Essential thrombocythemia in pregnancy: platelet count and pregnancy outcome.
TL;DR: Close monitoring of the platelet count is useful in the management of women who have essential thrombocythemia and a review of the literature and the own experience suggests that the degree of platelet number reduction may be correlated with pregnancy outcome.
Abstract: We report our experience of 4 successful pregnancies (including a pair of twins) in 3 women who have essential thrombocythemia (ET). All of the patients had a significant fall in platelet count (> 20% of non-pregnant state) during the course of the pregnancy. A review of the literature and our own experience suggests that the degree of platelet count reduction may be correlated with pregnancy outcome. Close monitoring of the platelet count is useful in the management of these cases.
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TL;DR: Pregnant patients with ET have an increased risk of first‐trimester abortion which is not predictable by preconception platelet count or aspirin therapy, and the experience does not support the use of prophylactic platelet apheresis during delivery.
Abstract: Objectives We describe the periconception circumstances and outcome of 43 consecutive pregnancies in an unselected group of young women with essential thrombocythemia (ET). Patients and methods We retrospectively studied 74 consecutive cases of young women with ET seen at our institution, among whom 43 pregnancies occurred in 20 patients. Results Of the 43 pregnancies, 22 (51%) were successful (21 term and 1 preterm live births) and 21 (49%) ended in miscarriages (1 ectopic pregnancy, 2 elective abortions, 16 first-trimester spontaneous abortions, 1 stillbirth at 22 wk, and 1 abruptio placentae at 33 wk). Management of ET at the time of conception included either no specific therapy (16 cases) or the use of aspirin alone (24 cases), a cytoreductive agent (2 cases), or heparin (1 case). There were no significant differences with respect to platelet count or the effect of treatment with aspirin, either at the time of conception or during the first trimester, among cases of successful pregnancies (22), all miscarriages (21), or first-trimester spontaneous abortions (16). The findings were similar when the analysis was restricted to only first-time pregnancies. In patients with multiple pregnancies, the outcome of a subsequent pregnancy was not predicted by the outcome of the first. In general, in successful cases the last two trimesters were mostly uneventful, with healthy offspring being reported in all cases. Conclusions Pregnant patients with ET have an increased risk of first-trimester abortion which is not predictable by preconception platelet count or aspirin therapy. In addition, our experience does not support the use of prophylactic platelet apheresis during delivery.
98 citations
TL;DR: Patients with essential thrombocythemia have an increased risk of first-trimester abortion, which does not appear to be predictable or influenced by therapy, however, most patients are able to carry pregnancies to term with little or no risk of obstetric or thrombohemorrhagic complications during or after delivery.
Abstract: Objective: To evaluate the clinical impact of essential thrombocythemia on the outcome of pregnancy or vice versa. Design: A retrospective study. Setting: All patients were seen at our tertiary referral center, and most were followed up by their local physicians. Patients: From 1975 through 1991, 73 women younger than 50 years with essential thrombocythemia were seen at our institution. All patients were followed up through patient or physician contact. A detailed obstetric history, including peripartum complications and management, was obtained. Results: Among the 73 women, 34 pregnancies occurred in 18 patients. There were two uncomplicated elective abortions and one ectopic pregnancy. Of the 31 other pregnancies, 17 (55%) resulted in live birth and 14 (45%) ended in spontaneous abortion (all but two in the first trimester). Abortion could not be predicted from history of disease complications before or during pregnancy or by the presence or absence of specific therapy during pregnancy. Preconception platelet counts in women whose pregnancies resulted in live birth were similar to those of women whose pregnancies ended in abortion. Other complications during pregnancy were rare. Conclusions: Patients with essential thrombocythemia have an increased risk of first-trimester abortion, which does not appear to be predictable or influenced by therapy. However, most patients are able to carry pregnancies to term with little or no risk of obstetric or thrombohemorrhagic complications during or after delivery. Overall, specific therapy during pregnancy did not appear to modify the clinical outcome, and the benefit of platelet apheresis during delivery could not be substantiated. (Arch Intern Med. 1995;155:1217-1222)
76 citations
TL;DR: Treatment with low-dose aspirin during pregnancy in ET seemed to reduce complications and also seemed beneficial duringregnancy in PV, and in high-risk pregnancies, the additional use of low molecular weight heparin and/or interferon alpha should be considered.
