Case Report Haemophagocytosis in a patient with Crimean– Congo haemorrhagic fever Atahan Cagatay, 1 Mahir Kapmaz, 1 Asli Karadeniz, 1 Seniha Basaran, 1 Mustafa Yenerel, 2 Selim Yavuz, 2 Kenan Midilli, 3 Halit Ozsut, 1 Haluk Eraksoy 1 and Semra Calangu 1 Correspondence Atahan Cagatay atayon@yahoo.com 1 Department of Infectious Diseases and Clinical Microbiology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey 2 Department of Internal Medicine, Division of Hematology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey 3 Department of Microbiology and Clinical Microbiology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey Received 22 August 2006 Accepted 7 May 2007 Crimean–Congo haemorrhagic fever (CCHF) is a severe disease with a case fatality of 2.8 to 80 %. A patient dwelling in an endemic region for CCHF was admitted with fever preceding bleeding diathesis and pancytopenia. Despite no history of tick exposure, CCHF was highly suspected. With an oral ribavirin therapy, clinical and laboratory improvements were obtained. The diagnosis was confirmed by detection of IgM antibody to CCHF virus and positive RT-PCR. Although the main pathogenesis of CCHF infection is not elucidated yet, haemophagocytosis, a symptom rarely reported in viral haemorrhagic fevers, was observed in this case. Haemophagocytosis is suggested to have a role in the development of pancytopenia in CCHF, the mechanism of which still needs to be investigated, probably with cytokine studies. Together with clinical symptoms and patient history, haemophagocytosis may be an indicator for CCHF. Introduction Crimean–Congo haemorrhagic fever (CCHF) is an acute illness affecting multiple organ systems and characterized by extensive ecchymosis, visceral bleeding and hepatic dysfunction; and it has a case-fatality of 2.8 to 80 % (Ergonul et al., 2004; Williams et al., 2000). CCHF was first clinically described in 1944 in Crimea of the former Soviet Union during a large outbreak of over 200 cases. CCHF virus, belonging to the genus Nairovirus (family Bunyaviridae), was identified in 1967 from a patient in Uzbekistan, and was found to be similar to a virus isolated in 1956 in the Congo, hence the name Crimean–Congo (Hoogstraal, 1979; Centers for Disease Control, 1983). CCHF is a potentially fatal fever that has been described in parts of Africa, Asia, eastern Europe and the Middle East (Hoogstraal, 1979). It may be transmitted by the bite of infected ticks, contact with infected animals, or person-to- person transmission by exposure to blood or other infected body fluids (Nabeth et al., 2004). In Turkey, CCHF cases were first published in 2004 (Ergonul et al., 2004; Karti et al., 2004). Clinical cases, however, go back to 2001 (Bakir et al., 2005). Here we report a severe case of CCHF with haemophagocytosis in bone marrow aspiration, in a patient living in an endemic region but without any history of tick exposure. Case report A 26-year-old woman living in a city in north-eastern Anatolia was admitted to a local healthcare setting with fever, frontal headache, malaise, arthralgia and myalgia. Initially she was given symptomatic therapy and sent home. On the seventh day after the first symptoms, she developed nasal and vaginal bleeding (at an expected time for menstruation but much higher amounts than usual). Because thrombocytopenia was noticed, she was referred to our hospital with a differential diagnosis of haemat- ological malignancy. On admission, a single ecchymose at the site of venopuncture, haemorrhagic crusts on her nostril without any active bleeding and a normal body temperature (no fever after the fourth day following initial symptoms) were detected. Laboratory results were found as follows: 3300 white blood cells mm 23 , 12.7 g haemoglobin dl 21 , 7000 platelets mm 23 , 581 IU alanine aminotransfer- ase l 21 , 319 IU aspartate aminotransferase l 21 , 1657 IU lactate dehydrogenase l 21 , 583 IU creatinine kinase l 21 , 245 mg fibrinogen dl 21 , and 1405 ng ferritin ml 21 .A peripheral blood smear revealed reactive lymphocytes, and platelets in decreased number. Bone marrow aspiration to Abbreviation: CCHF, Crimean–Congo haemorrhagic fever. Journal of Medical Microbiology (2007), 56, 1126–1128 DOI 10.1099/jmm.0.46910-0 1126 46910 G 2007 SGM Printed in Great Britain