Acquired Thrombophilia in Pregnancy: Essential Thrombocythemia

Martin Griesshammer; Martin Grünewald; Jan J. Michiels

doi:10.1055/s-2003-38836

Seminars in Thrombosis and Hemostasis, Table of Contents

Semin Thromb Hemost 2003; 29(2): 205-212
DOI: 10.1055/s-2003-38836

Acquired Thrombophilia in Pregnancy: Essential Thrombocythemia

Martin Griesshammer¹ , Martin Grünewald² , Jan J. Michiels³

¹Head, Research Laboratory on Hemostasis, Department of Internal Medicine III, Division of Hemostaseology, University of Ulm, Ulm, Germany
²Department of Internal Medicine III, Division of Hemostaseology, University of Ulm, Ulm, Germany
³Director, Goodheart Institute, European Working Group on MPD, Goodheart Institute and Foundation, Rotterdam, The Netherlands

Abstract

ABSTRACT

The management of pregnant patients with essential thrombocythemia (ET) is a difficult problem. The clinical course of ET is mainly determined by thromboembolic complications. Pregnancy itself is a physiological hypercoagulable state. When ET affects women during pregnancy, an adverse outcome caused by thrombotic complications is a matter of concern. We reviewed 155 pregnancies in 86 women with ET. The success rate (baby alive) was 59%. First-trimester abortion was the most frequent complication and occurred in 31% of pregnancies. Placental infarction caused by thrombosis seemed to be the most consistent pathologic event. Maternal thrombotic or hemorrhagic complications were rare but were more common than those seen in normal pregnancy. Pregnancy itself does not appear to affect adversely the natural course and prognosis of ET. A meta-analysis revealed a significant benefit for aspirin in comparison to no treatment. If cytoreductive therapy becomes necessary, interferon α appears to be the drug of choice. The value of heparin prophylaxis has not been established but may have a role in selected cases.