Abstract: The management of pregnant patients with essential thrombocythemia (ET) and polycythemia vera (PV) may be problematic. In the literature there are approximately 300 cases of pregnancies reported in ET and less than 50 pregnancies reported in PV. To reduce the effect of reporting bias, we selected articles with either > 10 pregnancies or at least six patients, and here report on the outcome of 195 pregnancies in ET and 36 pregnancies in PV patients. The live birth rate was approximately 60% in ET and 58% in PV. Spontaneous abortion during the first trimester was the most frequent fetal complication, occurring in 31% of ET pregnancies and in 22% of PV pregnancies, respectively. Major maternal complications were more frequent in PV compared with ET (44.4 versus 7.7%). Treatment with low-dose aspirin during pregnancy in ET seemed to reduce complications and also seemed beneficial during pregnancy in PV. In high-risk pregnancies, the additional use of low molecular weight heparin and/or interferon alpha should be considered. This article also describes a registry for an observational study concerning pregnancy in chronic Philadelphia chromosome-negative myeloproliferative disorders within the European LeukemiaNet. A potential management algorithm for pregnancies in ET or PV is also provided.
72 citations
TL;DR: Pregnancy in PV is per se a high risk situation and all women with PV should be treated with low-dose aspirin, whereas some pregnant PV patients may benefit from a more intensive therapy including interferon alpha +/- low molecular weight heparin throughout pregnancy and at least for six weeks post-partum.
Abstract: The management of pregnancy in Philadelphia negative chronic myeloproliferative disorders (CMPDs) is an increasingly frequent problem. In the literature, most pregnancies are reported for women with essential thrombocythemia (ET) with about 400 pregnancies in about 200 women. In ET, first trimester abortion is the most frequent complication occurring in about one third of pregnancies. Interestingly, the incidence of maternal complications is relatively low with 3% for major thromboembolic and 2% for major bleeding events. The presence of the Jak2 mutation seems to be an independent predictor of pregnancy complications. Pregnancies in ET should be stratified according to underlying risk factors in low, high and highest risk pregnancies. Women with low risk pregnancies are treated with low-dose aspirin, whereas women with high and higher risk pregnancies may benefit from low-dose aspirin plus interferon alpha +/- low molecular weight heparin throughout pregnancy and at least for six weeks post-partum. In polycythemia vera (PV) there is only very few information on pregnancy outcome with 36 pregnancies reported in the literature. According to these data pregnancy in PV is per se a high risk situation. Accordingly, all women with PV should be treated with low-dose aspirin. Some pregnant PV patients may benefit from a more intensive therapy including interferon alpha +/- low molecular weight heparin throughout pregnancy and at least for six weeks post-partum.
65 citations
TL;DR: In summary, 57% of women with ET had a live birth, maternal complications happened in 6%.
Abstract: A review of the literature disclosed 106 pregnancies (preg.) in 57 women with essential thrombocythemia (ET). The success rate (baby alive) was 57% (60 live births/106 preg.), the rate of miscarriage 43% (46 miscarriages/106 preg.). The most frequent complication was spontaneous abortion during the first trimester in 36% (38 abortions/106 preg.). Other complications such as intrauterine death and stillbirth after the 28th week, which occurred in 5% (7/106), premature delivery in 8% (8/106), pre-eclampsia in 4% (4/106), and fetal growth retardation in 4% (4/106) were rarer events. Placental infarction due to thrombosis seems to be the most consistent pathological event as far as the fetus is concerned. Maternal hemorrhage occurred in 4% (3 minor and 1 major bleeding) and only 2 minor maternal thrombotic episodes have been observed. Interestingly, a decline in platelet count has been observed in 14 women and was associated with a successful preg, in 13/14 cases (93%). Aspirin (ASS) was the most frequently used drug in 47 of 93 recorded cases (51%). In 16 evaluable women treated with ASS the live birth rate was higher (12/16 preg., 75%) than for 21 untreated women (9/21 preg., 43%). In 5 cases interferon alpha (IFN) has been used successfully. In summary, 57% of women with ET had a live birth, maternal complications happened in 6%. Promising treatment modalities might be ASS and IFN. However, no definitive answer can be given on the ideal management for women with ET during pregnancy. A European register should be set up.
