Glanzmann's Thrombasthenia in Pregnancy: Clinical Features and Platelet Serology

Abstract

A 24-year-old woman with a diagnosis of Glanzmann's Thrombasthenia (GT) type I (confirmed in our Hemostasis Section by sodium dodecyl sulphate-polyacrylamide gel electrophoresis) and with a history of several transfusions, was seen in our Service since the second month of her first pregnancy.In the first visit a strong anti-Rh(D) antibody was found in her serum. In 28th week an ammiocentesis was done. Seventy two hours later, she was hospitalized because of disminution of foetal movements. Then, a platelet-allo-antibody, IgG+IgM type (title IgG 1/512, IgM 1/2) was detected in her scrum by immunofluorescence test, as well as an anti-HLA antibody (by lymphocytotoxicity test) which reacted with her husband lyin-phocites.The platelet antibody wasn't EDTA or Paraformaldehide (PFA) dependent and reacted with all normal platelets of a panel of known platelet phenotypes and, also although significantly weakly, with GT type II platelets. The negative results were only observed with GT type I platelets. Therefore, it seemed to recognize an antigenic site located in GP IIb-IIIa. Besides, this antibody inhibited the normal platelet aggregation.A caesarian was advised because of foetal suffering an was performed without immediate complications. The patient was protected by our hemotherapy established support and delivered a boy with a severe hemolytic disease (Hematocrit: 6%, bilirrubine: 1,9 mgs./lOCimls., platelets: 40.000/ mm3), who died 48h. later. His platelets had a direct positive test IgG type and in his serum the platelet antibody as in his mother's was detected.We discuss the specificity of the antibody detected and also, its possible implication in the neonatal thrombocytopenia.

A systematic review of the management and outcomes of pregnancy in Glanzmann thrombasthenia

“…There were 3/41 (7%) in utero deaths arising from intracranial haemorrhage in foetuses at 24 and 31 weeks gestation [27] and a pregnancy loss at 9 weeks gestation [21]. There was 1/41 (2.4%) neonatal death at 48 h after an emergency caesarean section at 28 weeks for reduced foetal movements shortly after amniocentesis [36]. In all the pregnancies in which there was a foetal or neonatal death, the mothers had platelet alloantibodies.…”

“…Maternal gravidity (G) and parity (P) were documented for 36 pregnancies G1P0 in 27/36 (75%) [10,11,[13][14][15][16][17][18][19][20][21][22][25][26][27][28][29][30][31][32][33][35][36][37][38][39]; G2P1 in 6/36 (17%) [15,23,26,27,34,39]). There was a prior history of first trimester foetal loss (G2P0) in 3/36 (8%) [12,21,24].…”

“…Maternal alloantibody test results were documented for 22 pregnancies and in 6/22 (27%) were documented as negative [15,26,27,35]. During 16/22 (73%) pregnancies at least one alloantibody was detected and were identified at the start of pregnancy in 10/22 (45%) [13,18,21,27,29,36,39]. In 3/22 (14%) pregnancies, the alloantibody titre increased during pregnancy [13,18,29].…”

“…In one pregnancy, a platelet alloantibody present before pregnancy became undetectable at delivery [27]. Maternal alloantibodies showed specificity against GPIIb-IIIa alone in 6/22 (27%) pregnancies [13,21,27,29,32,37], HLA system epitopes alone in 3/22 (14%) pregnancies [12,27,39] and both GPIIb-IIIa and HLA epitopes in 4/22 (18%) pregnancies [11,18,21,36]. Alloantibodies were described as Ômulti specificÕ or against Ôseveral tissue antigensÕ in two pregnancies [15,32].…”

“…No other outcomes were reported. No antibody reduction was attempted in the remaining 10/16 (63%) pregnancies with alloantibodies [13,21,27,32,36,37,39].…”