Hemophilia and thrombocytopenia in a patient with impaired cellular immunity

✍ Scribed by Zeitlhuber, U. ;Haschke, F. ;P�sp�k, R. ;Lechner, K. ;Knapp, W. ;Imbach, P.

• Book ID: 104720804
• Publisher: Springer-Verlag
• Year: 1984
• Weight: 159 KB
• Volume: 48
• Category: Article
• ISSN: 1432-0584
• DOI: 10.1007/bf00319970

✦ Synopsis

ITP in hemophiliacs may produce severe bleeding complications. We here report on an eight-year-old boy suffering from severe hemophilia A, who developed ITP and an acquired impaired immune function similar to AIDS. Steroid therapy reverted the thrombocyte count to normal, however it had to be discontinued because of a severe Cushing syndrome. The thrombocytopenia also responded to IgG-therapy and the patient is treated with a long term schedule according to Imbach. It is of interest that the impaired T-helper/T-suppressor cell ratio (0.45) improved to a value of 1.0 after initiation of this therapeutic regimen. We conclude from our observation that i.v. immunoglobulin therapy is of particular value for the treatment of ITP in patients with impaired cellular immunity.