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Haemophagocytic

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Haemophagocytic syndromes

Clinical manifestation of an increased macrophagic activity with haemophagocytosis.

Aetiology

  • Familial

  • Acquired

    • Infection

      • EBV

      • Bacterial, fungal and parasitic infections

    • Associated with immunodeficiency, haematological malignancies, auto-immune disease

Pathophysiology

  • deregulation of T-lymphocytes

  • excessive production of cytokines

Clinical features

  • Fever

  • Hepatosplenomegaly

  • Lymphadenopathy

  • Skin rash

  • Neurological features

  • Anaemia, lymphopaenia, thrombocytopaenia

  • Hypertriglyceridaemia

  • Hyperferritinaemia

  • Coagulopathy

Diagnosis

Bone marrow: benign histiocytes actively phagocytosing haemopoietic cells

Management

  • Treat underlying disease

  • Cytotoxic therapy may help

 

©Charles Gomersall, October, 2009 unless otherwise stated. The author, editor and The Chinese University of Hong Kong take no responsibility for any adverse event resulting from the use of this webpage.
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