Hemophagocytic lymphohistiocytosis: a case series of a Brazilian institution

Abstract

ObjectiveTo describe the clinical and laboratory presentation of hemophagocytic lymphohistiocytosis in children treated at a referral institution.MethodsA 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.ResultsThe 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.ConclusionAs 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.

Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7 years

“…In a vulnerable host, these set off a robust and pathological immune response that ultimately leads to the observed clinical phenotype. Our knowledge of the pathogenesis and clinical course of HLH is in evolution, and much of our understanding derives from the paediatric literature and case reports, with very few larger case series (Majluf-Cruz et al, 1998;Miyahara et al, 2000;Takahashi et al, 2001;Han et al, 2007;Ishii et al, 2007;Machaczka et al, 2011;Park et al, 2012;Nair et al, 2013;Ferreira et al, 2014;Li et al, 2014;Parikh et al, 2014;Rivi ere et al, 2014). Here we describe our experience of diagnosing and treating patients with HLH across 3 large tertiary care centres: Brigham and Women's Hospital, Beth Israel Deaconess Medical Center and Massachusetts General Hospital.…”

Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the Literature

“…Beta2 microglobulin is a strong and independent prognostic factor for overall survival in lymphoma associated hemophagocytic syndrome [17]. Remission in secondary HLH following treatment of underlying disease has been reported in a Brazilian series [18]. For primary HLH, immunochemotherapy can lead to remission but bone marrow transplant is needed for cure [18].…”

“…Remission in secondary HLH following treatment of underlying disease has been reported in a Brazilian series [18]. For primary HLH, immunochemotherapy can lead to remission but bone marrow transplant is needed for cure [18]. …”

Simulação de Déficit Hídrico em Diferentes Genótipos de Feijão pela Diminuição do Potencial Osmótico

“…Foram submetidos os dados à análise de variância e comparação entre as médias pelo teste de Scott-Knott, a 5% de significância, e, então, realizadas as análise de regressão polinomial para as diferentes concentrações de manitol, utilizando o programa SISVAR 14 . Além disso, realizou-se a análise de correlação de Spearman (r) para avaliar o grau de associação entre as doses de manitol e a porcentagem de germinação das sementes.…”