Hemophagocytic lymphohistiocytosis of indeterminate cause: a fatal adult case

Autopsy And Case Reports

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Início Publicação: 28/02/2011
Periodicidade: Trimestral
Área de Estudo: Medicina

Hemophagocytic lymphohistiocytosis of indeterminate cause: a fatal adult case

Ano: 2012 | Volume: 2 | Número: 2
Autores: F. P. F. Campos, P. P. Lima, F. R. Lima, A. B. Simões, E. I. M. Kim, L. A. A. Smeili, M. C. N. Zerbini
Autor Correspondente: F. P. F. Campos | [email protected]

Palavras-chave: hemophagocytic syndrome; hemophagocytic lymphohistiocytosis; macrophage activation; autopsy.

Resumos Cadastrados

Resumo Inglês:

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon life-threatening
disorder characterized by wide spread non-neoplastic proliferation and
inappropriate activation of mature macrophages resulting in hypercytokinemia.
This uncontrollable and ineffective systemic immune response causes fever,
hepatosplenomegaly, cytopenias and subsequently multiorgan failure. The
authors report a case of a 41-year-old male patient with a 30-day history of
weight loss, fever, icterus, hepatomegaly, and cytopenias. The diagnostic
workup disclosed hypertriglyceridemia, hypofibrinogenemia, and elevated
ferritin. Bone marrow examination and clinical course raised the suspicion of
HLH and treatment was started with high-dose corticosteroids and immune
globulin. The patient underwent multi-organ failure and expired after 58 days of
hospitalization. The autopsy finding included massive bone marrow infiltration
by non-neoplastic histiocytes, many of them showing hemophagocytosis,
which immunohistochemical study revealed diffuse CD68-positive histiocytes,
which were negative for S100 protein. Hemophagocytosis was also observed
in the lungs, lymph nodes and liver. The immediate cause of death was
attributed to a massive intestinal bleeding due to extensive ischemic necrosis
at the duodenum/jejunal transition area.