (ESR) [5,9,10]. Different haematologic abnormalities have also been reported including anemia, neutropenia, lymphopenia, leukemoid reaction but rarely pancytopenia [5,8,9].…”

Microbe-Induced Inflammatory Signals Triggering Acquired Bone Marrow Failure Syndromes

“…This may be due to granulomatous inflammation and focal necrosis in the BM ( 92 ). Although not clearly defined, other mechanisms that may lead to pancytopenia in these patients include histiocytic hyperplasia, HSC maturation arrest, and hypersplenism ( 92 , 93 ).…”