Hemophagocytic Lymphohistiocytosis in a Patient With Familial Mediterrean Fever and Miliary Tuberculosis: A Case Report.

Abstract

Hemophagocytic Lymphohistiocytosis (HLH) is a lethal complication of several infections especially viral origin. M.Tuberculosis infection can also lead HLH, yet it is an uncommon trigger. Considering role of increased cytokines in HLH, autoinflammatory conditions as Familial Mediterrean Fever (FMF) might contribute to development of it. Nevertheless, possible relationship between FMF and HLH has only suggested in some case reports. We present a case with FMF was admitted to hospital with the complaints of constitutional symptoms and chest pain regarding to recurrent pericarditis. On blood test, pancytopenia and elevated acute phase reactants were seen. FDG Positron Emission Tomography/Computed Tomography (FDG PET/CT) demonstrated positive FDG uptake sites on both right and left surrenal glands, visseral layer of pericard and reactive lymphadenomegalies at multipl mediastinal regions. Bone marrow biopsy revealed hemophagocytosis. Methylprednisolone treatment was initiated. Despite immunosupressive treatment, clinical and biochemical parameters deteriorated thus a thorax CT was executed. Findings were consistent with miliary tuberculosis (TB) infection. M.Tuberculosis was detected in blood culture and bronchoalveolar lavage culture material. Also bone marrow and surrenal biopsy material revealed necrotising caseating granuloma.