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Significant and persistent improvement of thrombocytopenia after splenectomy in an adult with the wiskott‐aldrich syndrome and intra‐cerebral bleeding
Author(s) -
Gaspoz JeanMichel,
Waldvogel Francis,
Cornu Pierre,
Gugler Edouard,
Dayer JeanMichel
Publication year - 1995
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830480308
Subject(s) - wiskott–aldrich syndrome , splenectomy , medicine , complication , surgery , antibiotics , immunodeficiency , pediatrics , spleen , immunology , immune system , biochemistry , chemistry , biology , microbiology and biotechnology , gene
The Wiskott‐Aldrich syndrome is an X‐linked inherited immunodeficiency disorder characterized by thrombocytopenia, recurrent infections and eczema. Its best management option is HLA‐ldentical bone marrow transplantation; when this is not feasible, splenectomy, followed by continuous prophylactic antibiotics, represents the alternative of choice. The present case report relates the excellent outcome of an adult with the Wiskott‐Aldrich syndrome who suffered his first major complication of the disease at age 33 years, an intracerebral hemorrhage. Since an uneventfull splenectomy, thrombocytopenia has significantly improved, and he has remained free of infections for a follow‐up period of 3 years while being treated with prophylactic antibiotics. ©1995 Wiley‐Liss, Inc.