Hemophagocytic lymphohistiocytosis and visceral leishmaniasis in children: case report and systematic review of literature

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.

Haemophagocytic lymphohistiocytosis complicating visceral leishmaniasis in the UK: a case for detailed travel history, a high index of suspicion and timely diagnostics

“…The pathogenesis of sHLH remains unclear. Both the dysregulation of CD8 +T lymphocytes and non-malignant proliferation of macrophages lead to uncontrolled hypercytokinaemia 1 7 8. This, in turn, causes migration of these inflammatory cells and organ infiltration causing cell necrosis and multi-organ failure.…”

“…Attempts to diagnose VL in the context of sHLH are hampered by many challenges. First, microscopy of splenic aspirations have a high specificity, but risk haemorrhage and hence are rarely used in children 7. Second, haemophagocytosis may be seen on the BMA but is not always present and is a non-specific finding 2 7 10.…”

“…First, microscopy of splenic aspirations have a high specificity, but risk haemorrhage and hence are rarely used in children 7. Second, haemophagocytosis may be seen on the BMA but is not always present and is a non-specific finding 2 7 10. Furthermore, the first microscopy of bone marrow does not reveal Leishmania amastigotes in 36%–64% of cases due to the pauci-microbial nature of this infection 1 9–11.…”

“…Furthermore, the first microscopy of bone marrow does not reveal Leishmania amastigotes in 36%–64% of cases due to the pauci-microbial nature of this infection 1 9–11. Repeating BMA leads to an increased diagnostic yield 7 10. Some patients require up to 5 BMAs to reach a diagnosis 10.…”

“…If available, the newer rapid diagnostic anti-rk39 ELISA rapid test has a high sensitivity (97%) and sensitivity (98%) 10 11. The median time to diagnosis is 1–2 months7 8 10 with some diagnoses delayed until 134 days9 or postmortem. The rk39 ELISA rapid test has the capacity to make a difference to patient outcome, as sHLH with untreated VL is invariably fatal 10…”