Disseminated Tuberculosis with Hemophagocytic Lymphohistiocytosis (HLH)

Abstract 4686

A 38 year old woman presented with high grade fever and jaundice for one month. Patient also had reduced appetite and loss of weight for the same period. On examination, patient had significant pallor, icterus and pedal edema. Ultrasonogram showed enlarged liver (20 cms) and enlarged spleen (17.2 cms). Patient was empirically treated for malaria. Her subsequent investigations revealed Hb 35g/dl, total leucocyte count 2×10⁹/l, differential leucocyte count - neutro 82%, lympho 18%, platelet count − 59×10⁹/l, and red cell indices were as follows: MCV 71.6 fL, MCH 21.6 pg/cell, MCHC 30.2 g/dl. Her reticulocyte count was 0.5%. Peripheral blood smear showed pancytopenia with moderate anisopoikilocytosis. Her total bilirubin was 4.2 mg/dl and serum ferritin was found to be 1720 μg/L. In view of pancytopenia and non-response to antimalarials, patient was treated in line of septicemia with piperacillin and tazobactam, and simultaneously a bone marrow biopsy was performed. Bone marrow biopsy subsequently revealed a hypercellular marrow with erythroid hyperplasia. Number of macrophages was increased with some showing ingested red cells (hemophagocytosis) within them. In view of fever, splenomegaly, pancytopenia, hemophagocytosis and hyperferritinemia, a diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made as per HLH 2004 diagnostic criteria. Bone marrow also revealed multifocal epithelial granulomas with caseation, pointing the etiology to that of disseminated tuberculosis. Patient expired before any anti-tuberculous treatment could be instituted.

Causes of HLH are broadly malignancy, collagen diseases and infections. Though malignancy and collagen diseases are common causes in the Western countries, tuberculosis is an important cause in a tropical country like India.