Hemophagocytic lymphohistiocytosis of indeterminate cause: a fatal adult case

Authors

  • Fernando Peixoto Ferraz de Campos Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Elizabeth In Myung Kim Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Luciana Andréa Avena Smeili Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Patrícia Picciarelli de Lima Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Fabiana Roberto Lima Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Angélica Braz Simões Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Maria Claudia Nogueira Zerbini Faculdade de Medicina - Universidade de São Paulo, São Paulo/SP - Brazil.

DOI:

https://doi.org/10.4322/acr.%25y.36457

Keywords:

Hemophagocytic syndrome, Hemophagocytic lymphohistiocytosis, Macrophage activation, Autopsy.

Abstract

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.

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Author Biographies

  • Fernando Peixoto Ferraz de Campos, Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
    Department of Internal Medicine - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Elizabeth In Myung Kim, Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
    Department of Internal Medicine - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Luciana Andréa Avena Smeili, Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
    Department of Internal Medicine - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Patrícia Picciarelli de Lima, Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
    Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Fabiana Roberto Lima, Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
    Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Angélica Braz Simões, Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
    Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  • Maria Claudia Nogueira Zerbini, Faculdade de Medicina - Universidade de São Paulo, São Paulo/SP - Brazil.
    Department of Pathology - Faculdade de Medicina - Universidade de São Paulo, São Paulo/SP - Brazil.

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Published

2012-06-08

Issue

Section

Article / Autopsy Case Report

How to Cite

Hemophagocytic lymphohistiocytosis of indeterminate cause: a fatal adult case. (2012). Autopsy and Case Reports, 2(2). https://doi.org/10.4322/acr.%y.36457