Visceral leishmaniasis associated with macrophage activation syndrome: Case report and literature review

Abstract

Background The combination of visceral leishmaniasis (VL) and macrophage activation syndrome (MAS) makes the diagnosis difficult due to their similar clinical presentation, with a poor prognosis especially since the treatment is still poorly codified. We report the case of a 17-month-old female patient from Berkane, presenting for a 3 months history of anarchic fever with anemic syndrome made up of pallor and hemorrhagic syndrome made up of epistaxis. Physical examination revealed a temperature of 39 ° C, lower limbsedema, paleness of skin and mucous membranes, gingival petechiae, bleached hair, and hepatosplenomegaly. Case presentation The complete blood count showed pancytopenia with deep aregenerative normochromic normocytic anemia at 3 g/dL, leukocytes were at 4860/mm 3 with neutropenia at 680/mm 3 and thrombocytopenia at 12.000/mm 3 , the blood smear was without abnormality. These anomalies were associated with a hypoalbunemia, hypertriglyceridemia, hyperferritinemia, lactate dehydrogenase (LDH) level was at 337 IU/L, low prothrombin time (PT) at 56 % and fibrinogen level at 1 g/L. The direct Coombs test was positive. Examination of the myelogram revealed the presence of leishmania bodies and figures of hemophagocytosis. A diagnosis of visceral leishmaniasis associated with MAS was made. The patient was put on liposomal amphotericin B and corticosteroid therapy with good clinical and biological evolution and good therapeutic tolerance. Conclusion The association of VL and MAS remains rare and should be evoked even in non-endemic areas since late diagnosis worsens the prognosis and may even be responsible for the death of patients despite an aggressive treatment.

Macrophage activation syndrome in a Sudanese child: a case report from Sudan

“…Another reported case is a case of a 17-month-old female patient born to consanguineous parents who presented with a combination of Visceral leishmaniasis and MAS 17 . Diagnosing MAS in this patient was challenging because of the similarity of these two conditions.…”

“…Bone marrow examination revealed features of hemophagocytosis and the diagnosis of visceral leishmaniasis with MAS was made. She was managed with liposomal amphotericin B coupled with corticosteroid therapy resulting in a favourable outcome 17 .…”