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Hemophagocytic lymphohistiocytosis: a case series of a Brazilian institution
Ferreira, Daniela Guimarães Rocha; Rezende, Paulo do Val; Murao, Mitiko; Viana, Marcos Borato; Oliveira, Benigna Maria de.
Afiliación
  • Ferreira, Daniela Guimarães Rocha; Universidade Federal de Minas Gerais. Belo Horizonte. BR
  • Rezende, Paulo do Val; Universidade Federal de Minas Gerais. Belo Horizonte. BR
  • Murao, Mitiko; Universidade Federal de Minas Gerais. Belo Horizonte. BR
  • Viana, Marcos Borato; Universidade Federal de Minas Gerais. Belo Horizonte. BR
  • Oliveira, Benigna Maria de; Universidade Federal de Minas Gerais. Belo Horizonte. BR
Rev. bras. hematol. hemoter ; Rev. bras. hematol. hemoter;36(6): 437-441, Nov-Dec/2014. tab
Article en En | LILACS | ID: lil-731236
Biblioteca responsable: BR408.1
ABSTRACT

Objective:

To describe the clinical and laboratory presentation of hemophagocytic lymphohistiocytosis in children treated at a referral institution.

Methods:

A retrospective descriptive study was carried out of seven children diagnosed with hemophagocytic lymphohistiocytosis between 2010 and 2012. The criteria for diagnosis were those proposed by the Histiocyte Society. When indicated, immunochemotherapy was prescribed according to the HLH94 and HLH2004 protocols of the Histiocyte Society.

Results:

The patients' ages at diagnosis ranged from one month to nine years. All patients had splenomegaly, fever, anemia, thrombocytopenia, hyperferritinemia and hypertriglyceridemia. Bone marrow hemophagocytosis was detected in six patients. In six cases, infectious diseases triggered the syndrome. In two cases, associated with visceral leishmaniasis, remission was achieved after treatment of the underlying infection. Three patients, who had Epstein–Barr-related hemophagocytic lymphohistiocytosis, required treatment with immunochemotherapy. They are alive and in remission; one patient had symptoms of juvenile rheumatoid arthritis and another, who was suspected of having primary hemophagocytic lymphohistiocytosis, entered into remission after bone marrow transplantation. Two deaths (28.6%) occurred in patients with suspected primary hemophagocytic lymphohistiocytosis; one whose clinical picture was triggered by cytomegalovirus infection did not respond to immunochemotherapy and the other died before any specific treatment was provided.

Conclusion:

As reported before, hemophagocytic lymphohistiocytosis has a multifaceted presentation with nonspecific signs and symptoms. In secondary forms, remission may be achieved by treating the underlying disease. In the primary forms, remission may be achieved with immunochemotherapy, but bone marrow transplantation is required for cure...
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Texto completo: 1 Índice: LILACS Asunto principal: Trasplante de Médula Ósea / Linfohistiocitosis Hemofagocítica / Leishmaniasis Visceral Tipo de estudio: Guideline Límite: Child / Child, preschool / Female / Humans / Infant / Male / Newborn País/Región como asunto: America do sul / Brasil Idioma: En Revista: Rev. bras. hematol. hemoter Asunto de la revista: HEMATOLOGIA Año: 2014 Tipo del documento: Article

Texto completo: 1 Índice: LILACS Asunto principal: Trasplante de Médula Ósea / Linfohistiocitosis Hemofagocítica / Leishmaniasis Visceral Tipo de estudio: Guideline Límite: Child / Child, preschool / Female / Humans / Infant / Male / Newborn País/Región como asunto: America do sul / Brasil Idioma: En Revista: Rev. bras. hematol. hemoter Asunto de la revista: HEMATOLOGIA Año: 2014 Tipo del documento: Article