Jm. Gaspoz et al., SIGNIFICANT AND PERSISTENT IMPROVEMENT OF THROMBOCYTOPENIA AFTER SPLENECTOMY IN AN ADULT WITH THE WISKOTT-ALDRICH SYNDROME AND INTRACEREBRAL BLEEDING, American journal of hematology, 48(3), 1995, pp. 182-185
The Wiskott-Aldrich syndrome is an X-linked inherited immunodeficiency
disorder characterized by thrombocytopenia, recurrent infections and
eczema, Its best management option is HLA-identical bone marrow transp
lantation; when this is not feasible, splenectomy, followed by continu
ous prophylactic antibiotics, represents the alternative of choice. Th
e present case report relates the excellent outcome of an adult with t
he 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 yea
rs while being treated with prophylactic antibiotics. (C) 1995 Wiley-L
iss, Inc.