Trombotická trombocytopenická purpura v těhotenství
Kazuistika
Authors:
J. Škultéty 1; M. Nováčková 1; T. Binder 1; I. Hadačová 2; P. Salaj 3; Lukáš Rob 1
Authors place of work:
Gynekologicko-porodnická klinika 2. LF UK a FN Motol, Praha, přednosta prof. MUDr. L. Rob, CSc.
1; Oddělení klinické hematologie, FN Motol, Praha, primářka MUDr. I. Hochová
2; Ústav hematologie a krevní transfúze, Praha, ředitel prof. MUDr. M. Trněný, CSc.
3
Published in the journal:
Ceska Gynekol 2010; 75(4): 306-308
Category:
Case Report
Summary
Objective:
Description of case of patient with rare thrombotic thrombocytopenic purpura in pregnancy.
Subject:
Case report.
Setting:
Department of Gynecology and Obstetrics, Charles University and University Hospital Motol, Prague.
Conclusion:
Thrombotic thrombocytopenic purpura (TTP) is a rare and substantial disorder characterized with combination of microangiopathic haemolytic anemia, consumption trombocytopenia and symptoms of organs dysfunction – especially kidneys and neurological deficiency. It‘s caused by production of microthrombi affecting small blood vessels. These palatelets-rich microtrombi are formed due to deficiency of the enzyme ADAMTS13 – metalloprotease which is responsible for cleaving of ultralarge multimers of von Willebrand factor into smaller units. In our case report we describe patient with TTP in pregnancy. Therapy with corticosteroids and immunoglobulines was not effective, improvement of thrombocytopenia appeared after plasmapheresis (total count 14). The delivery was induced at term without complications. Target examination confirmed diagnosis of secondary TTP.
Key words:
thrombotic thrombocytopenic purpura, TTP, thrombocytopenia, pregnancy, plasmapheresis.
Zdroje
1. Binder, T., Salaj, P., Vavřinková, B. Hematologické nemoci a poruchy v porodnictví a gynekologii. Praha: Triton 2004, s. 47-62.
2. Crowther, MA., George, JN. Thrombotic thrombocytopenic purpura: 2008 update. Cleve Clin J Med 2008, 75(5), p. 369-375.
3. Fontana, S., Hovinga, JA., Lämmle, B., Teleghani, M. Treatment of thrombotic thrombocytopenic purpura. Vox Sanguinis 2008, 90, p. 245-254.
4. Jaffey, PB., Feldman, HA. The clinical spectrum of thrombotic thrombocytopenic purpura. Emergency Med 2005, p. 36-44.
5. Karim, R., Sacher, RA. Thrombocytopenia in pregnancy. Curr Hematol Rep 2004, 3(2), p. 128-133.
6. Moake, JL. Von Willebrand factor, ADAMTS-13 and thrombotic thrombocytopenic purpura. Semin Hematol 2004, 41(1), p. 4-14.
7. Murrin, RJ., Murray, JA. Thrombotic thrombocytopenic purpura: aetiology, pathophysiology and treatment. Blood Rev 2006, 20(1), p. 51-60.
Štítky
Paediatric gynaecology Gynaecology and obstetrics Reproduction medicineČlánok vyšiel v časopise
Czech Gynaecology
2010 Číslo 4
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