A 11 months old female infant from Portugal, free of family history,
consults for apathy, weight loss, tachycardia, tachypnea, petechiae,
pallor without icterus and hepatoslenomegaly. Seven months earlier,
while being in Portugal, she presented a persistent bluish pimple on
her buttock. Laboratory results showed anemia (35 g/l), leucopenia
(3.3 G/l), thrombocytopenia (13 G/l), impaired coagulation (INR 1.4,
PTT 41 sec.), hyponatremia (124 mmol/l), elevated CRP (139 mg/l),
high ferritin (34.775 μg/l) and high triglycerides (5.22 mmol/l). After
correction of vital parameters, a bone marrow aspiration and biopsy
(BMB) revealed both the etiological diagnosis, namely a visceral
leishmaniasis (VL) as well as one of its potential complications, the
hemophagocytic syndrome (HS). Transfusions of whole blood,
platelets and fresh frozen plasma were immediately started.
Dexamethasone (10 mg/m2) and amphotericin B (3 mg/kg/day) have
also been administrated. Visceral leishmaniasis is caused by a
protozoan (Leishmania donovani) transmitted by the female sandfly. It
is endemic in the Mediterranean basin (including France, Italy,
Spain and Portugal), South America, sub-Saharan Africa as well as
in India and Bangladesh. The parasite infects macrophages and, after
several weeks of incubation, the disease occurs by affection of
bloodlines (anemia, leucopenia, thrombocytopenia),
hepatosplenomegaly, cachexia, gastrointestinal damage. The
complications of the disease may lead to death. Liposomal
amphotericin B is the currently recommended treatment. HS is caused
by the proliferation and activation of macrophages in the marrow in
response to a cytokine storm. It may be of primary cause. When it is
secondary, it may be related to infections such as leishmaniasis.
Patients present with fever and laboratory diagnostic criteria include
cytopenia, hypertriglyceridemia, high ferritin and hemophagocytosis in
the BMB. The treatment consists among other in the administration
of high doses corticosteroids and, in secondary cases, in the
treatment of the underlying cause. In conclusion, the clinical and
biological features of VL may mimic haematological disorders as
leukemia, but an enlargement of the liver and especially of the spleen
should remind in this parasitic infection and its potential fatal
complication, the HS.