Síndrome hemofagocítico adquirido: reporte de casos de cuatro pacientes adultos tratados con protocolo HLH 94-04 y revisión de la literatura

C, Nicole Beffermann; S, Javier Pilcante; T, Mauricio Ocqueteau; M, Mauricio Sarmiento

doi:10.4067/s0034-98872015000900010

Cited by 4 publications

(6 citation statements)

References 9 publications

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“…[25] This protocol is indicated in all patients younger than 18 years of age who have severe and persistent or reactivated HLH. Although case reports and retrospective studies have shown that the treatment strategy containing etoposide improved the survival of patients with acquired HLH, [36,37] the efficacy of the HLH-2004 protocol in adult patients with HLH still remains to be proved. [24,28] The hepatotoxicity of etoposide makes the treatment of patients with ALF induced by HLH more difficult.…”

Section: Discussionmentioning

confidence: 99%

Acute liver failure caused by hemophagocytic lymphohistiocytosis in adults

et al. 2016

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Background:Hemophagocytic lymphohistiocytosis (HLH) is a rare condition that can be caused by a primary or acquired disorder of uncontrolled immune response. Liver injury is a common complication of HLH; however, HLH presenting as acute liver failure (ALF) has rarely been reported in adults.Case summary:A 34-year-old man was admitted to our hospital with nausea and fatigue persisting for 2 weeks and jaundice for 1 week. He had hyperthermia at the onset of disease. At admission, he had severe liver injury with unknown etiology. The laboratory data showed that he had hyperferritinemia, thrombocytopenia, anemia, hypertriglyceridemia, and hypofibrinogenemia. Finally, a bone marrow biopsy revealed hemophagocytic cells, and he was diagnosed with HLH. The patient was treated with prednisone and plasma exchange. However, the liver function of the patient deteriorated, and he finally died of multiorgan failure.Conclusions:Reports of adult patients with ALF caused by HLH have increased, and HLH should be suspected in patients with ALF of indeterminate cause. Although the efficacy of the treatment strategy recommended by the HLH 2004 remains to be confirmed in adult patients with ALF caused by HLH, early diagnosis and prompt combined treatment with steroids and cyclosporin A or etoposide should be emphasized.

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Section: Discussionmentioning

confidence: 99%

Acute liver failure caused by hemophagocytic lymphohistiocytosis in adults

et al. 2016

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“…Sin embargo, hay casos reportados de adultos con SHF, en quienes se han determinado alteraciones genéticas de manifestación tardía. (6) El SHF se describió inicialmente vinculado a infecciones virales, pero posteriormente se ha relacionado a infecciones por hongos, bacterias y parásitos (particularmente, leishmaniasis). (1) Una revisión de 2197 casos de adultos con SHF identificó que en el 50,5% el desencadenante fue una infección (predominantemente VEB y VIH), 48% malignidad, 44% en contexto de linfoma y 13% enfermedades autoinmunes, principalmente lupus eritematoso sistémico.…”

Section: Discussionunclassified

“…(17) El pronóstico es ominoso con una mortalidad mayor a 90% a 2 meses del diagnóstico. (6) Sin tratamiento la sobrevida es menor a dos meses. (18) El linfoma como causa secundaria, la plaquetopenia, el aumento de la aspartato-aminotransferasa (ASAT) y de la LDH, y la edad mayor a 50 años son elementos de mal pronóstico.…”

Section: Discussionunclassified

Fallo multiorgánico en pacientes portadores del virus de inmunodeficiencia humana, ¿cuándo pensar en Síndrome Hemofagocítico?. A propósito de dos casos clínicos.

Garino

Más

Quiroz

et al. 2021

Rev. urug. med. interna

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“…Se solicitaron precozmente niveles de ferritina y se hizo seguimiento al empeorar el cuadro clínico, lo cual fue de utilidad para el diagnóstico, de la misma manera que en una serie de casos reportados en Chile por el grupo de Beffermann y cols 22 . Esto apoya que se solicite la medición de ferritina durante el estudio de FOD, y en particular en adultos, también debería considerarse el sCD25 por su mayor especificidad en este grupo 29 .…”

Section: Discussionunclassified

“…En Chile no hay una incidencia conocida para esta enfermedad y la información publicada se basa en reportes de casos 19,[20][21][22] .…”

Section: Epidemiologíaunclassified

Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr

B.¹,

L.²,

C.³

et al. 2020

Rev. méd. Chile

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Hemophagocytic syndrome, current diagnostic and therapeutic approachHemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a highly lethal hyper-inflammatory disorder that leads to a storm of cytokines, hemophagocytosis and multiple organ failure. It can be primary, which is inherited, or secondary. In the latter, virus infections are a frequent trigger, predominantly the family of herpes viruses, such as Epstein-Barr virus. An early treatment is recommended. Until recently there was no consensus about the management of secondary cases. The protocols for the treatment of primary HLH were used, which include cytotoxic agents and corticosteroids. We herein review the current diagnostic and therapeutic approach of HLH, based on a case associated with a reactivation of the Epstein-Barr virus in an immunocompetent adolescent. We highlight the importance of suspecting this disease in patients with a persistent inflammatory response state or with a fever of unknown origin, in order to carry out a timely treatment, with the least toxicity, and appropriate to the characteristics of each individual, which is the current therapeutic trend.

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Síndrome hemofagocítico adquirido: reporte de casos de cuatro pacientes adultos tratados con protocolo HLH 94-04 y revisión de la literatura

Abstract: (Rev Med Chile 2015; 143: 1172-1178

Cited by 4 publications

References 9 publications

Acute liver failure caused by hemophagocytic lymphohistiocytosis in adults

Acute liver failure caused by hemophagocytic lymphohistiocytosis in adults

Fallo multiorgánico en pacientes portadores del virus de inmunodeficiencia humana, ¿cuándo pensar en Síndrome Hemofagocítico?. A propósito de dos casos clínicos.

Síndrome hemafagocítico, enfrentamiento diagnóstico y terapéutico actual. Revisión a partir de un caso relacionado a virus Epstein-Barr

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