KEYWORDS

Essential thrombocythemia - pregnancy - treatment - outcome

Full Text

References

REFERENCES

1 Pearson T C. Primary thrombocythaemia: diagnosis and management. Br J Haematol . 1991; 78 145-148
2 Genderen van J J P, Michiels J J. Primary thrombocythemia: diagnosis, clinical manifestations and management. Ann Hematol . 1993; 67 57-62
3 Thiele J, Kvasnicka H M, Diehl V, Fischer R, Michiels J J. Clinicopathological diagnosis and differential criteria of thrombocythemias in various myeloproliferative disorders by histopathology, histochemistry and immunostaining from bone marrow biopsies. Leuk Lymphoma . 1999; 33 207-218
4 Rozman C, Giralt M, Feliu E, Rubio D, Cortes M T. Life expectancy of patients with chronic nonleukemic myeloproliferative disorders. Cancer . 1991; 67 2658-2663
5 Murphy S. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. Semin Hematol . 1999; 36 9-13
6 Tefferi A, Solberg L A, Silverstein M N. A clinical update in polycythemia vera and essential thrombocythemia. Am J Med . 2000; 109 141-149
7 Ruggeri M, Finazzi G, Tosetto A. et al . No treatment for low-risk thrombocythaemia: results from a prospective study. Br J Haematol . 1998; 103 772-777
8 Griesshammer M, Bergmann L, Pearson T C. Fertility, pregnancy and the management of myeloproliferative disorders. Baillieres Clin Haematol . 1998; 11 859-874
9 Tefferi A, Fonseca R, Pereira D L, Hoagland H C. A long-term retrospective study of young women with essential thrombocythemia. Mayo Clin Proc . 2001; 76 22-28
10 Finazzi G, Ruggeri M, Rodeghiero F, Barbui T. Second malignancies in patients with essential thrombocythaemia treated with busulphan and hydroxyurea: long-term follow-up of a randomized clinical trial. Br J Haematol . 2000; 110 577-583
11 Griesshammer M, Heimpel H, Pearson T C. Essential thrombocythemia and pregnancy. Leuk Lymphoma . 1996; 22 57-63
12 Wright C A, Tefferi A. A single institutional experience with 43 pregnancies in essential thrombocythemia. Eur J Haematol . 2001; 66 152-159
13 Greer I A. Thrombosis in pregnancy: maternal and fetal issues. Lancet . 1999; 353 1258-1265
14 Macklon N S, Greer I A. Venous thromboembolic disease in obstetrics and gynaecology: the Scottish experience. Scot Med J . 1996; 41 83-86
15 Bates S M, Ginsberg J S. Thrombosis in pregnancy. Curr Op Hematol . 1997; 4 335-343
16 Cortelazzo S, Viero P, Finazzi G. et al . Incidence and risk factors for thrombotic complications in a historical cohort of 100 patients with essential thrombocythemia. J Clin Oncol . 1990; 8 556-562
17 van Genderen J J P, Budde U, Michiels J J, van Strik R, van Vliet H D M H. The reduction of large von Willebrand factor multimers in plasma in essential thrombocythaemia is related to the platelet count. Br J Haematol . 1996; 93 962-965
18 Hoagland H, Silverstein M. Primary thrombocythemia in the young patient. Mayo Clin Proc . 1978; 53 578-580
19 Bellucci S, Janvier M, Tobelem G. et al . Essential thrombocythemias: clinical evolutionary and biological data. Cancer . 1986; 58 2440-2447
20 Beard J, Hillmen P, Anderson C C, Lewis S M, Pearson T C. Primary thrombocythaemia in pregnancy. Br J Haematol . 1991; 77 371-374
21 Leone G, De Stefano V, D'Addosio A. Essential thrombocythemia: pregnancy. Haematologica . 1991; 76(Suppl 3) 365-367
22 Beressi A H, Tefferi A, Silverstein M N, Petitt R M, Hoagland H C. Outcome analysis of 34 pregnancies in women with essential thrombocythemia. Arch Intern Med . 1995; 155 1217-1222
23 Pagliaro P, Arrigoni L, Muggiasca M L. et al . Primary thrombocythemia and pregnancy: treatment and outcome in fifteen cases. Am J Hematol . 1996; 53 6-10
24 Randi M L, Rossi C, Fabris F, Girolami A. Essential thrombocythemia in young adults: treatment and outcome of 16 pregnancies. J Intern Med . 1999; 246 517-518
25 Bangerter M, Guthner C, Beneke H. et al . Pregnancy in essential thrombocythaemia: treatment and outcome of 17 pregnancies. Eur J Haematol . 2000; 65 165-169
26 Schmidt-Matthiesen H. Gynäkologie und Geburtshilfe, 8th ed. Stuttgart: Schattauer Verlag 1992
27 Zahner J, Wehmeier A, Schneider W. Pregnancy in essential thrombocythemia. Manifestation time and risk for mother and child. Dtsch Med Wochenschr . 1995; 120 1517-1523
28 Falconer J, Pineo G, Blahey W. et al . Essential thrombocythemia associated with recurrent abortions and fetal growth retardation. Am J Hematol . 1987; 25 345-347
29 Willoughby S JB, Fairhead S, Woodcock B E, Pearson T C. Postpartum thrombosis in primary thrombocythaemia. Eur J Haematol . 1997; 59 121-123
30 Sejeny S A, Eastham R D, Baker S R. Platelet counts during normal pregnancy. J Clin Pathol . 1975; 28 812-813
31 Turhan A G, Humphries R K, Cashman J D. et al . Transient suppression of clonal hemopoiesis associated with pregnancy in a patient with a myeloproliferative disorder. J Clin Invest . 1988; 81 1999-2003
32 Griesshammer M, Bangerter M, van Vliet H D M H, Michiels J J. Aspirin in essential thrombocythemia: status quo and quo vadis. Semin Thromb Hemost . 1997; 23 371-377
33 Beaufils M, Uzan S, Donsimoni R, Colau J. Prevention of pre-eclampsia by early anti-platelet therapy. Lancet . 1985; 840-842
34 Lowenwirt I, Dadic P, Krishnamurthy V. Essential thrombocythemia and epidural analgesia in the parturient. Does thromboelastography help?. Reg Anesth . 1996; 21 525-528
35 Rai R S, Cohen H, Dave M, Regan L. Randomized controlled trial of aspirin and aspirin plus heparin in pregnant women with recurrent miscarriage associated with phospholipid antibodies (or antiphospholipid antibodies). Br Med J . 1997; 314 253-257
36 Doll D C, Ringenberg Q S, Yarbo J W. Antineoplastic agents and pregnancy. Semin Oncol . 1989; 16 337-346
37 Chaube S, Simmel E, Lacon C. Hydroxyurea, a teratogenic chemical. Proc Am Assoc Cancer Res . 1963; 4 10
38 Diav-Citrin O, Hunnisett L, Sher G D, Koren G. Hydroxyurea use during pregnancy: a case report in sickle cell disease and review of the literature. Am J Hematol . 1999; 60 148-150
39 Beard M EJ, Conder J L, Clark V A. Ovarian failure following cytotoxic therapy. N Z Med J . 1984; 97 759-762
40 Goldfinger D, Thompson R, Lowe C, Kurz L, Belkin G. Long-term plateletpheresis in the management of primary thrombocytosis. Transfusion . 1979; 19 336-338