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

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Cerme E., Oztas M., Balkan I. I., Aktas Cetin E., Ugurlu S.

Modern rheumatology case reports, vol.6, pp.140-144, 2022 (ESCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 6
  • Publication Date: 2022
  • Doi Number: 10.1093/mrcr/rxab026
  • Journal Name: Modern rheumatology case reports
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus
  • Page Numbers: pp.140-144
  • Keywords: Cytokine storm, familial Mediterranean fever, haemophagocytic lymphohistiocytosis, macrophage activation syndrome, tuberculosis, NECROSIS-FACTOR ANTAGONISTS, INTERLEUKIN-18 LEVELS, CYTOKINES, RISK
  • Istanbul University Affiliated: Yes


Haemophagocytic lymphohistiocytosis (HLH) is a lethal complication of several infections, especially viral origin. Mycobacterium tuberculosis infection can also lead to HLH, yet it is an uncommon trigger. Considering the role of increased cytokines in HLH, autoinflammatory conditions, such as familial Mediterranean fever (FMF), might contribute to its development. Nevertheless, the possible relationship between FMF and HLH has been suggested only in some case reports. We present a case of FMF who admitted to the hospital with consitutional symptoms and chest pain regarding to recurrent pericarditis. On a blood test, pancytopenia and elevated acute phase reactants were seen. Fluorine-18 fluorodeoxyglucose (FDG) positron emission tomography/computed tomography demonstrated positive FDG uptake sites on both the right and left surrenal glands, the visceral layer of pericard, and reactive lymphadenomegalies at multiple mediastinal regions. Bone marrow biopsy revealed haemophagocytosis. Methylprednisolone treatment was initiated. Despite immunosuppressive treatment, clinical and biochemical parameters deteriorated; thus, a thorax computed tomography was executed. Findings were consistent with miliary tuberculosis infection. M. tuberculosis was detected in blood culture and bronchoalveolar lavage culture material. Also, bone marrow and surrenal biopsy material revealed necrotising caseating granuloma.