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Fever and Blonde Skin

  • Javad Ghaffari
Chapter

Abstract

  • Abnormal large granules in several cell types, including granulocytes, melanocytes, NK cells, and platelets, as well as decreased bactericidal activity of neutrophils are among hematologic findings of Chediak-Higashi syndrome (CHS)

  • About 50–85% of patients with CHS eventually enter an accelerated phase, manifested by fever, lymphadenopathy, anemia, jaundice, neutropenia, thrombocytopenia, and widespread lymphohistiocytic organ infiltrates

  • The Griscelli-syndrome type 2 should be considered as an alternative diagnosis in patients with mild skin hypopigmentation and silvery gray hair, but platelet function is normal in these patients

  • The distinction between CHS and Hermansky-Pudlak syndrome type 2 depends on identifying giant intracellular granules within neutrophils of individuals with CHS

Keywords

Chediak-Higashi syndrome Hermansky-Pudlak syndrome Griscelli-syndrome 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Javad Ghaffari
    • 1
  1. 1.Mazandaran University of Medical SciencesSariIran

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