First example of familial posttransfusion purpura in two PlA1-negative sisters. Transfusion 1988;28(4):326-9
Date
07/01/1988Pubmed ID
3388478DOI
10.1046/j.1537-2995.1988.28488265259.xScopus ID
2-s2.0-0023790295 (requires institutional sign-in at Scopus site) 4 CitationsAbstract
Posttransfusion purpura (PTP) (platelet count 5000/microliter) 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 PlA1 negative, and her plasma contained potent anti-PlA1. In 1986, her sister (never transfused, gravida III, para III) developed PTP (platelet counts 5-15,000/microliter) following surgery-associated transfusion. She did not respond to pooled random-donor platelets. Platelet-associated IgG was markedly elevated (5365) molecules/platelet; normal, less than 660); her plasma contained a potent platelet antibody with anti-PlA1 specificity. Her platelets were subsequently shown to be PlA1 negative. The platelet count did not rise above 30,000 per microliter, 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-PlA1.
Author List
Chaplin H, Aster RH, Morgan LK, Horn B, McDonagh KT, Baskin RAuthor
Richard H. Aster MD Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Antigens, Human PlateletBlood Grouping and Crossmatching
Blood Platelets
Female
HLA Antigens
Humans
Integrin beta3
Isoantigens
Pregnancy
Pregnancy Complications, Hematologic
Purpura
Transfusion Reaction
