Acute liver failure and severe hemophagocytosis secondary to parvovirus B19 infection.

Parvovirus infection presenting as severe hemophagocytosis is extremely rare. We report a 13-year-old girl with acute parvovirus infection who had severe hemophagocytosis resulting in severe pancytopenia and hepatic failure.

Hemophagocytic Syndrome Associated with Bilateral Adrenal Gland Tuberculosis

We report a case of a patient who presented with hemophagocytic syndrome (HPS) and adrenal crisis associated with bilateral adrenal gland tuberculosis, and resulted in a poor outcome. A 50-year-old man was transferred to our hospital from a local clinic due to fever, weight loss, and bilateral adrenal masses. Laboratory findings showed leukopenia, mild anemia, and elevated lactate dehydrogenase. Computed tomography (CT) of the abdomen revealed bilateral adrenal masses and hepatosplenomegaly. CT-guided adrenal gland biopsy showed numerous epithelioid cells and infiltration with caseous necrosis consistent with tuberculosis. Bone marrow aspiration and biopsy showed significant hemophagocytosis without evidence of malignancy, hence HPS associated with bilateral adrenal tuberculosis was diagnosed. During anti-tuberculosis treatment the patient showed recurrent hypoglycemia and hypotension. Rapid ACTH stimulation test revealed adrenal insufficiency, and we added corticosteroid treatment. But pancytopenia, especially thrombocytopenia, persisted and repeated bone marrow aspiration showed continued hemophagocytosis. On treatment day 41 multiple organ failure occurred in the patient during anti-tuberculous treatment and steroid replacement.

Síndrome de linfohistiocitosis hemofagocítica: a propósito de un caso / Hemophagocytic lymphohistiocytosis syndrome: apropos of a case

El síndrome de linfohistiocitosis hemofagocítica (LHH), aunque infrecuente en la práctica clínica, resulta de extraordinario interés dado su comportamiento agresivo y mal pronóstico. El objetivo de esta publicación fue exponer, a partir de la experiencia derivada de la observación de un caso, las características clínicas fundamentales: anemia, trombocitopenia y neutropenia unido a fiebre y esplenomegalia. Se discutieron las causas que pueden producir dicho síndrome y entre ellas los linfomas, como sucedió en este caso. Se concluyó que el síndrome de LHH constituye una entidad en específico y que se necesita una búsqueda minuciosa desde el punto de vista etiológico para determinar su causa. Se destacó que este caso fue secundario a un linfoma no Hodking extranodal de células T de alto grado de malignidad con toma renal que llevó a la muerte a la enferma en breve período

Haemophagocytosis in tuberculosis--a case report.

Reactive histiocytosis with hemophagocytosis is most commonly associated with viral infections. Appearance of hemophagocytosis in the tissues is associated with a rapid progression of the underlying disease, and therefore it is important to diagnose and treat the etiological factor at the earliest. Hemophagocytosis is uncommonly associated with tuberculosis and we report one such case.

Hemophagocytic syndrome as the presenting manifestation of visceral leishmaniasis.

Hemophagocytosis in visceral leishmaniasis is rare and usually mild. However, presentation as hemophagocytic syndrome is exceptionally rare. We report the case of a 28 years man who presented with fever, hepatosplenomegaly, pancytopenia, reactive histiocytes and severe hemophagocytosis. Subsequent investigations revealed he had Leishmania donovani infection.

Síndrome hemofagocítico en pediatría / Hemophagocytic syndrome in pediatrics

El síndrome hemofagocítico, también conocido como síndrome de activación del macrófago o histiocitosis reactiva, es una entidad nosológica heterogénea que fue descrita por vez primera hace más de 60 años. Su etiología es aún desconocida, pero se ha relacionado con un descontrol y respuesta exagerada del sistema inmunológico. Tiene una incidencia de 1-2 por cada millón de niños sin predominio de edad o sexo. Las manifestaciones clínicas incluyen la presencia de síndrome febril, hepatoesplenomegalia, alteraciones de la coagulación, disfunción hepática, trastornos neurológicos y falla orgánica múltiple. En los estudios paraclínicos se demuestra pancitopenia y especialmente en el mielograma una franca hemofagocitosis. El tratamiento es variado, dependiendo de la causa desencadenante. El pronóstico también depende directamente del tipo de síndrome hemofagocítico de que se trate. La mortalidad promedio puede ser de 60 por ciento.

Infection associated hemophagocytic syndrome: a report of 50 children.

Fifty children were diagnosed with infection associated hemophagocytic syndrome (IAHS) over the 10 year period from January 1988 through December 1997 at Queen Sirikit National Institute of Child Health, Thailand. Their ages ranged from 2 months to 14 years (mean 4.14 years). There was no difference in sex. Bacterial, mycobacterial, viral, fungal and protozoa were the associated infections in some of these patients. Supportive with specific therapy for the underlying disease was administered aggressively in all patients. Intravenous immuneglobulin (IVIG) was given in 8 patients. Thirty-five patients (70%) died, mostly as a result of coagulopathy with multiple organ failure and opportunistic infections. Two patients developed acute lymphoblastic leukemia 25 days and 3 months after recovering from IAHS.

Reticulohistiocitose multicêntrica / Multicentric reticulohistiocytosis

A reticulohistiocitose multicêntrica (RM) é doença sistêmica proliferativa de histiócitos, de causa desconhecida, caracterizada clinicamente por nódulos cutâneo-mucosos que se distribuem craniocaudalmente de forma decrescente e por lesöes osteoarticulares distintivas. Evolui por surtos que progridem em gravidade por período de seis a oito anos, regredindo espontaneamente mas deixando, em geral, artrite incapacitante e lesöes faciais desfigurantes. Acomete preferencialmente pessoa em torno dos 45 anos de idade, mormente, mulheres caucasianas. Há significativa associaçäo com câncer interno (de 15 a 31 porcento, dependendo da série), sem apresentar com ele paralelismo evolutivo. Os aspectos histopatológicos säo característicos, com denso infiltrado mononuclear contendo as típicas células multinucleadas que apresentam, em seu interior, material PAS-positivo e diastase-resistente, que confere aspecto de vidro moído na coloraçäo pela hematoxilina-eosina. Embora se considere que näo exista tratamento capaz de influir em sua história natural, esquemas recentes com imunomoduladores têm obtido resultados animadores.

Haemophagocytic syndrome

Two fatal cases of haemophagocytic syndrome diagnosed on the basis of autopsy findings at the Queen Elizabeth Hospital, Barbados, are presented. They were both young patients, a male 20 years of age and a female 28 years of age, with common clinical features of severe constitutional symptoms, pharyngeal haemorrhages, pancytopenia, and fever. The female patient had elevated titres to herpes simplex virus indicative of recent infection as well as postmortem evidence of overwhelming mixed bacteria sepsis. In both cases, histopathological studies showed lymphoid depletion and histiocytes displaying haemophagocytosis.