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First example of familial posttransfusion purpura in two Pl A1 ‐negative sisters
Author(s) -
Chaplin H.,
Aster R.H.,
Morgan L.K.,
Horn B.,
McDonagh K.T.,
Baskin R.
Publication year - 1988
Publication title -
transfusion
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.045
H-Index - 132
eISSN - 1537-2995
pISSN - 0041-1132
DOI - 10.1046/j.1537-2995.1988.28488265259.x
Subject(s) - platelet , medicine , prednisone , methylprednisolone , platelet transfusion , purpura (gastropod) , platelet factor 4 , immunology , gastroenterology , biology , ecology
Posttransfusion purpura (PTP) (platelet count 5000/μl) was diagnosed in a female patient (never transfused, gravida IV, para IV) 1 week after transfusion for hysterectomy in 1978. She did not respond to pooled random‐donor platelets but recovered following a single plasma exchange. Her platelets were Pl A1 negative, and her plasma contained potent anti‐Pl A1 . In 1986, her sister (never transfused, gravida III, para III) developed PTP (platelet counts 5–15,000/μl) following surgery‐associated transfusion. She did not respond to pooled random‐donor platelets. Platelet‐associated IgG was markedly elevated (5365) molecules/platelet; normal, < 660); her plasma contained a potent platelet antibody with anti‐Pl A1 specificity. Her platelets were subsequently shown to be Pl A1 negative. The platelet count did not rise above 30,000 per μl, despite 3 days of high‐dose methylprednisolone sodium succinate and 2 weeks of prednisone (80 mg/day). Later, her platelet count increased and remained normal after steroids were discontinued. The two sisters proved to be HLA‐identical, and each possessed one haplotype carrying the DR3 marker, which has been implicated as a risk factor in neonatal alloimmune thrombocytopenia associated with anti‐Pl A1 .