An update on renal involvement in hemophagocytic syndrome (macrophage activation syndrome)

Haydarali Esmaili, Elmira Mostafidi, Bahareh Mehramuz, Mohammadreza Ardalan, Mohammadali Mohajel Shoja

Research output: Contribution to journalArticlepeer-review

12 Scopus citations


Context: Hemophagocytic syndrome (HPS) is mainly characterized by massive infiltration of bone marrow by activated macrophages and often presents with pancytopenia. Thrombotic microangiopathy (TMA) is also present with thrombocytopenia and renal involvement. Both conditions could coexist with each other and complicate the condition. Evidence Acquisition: Directory of Open Access Journals (DOAJ), EMBASE, Google Scholar, PubMed, EBSCO, and Web of Science with keywords relevant to; Hemophagocytic syndrome, macrophage activation syndrome, interferon-gamma and thrombotic microangiopathy, have been searched. Results: Viral infection, rheumatologic disease and malignancies are the main underlying causes for secondary HPS. calcineurin inhibitors and viral infections are also the main underlying causes of TMA in transplant recipients. In this review, we discussed a 39-year-old male who presented with pancytopenia and renal allograft dysfunction. With the diagnosis of HPS induced TMA his renal condition and pancytopenia improved after receiving intravenous immunoglobulin (IVIG) and plasmapheresis therapy. Conclusions: HPS is an increasingly recognized disorder in the realm of different medical specialties. Renal involvement complicates the clinical picture of the disease, and this condition even is more complex in renal transplant recipients. We should consider the possibility of HPS in any renal transplant recipient with pancytopenia and allograft dysfunction. The combination of HPS with TMA future increases the complexity of the situation.

Original languageEnglish (US)
Pages (from-to)8-14
Number of pages7
JournalJournal of Nephropathology
Issue number1
StatePublished - Jan 1 2016
Externally publishedYes


  • Hemophagocytic syndrome
  • Interferon-gamma
  • Macrophage activation syndrome
  • Thrombotic microangiopathy

ASJC Scopus subject areas

  • Nephrology


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