Am J Perinatol 2008; 25(4): 219-223
DOI: 10.1055/s-2008-1064932
© Thieme Medical Publishers

Fetomaternal Platelet Immunization Associated with Maternal Bernard-Soulier Syndrome

Jukka Uotila1 , Outi Tammela2 , Anne Mäkipernaa3
  • 1Department of Obstetrics, Tampere University Hospital, Tampere, Finland
  • 2Department of Neonatology, Tampere University Hospital, Tampere, Finland
  • 3Department of Medicine, Division of Hematology and Coagulation Disorders, Helsinki University Central Hospital, Helsinki, Finland
Further Information

Publication History

Publication Date:
14 March 2008 (online)

ABSTRACT

Our objective was to describe the clinical outcome and the association of fetomaternal platelet immunization in pregnancies with maternal Bernard-Soulier syndrome. We conducted an observational study of two mothers, six pregnancies, and six neonates, with special emphasis on platelet antibodies and thrombocytopenia-associated complications. The first woman presented with platelet-associated autoantibodies and thrombocytopenia, but her three newborn infants showed normal platelet count and function. The other woman had three pregnancies, all of which were complicated by severe fetal and neonatal alloimmune-type thrombocytopenia, without demonstrable antibodies. We concluded that fetomaternal immunization with severe fetal and neonatal thrombocytopenia may be encountered even in the absence of demonstrable antibodies.

REFERENCES

  • 1 Lopez J A, Andrews R K, Afshar-Kharghan V, Berndt M C. Bernard-Soulier syndrome.  Blood. 1998;  91 4397-4418
  • 2 Nurden T A, Nurden P. Inherited defects of platelet function.  Rev Clin Exp Hematol. 2001;  5 314-334
  • 3 Balduini C L, Iolascon A, Savoia A. Inherited thrombocytopenias: from genes to therapy.  Haematologica. 2002;  87 860-880
  • 4 Clemetson K J. Platelet glycoproteins and their role in diseases.  Transfus Clin Biol. 2001;  8 155-162
  • 5 Peng T C, Kickler T S, Bell W R, Haller E. Obstetric complications in a patient with Bernard-Soulier syndrome.  Am J Obstet Gynecol. 1991;  165 425-426
  • 6 Michalas S, Malamitsi-Puchner A, Tsevrenis H. Pregnancy and delivery in Bernard-Soulier syndrome.  Acta Obstet Gynecol Scand. 1984;  63 185-186
  • 7 Heslop H E, Hickton C M, Laird E, Tait J D, Doig J R, Beard E J. Twin pregnancy and parturition in a patient with Bernard Soulier syndrome.  Scand J Haematol. 1986;  37 71-73
  • 8 Peaceman A M, Katz A R, Laville M. Bernard-Soulier syndrome complicating pregnancy: a case report.  Obstet Gynecol. 1989;  73 457-459
  • 9 Saade G, Homsi R, Seoud M. Bernard-Soulier syndrome in pregnancy: a report of four pregnancies in one patient, and review of the literature.  Eur J Obstet Gynecol Reprod Biol. 1991;  40 149-152
  • 10 Khalil A, Seoud M, Tannous R, Usta I, Shamseddine A. Bernard-Soulier syndrome in pregnancy: case report and review of the literature.  Clin Lab Haematol. 1998;  20 125-128
  • 11 Fujimori K, Ohto H, Honda S, Sato A. Antepartum diagnosis of fetal intracranial hemorrhage due to maternal Bernard-Soulier syndrome.  Obstet Gynecol. 1999;  94 817-819
  • 12 Kriplani A, Singh B, Sowbernika R, Choudhry V. Successful pregnancy outcome in Bernard-Soulier syndrome.  J Obstet Gynaecol Res. 2005;  31 52-56
  • 13 Prabu P, Parapia L A. Bernard-Soulier syndrome in pregnancy.  Clin Lab Haematol. 2006;  28 198-201
  • 14 Bussel J B, Schreiber A D. Immune thrombocytopenia purpura, neonatal alloimmune thrombocytopenia and PST-transfusion purpura. In: Hoffman R, Benz EJ, Shattil SJ, et al. Hematology: Basic Principles and Practice. New York; Churchill Livingstone 1991: 1485-1494
  • 15 Williamson L M, Hackett G, Rennie J et al.. The natural history of fetomaternal alloimmunization to the platelet-specific antigen HAP-1a (PLA1, Zwa) as determined by antenatal screening.  Blood. 1998;  92 2280-2287
  • 16 Tiblad E, Olsson I, Petersson K et al.. Experiences with fetomaternal alloimmune thrombocytopenia at a Swedish hospital over a 10-year period.  Acta Obstet Gynecol Scand. 2003;  82 803-806
  • 17 Birchall J E, Murphy M F, Kaplan C, Kroll H. European Fetomaternal Alloimmune Thrombocytopenia Study Group . European collaborative study of the antenatal management of feto-maternal alloimmune thrombocytopenia.  Br J Haematol. 2003;  122 275-288
  • 18 Koskela S, Javela K, Jouppila J, Nyblom O, Partanen J, Kekomäki R. Variant Bernard-Soulier syndrome due to homozygous Asn45Ser mutation in the platelet glycoprotein (GP) IX in seven patients of five unrelated Finnish families.  Eur J Haematol. 1999;  62 256-264
  • 19 McFarland J G, Frenzke M, Aster R H. Testing of maternal sera in pregnancies at risk for neonatal alloimmune thrombocytopenia.  Transfusion. 1989;  29 128-133
  • 20 Porcelijn L, Kanhai H. Fetal thrombocytopenia.  Curr Opin Obstet Gynecol. 1998;  10 117-122
  • 21 Daffos F, Capella-Pavlovsky M, Forestier F. Fetal blood sampling during pregnancy with use of a needle guided by ultrasound. A study of 606 consecutive cases.  Am J Obstet Gynecol. 1985;  153 655-660
  • 22 Daffos F, Forestier F, Kaplan C, Cox W. Prenatal diagnosis and management of bleeding disorders with fetal blood sampling.  Am J Obstet Gynecol. 1988;  158 939-946
  • 23 Bussel J B, Zabusky M R, Berkowitz R L, McFarland J G. Fetal alloimmune thrombocytopenia.  N Engl J Med. 1997;  337 22-26
  • 24 Mueller-Eckhardt C, Kiefel V, Grubert A. High-dose IgG treatment for neonatal alloimmune thrombocytopenia.  Blut. 1989;  59 145-146
  • 25 Bussel J B. Alloimmune thrombocytopenia in the fetus and newborn.  Semin Thromb Hemost. 2001;  27 245-252
  • 26 Blanchette V S, Johnson J, Rand M. The management of alloimmune neonatal thrombocytopenia.  Baillieres Best Pract Res Clin Haematol. 2000;  13 365-390
  • 27 Kaplan C. Immune thrombocytopenia in the foetus and newborn: diagnosis and therapy.  Transfus Clin Biol. 2001;  8 311-314

Jukka UotilaM.D. Ph.D. 

Department of Obstetrics, Tampere University Hospital

P.O. Box 2000, 33521, Tampere, Finland

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