Bilateral hand squamous-cells carcinoma in patient affected with non-Hodgkin lymphoma. Case report and literature review.

INTRODUCTION

Crimean-Congo haemorrhagic fever is an acute, often severe haemorrhagic disease caused by the homonymous virus. On the other hand, severe life-threatening secondary haemophagocytic lymphohistiocytosis is a rare entity usually associated with underlying haematological malignancies, infectious or autoimmune diseases. Herein, we report a fatal case of severe secondary haemophagocytic lymphohistiocytosis due to the abovementioned virus but also a second case of Crimean-Congo haemorrhagic fever with virus-induced threatening haemophagocytic lymphohistiocytosis in an internist who was responsible for the patient's management.

CASE DESCRIPTION: A 72-year-old stockbreeder progressed to fatal secondary haemophagocytic lymphohistiocytosis due to Crimean-Congo haemorrhagic fever virus (fever, hepatosplenomegaly, severe cytopenias, low fibrinogen, hypertriglyceridemia and extremely high ferritin levels), despite aggressive supportive measures and specific treatment with corticosteroids and intravenous γ-immunoglobulin. The second case was a 54-year-old internist who acquired the Crimean-Congo haemorrhagic fever virus through mucocutaneous exposure to the patient's blood, recovering fully with prompt double treatment against active viral replication and early stage haemophagocytic lymphohistiocytosis.

DISCUSSION: Crimean-Congo haemorrhagic fever virus is a rare cause of secondary haemophagocytic lymphohistiocytosis with an unfavourable outcome if the patient has a rapidly progressing course as in our first case. Clinicians should maintain a high level of clinical suspicion in the appropriate clinical setting since prompt initiation of treatment for secondary haemophagocytic lymphohistiocytosis as in the second case, can be lifesaving.

CONCLUSION

These cases emphasize that Crimean-Congo haemorrhagic fever virus can be a cause of severe life-threatening haemophagocytic lymphohistiocytosis even in non-endemic areas, while stringent infection control to prevent secondary healthcare-associated transmission is of the utmost importance.

LEARNING POINTS: Crimean-Congo haemorrhagic fever virus can be a rare cause of severe life-threatening haemophagocytic lymphohistiocytosis syndrome even in non-endemic areasClinical suspicion for haemophagocytic lymphohistiocytosis due to Crimean-Congo haemorrhagic fever virus should be high in the appropriate clinical setting as prompt initiation of therapy can be lifesavingIt is important to apply secondary prevention of healthcare-associated transmission of Crimean-Congo haemorrhagic fever.