Hemophagocytic lymphohistiocytosis associated with visceral leishmaniasis. Review of cases reported

Introduction: Leishmaniasis is a tropical and subtropical disease highly reported in Southeast Asia, East Africa, Latin America, and the Mediterranean basin, with an incidence of two million new cases by year and 500,000 cases of visceral leishmaniasis. One of the more severe and rare complications of visceral leishmaniasis is hemophagocytic lymphohistiocytosis. Objective: To describe the clinical characteristics of hemophagocytic lymphohistiocytosis associated with visceral leishmaniasis Methods: We performed a literature review based on the case reports indexed in MEDLINE/PubMed. Results: Twenty-five cases were included; 52 percent under two years of age. All cases presented splenomegaly and 84 percent hepatomegaly. Cytopenias were described in all patients: 100 prcent thrombocytopenia, 96 percent anemia, and 84 percent leukopenia or neutropenia. Hypertriglyceridemia and hypofibrinogenemia were found in 68 percent and 32 percent, respectively, and hyperferritinemia in 80 percent. Additionally, hemophagocytosis was documented in 84 percent, with Leishmania detection in 92 percent. All patients were treated against Leishmania: 80% with liposomal amphotericin B. regarding the treatment for hemophagocytic lymphohistiocytosis; corticosteroid were used in 36 percent, endovenous immunoglobulin in 28 percent, cyclosporine in 28 prcent and etoposide in 16 percent The complications reported included gastrointestinal hemorrhage (8 percent), disseminated intravascular coagulation (8 percent), autoimmune hemolytic anemia (12 percent), multiple-organ dysfunction/septic shock (12 prcent), petechial rash (16 percent), and four patients deceased. Variables such as fever (p=0.031), hemoglobin level (p=0.031), platelet count (p=0.0048), and ferritin (p=0.0072) were associated with mortality Conclusions: During visceral leishmaniasis, the hemophagocytic syndrome is a rare condition that mainly affects pediatric patients, but with excellent outcomes using liposomal amphotericin B. However, there is a lack of strong evidence to make a recommendation(AU)

Introducción: La leishmaniasis es una enfermedad tropical y subtropical con una elevada incidencia, dos millones de casos nuevos por año y 500 000 de leishmaniasis visceral. La linfohistiocitosis hemofagocítica es una complicación grave y rara de la leishmaniasis visceral. Objetivo: Describir las características clínicas de la linfohistiocitosis hemofagocítica asociada con leishmaniasis visceral. Métodos: Se realizó una revisión bibliográfica basada en los informes de casos indexados en MEDLINE/PubMed. Se identificaron 34 publicaciones; después de analizarlas en función de los criterios de inclusión se trabajó con 22 trabajos. Resultados: En los trabajos incluidos se informaron 25 casos; el 52 por ciento fueron pacientes menores de 2 años. Todos presentaron esplenomegalia y 84 por ciento hepatomegalia. Se describieron citopenias en todos los pacientes: 100 por ciento trombocitopenia, 96 por ciento anemia y 84 por ciento leucopenia o neutropenia. Se encontró hipertrigliceridemia e hipofibrinogenemia en 68 por ciento y 32 por ciento, respectivamente, e hiperferritinemia en 80 por ciento. Todos los pacientes fueron tratados contra leishmania, 80 por ciento con anfotericina B liposomal. Las complicaciones incluyeron: hemorragia gastrointestinal, coagulación intravascular diseminada, anemia hemolítica autoinmune, falla multiorgánica/shock séptico, erupción petequial y cuatro pacientes fallecieron. Conclusiones: En la leishmaniasis visceral, el síndrome hemofagocítico es una afección poco frecuente que afecta principalmente a pacientes pediátricos. Para el tratamiento, usando la anfotericina B liposomal se obtienen excelentes resultados; sin embargo, la evidencia es insuficiente para hacer una recomendación(AU)

Viral markers in patients with hemophagocytosis: A prospective study in a tertiary care hospital.

Background : Hemophagocytic syndrome (HPS) is a rare clinicopathological condition characterized by the activation of macrophages with prominent hemophagocytosis in bone marrow and other reticulo-endothelial systems. HPS can be familial or secondary to infections including viruses. Aim : To study the viral markers in patients with HPS. Materials and Methods : Serum samples of patients with HPS and control group were screened for anti EBV VCA IgM, and IgG, anti-Parvo B19 IgM, and anti-CMV IgM antibodies using commercially available ELISA kits and CMV and ParvoB19 DNA by polymerase chain reaction (PCR). Results and Discussion : The present prospective study reports the profile of viral markers in HPS cases from north India. Among the 14 HPS cases 43% (6/14) were positive for at least one viral marker tested, of which EBV was found to be the most prevalent (3/6: 50%) followed by parvovirus B19(2/6: 33%) and cytomegalovirus (1/6: 17%). Mortality was noted in 33% of virus associated HPS patients. Our study highlights the higher association of Epstein-Barr virus (EBV) with HPS as compared to other viruses along with higher rate of mortality in both parvovirus B 19 and EBV associated HPS.

Reactive haemophagocytic lymphohistiocytosis.

We present a series of five cases diagnosed and treated as reactive haemophagocytic lymphohistiocytosis (HLH) in three tertiary referral centers of Kolkata, within a time frame of 3 months. The initial presentations were very variable, the most prominent clinical feature being - acute renal failure in the first patient, convulsions in the second, encephalopathy the third, marked cervical lymphadenopathy in the fourth and polyserositis in the fifth. All had a history of prolonged fever preceding admission and hepatosplenomegaly on examination. Investigations revealed multi-organ involvement with pancytopenia;- haemophagocytosis was eventually diagnosed by bone marrow examination. These cases highlight the diagnostic challenge posed by infection associated haemophagocytosis and the need for maintaining a high index of suspicion to promptly diagnose and treat this potentially life threatening condition.