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Griscelli syndrome: Report of three cases

  • Safiye Göğüş
  • , Meral Topçu
  • , Türkan Küçükali
  • , Zuhal Akçören
  • , Izzet Berkel
  • , Figen Ersoy
  • , Meral Günay
  • , Işil Saatçi
  • Hacettepe University

Research output: Contribution to journalArticlepeer-review

23 Citations (Scopus)

Abstract

The clinical features of three children with Griscelli syndrome and autopsy findings of two are presented. The patients were 5 years, 9 months, and 3 months old, respectively. Clinical features included partial albinism, hepatosplenomegaly, and various neurological symptoms. Light and electron microscopic studies of the skin were compatible with Griscelli syndrome. Postmortem examination of the viscera and central nervous system revealed lymphohistiocytic infiltration with erythrophagocytosis. Bilateral diffuse involvement of the central nervous system, cranial nerve, and spinal cord was detected in both cases..

Original languageEnglish
Pages (from-to)309-319
Number of pages11
JournalFetal and Pediatric Pathology
Volume15
Issue number2
DOIs
Publication statusPublished - 1995

Keywords

  • Griscelli syndrome
  • Hemophagocytosis
  • Lymphohistiocytosis
  • Partial albinism

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