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Objective: Hemophagocytic lymphohistiocytosis syndrome (HLHS) is characterized by an immunological hyperactivation of cytotoxic T cells, natural killer cells, and macrophages, leading to the secretion of proinflammatory cytokines. HLHS associated with Visceral Leishmaniasis might be difficult to diagnose once symptoms are similar, resulting in the death of untreated patients. Our aim is to describe a series of cases of Visceral Leishmaniasis with HLHS admitted to a referral hospital for infectious diseases. Case description: All 115 cases of Visceral Leishmaniasis referred to a referral center for pediatric infectious diseases were reviewed to identify the cases of HLHS. Five cases (4.5%) were confirmed with HLHS and they presented fever, splenomegaly, cytopenia, hypertriglyceridemia or hypofibrinogenemia, increased ferritin and hemophagocytosis in the bone marrow. Comments: It important to rule out HLHS in children with infectious diseases that do not respond adequately to treatment or in patients with severe symptoms, especially in leishmaniasis endemic areas. [ABSTRACT FROM AUTHOR]

Visceral leishmaniasis (VL) is transmitted to humans by the bite of sandflies. VL is an endemic parasitic disease which is characterized by fever, splenomegaly and pancytopenia in most of the cases. Delays in diagnosis of VL are common because of nonspecific symptoms and variable incubation time. Specific serology and polymerase chain reaction are useful for the diagnosis. Anti- K39 strip test is highly sensitive. Splenic multifocal hypoechoic nodules should be evaluated for VL with clinical and laboratory features in patient. Herein we report a twenty-two month-old female infant with diagnosis of VL who presented with recurrent fever, pancytopenia and ultrasonography revealed enlarged spleen with multifocal hypoechoic nodules. We considered that splenic nodules can help to diagnosis of VL in children as literature case reports support to this situation. [ABSTRACT FROM AUTHOR]

Abstract: Aims of the study. – First, to describe liposomal amphotericin B (AmBisome®) use as first line treatment of pediatric visceral leishmaniasis and secondly, to evaluate the incidence of the disease in southern France (Provence – Alpes – Côte d''Azur – Corse). Material and methods. – Retrospective chart review of children referred for visceral leishmaniasis from 1996 to 2003. Results. – Thirty-two children under 15 years of age and suffering from visceral leishmaniasis were treated with liposomal amphotericin B as first line treatment. Clinical and biological features were usual: age <5 years, no immunodeficiency, spleen enlargement and fever, cytopenia. In this population, treatment effectiveness was evaluated to 97% (one relapse). Under treatment, patients quickly improved. Drug regimens varied from 18 to 24 mg/kg (day 1 to 5, and day 10). Four other children were not treated with first-line liposomal amphotericin B during the period. Thus, the incidence of pediatric visceral leishmaniasis was evaluated to be 0.61/100,000 children <15 years/year in the region (2.74 in the Alpes-Maritimes department, French Riviera, and 0.6 in the Bouches-du-Rhône department, Marseilles area). Conclusion. – Liposomal amphotericin B treatment is usual for children referred for visceral leishmaniasis in this region. This treatment may be approved regarding the high level of effectiveness and the low number of adverse events. A two days drug regimen with 20 mg/kg should be evaluated. Moreover, the incidence of the pediatric visceral leishmaniasis in southern France is decreasing, but local variations may be observed. [Copyright &y& Elsevier]