Abstract
Hemophagocytic lymphohistiocytosis is a potentially fatal disorder resulting from excessive activation and non-malignant proliferation of T lymphocytes and macrophages. Neoplasms, autoimmune disorders and systemic infections can cause secondary hemophagocytic syndrome. The association of hemophagocytic syndrome and visceral leishmaniasis is rarely found in childhood. We report a case of an infant affected by hemophagocytic lymphohistiocytosis secondary to visceral leishamniasis and describe all cases of hemophagocytic syndrome associated with visceral leishamniasis in childhood reported in literature, focusing on clinical manifestation, diagnosis and treatment.
Highlights
Hemophagocytic lymphohistiocytosis (HLH), known as hemophagocytic syndrome, is a clinicalpathological condition characterized by uncontrolled and non-malignant proliferation of macrophages and T lymphocytes with cytokine overproduction
HLH secondary to Visceral leishamniasis (VL) is very rare in childhood and the overlap in the clinical characteristics of VL and HLH can be a diagnostic challenge for physicians managing HLH.We describe the case of an infant presenting HLH secondary to VL
All cases of hemophagocytic syndrome related to VL in childhood described in literature to date are reviewed
Summary
Hemophagocytic lymphohistiocytosis (HLH), known as hemophagocytic syndrome, is a clinicalpathological condition characterized by uncontrolled and non-malignant proliferation of macrophages and T lymphocytes with cytokine overproduction. Because of the presence of erythrophagocytosis to BMA, hyperferritinemia, pancytopenia, hypertriglyceridemia, hypofibrinogenemia and splenomegaly, and HLH diagnosis were performed. Treatment with corticosteroids and etoposide (a total of 9 doses) was administered, but elevated ferritinemia and severe hepatosplenomegaly persisted. Serological tests for Epstein-Barr Virus (EBV), Cytomegalovirus (CMV), Hepatitis viruses, HIV and Leishmania were performed and proved to be Scalzone et al – Hemophagocytic lymphohistiocytosis and leishmaniasis. At 3.5 months after admission, because of deterioration of the overall conditions, a second BMA was performed which revealed some inclusive bodies inside the phagocytic cells resembling Leishmania amastigotes. The patient was found to be well, at a follow-up examination performed 4 months after completion of the treatment. Hematological examination revealed a progressive decrease in ferritinemia and abdominal ultrasound that indicated complete regression of splenomegaly
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