64 citations
References
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Journal Article•
485 citations
TL;DR: Nin 56j~hriger Mann, der bis auf eine Ischias im Jahre 1917 und eine beginnende Gangr~n an 4er zweiten Zehe des rechten Fuges immer gesund gewesen war, erkrankte im Juni 1928 mit Neigung zu heftigen Zahnfleischand Zwischengewebsblutungen an beiden Oberschenkeln.
Abstract: Nin 56j~hriger Mann, der bis auf eine Ischias im Jahre 1917 und eine beginnende Gangr~n an 4er zweiten Zehe des rechten Fuges immer gesund gewesen war, erkrankte im Juni 1928 mit Neigung zu heftigen Zahnfleischand Zwischengewebsblutungen an beiden Oberschenkeln. Besonders naeh einer Zahnextraktion blutete er 4urch 3 Woehen hindurch sehr stark. Am XuBeren des Patienten fiel in der letzten Zeit eine znnehmende l~otf~rbung auf. Er lift an starken Kopfsehmerzen und an Druekgeffihl im Kopfe. Bei der Untersuehnng des kr~ftigen, muskulSsen ~annes fand sieh eine eigenartige t{Stung des Kopfes und der Sehleimhgute, die bei leieht eyanotischem Farbton an das Bestehen einer Erythrgmie denken lieB. Der Befund~ yon Lunge and ~erz war v611ig normal. Leber un4 Milz waren nieht vergrSBert. Der Blutdruek betrug 150 mg tIg. Im t L r n war Albumen in Spuren nachweisbar. Probe auf Zueker negativ. Urobilinogen nieht vermehrg. ~ikroskopiseher Befund o. ]3. Die Wa.t~. war negativ.
108 citations
01 Sep 1978
TL;DR: Thrombocythemia without a secondary cause appears to be much more benign in the young patient than in the older, and aggressive chemotherapy to lower the platelet count is not necessarily justified unless serious complications develop.
Abstract: Primary thrombocythemia is typically a disease of older patients and frequently is associated with thrombosis and hemorrhage. Seven female and two male patients younger than 30 years were evaluated because of platelet counts exceeding 1 million/mm3. No hemorrhagic or thrombotic problems existed before diagnosis or developed during the follow-up period of 14 months to 10 years. Platelet function studies done in six of the nine patients showed absent aggregation with epinephrine and variable aggregation with adenosine diphosphate, as are seen in other chronic myeloproliferative states. One patient had spontaneous aggregation. Secondary causes of thrombocytosis were excluded. Only one patient received specific chemotherapy, which was given before 1973. Thus, thrombocythemia without a secondary cause appears to be much more benign in the young patient than in the older, and aggressive chemotherapy to lower the platelet count is not necessarily justified unless serious complications develop.
84 citations
TL;DR: It is concluded that ET in young adults is not always a benign disease and that potentially life‐threatening complications are not rare, and the optimum approach to treatment in this or any other age group remains uncertain.
Abstract: Essential thrombocythemia (ET) is a myeloproliferative disorder characterized by isolated overproduction of platelets, thrombohemorrhagic complications, and a median age of 50-60. When it occurs in younger patients, the incidence of complications has been reported to be quite low, with a good long-term prognosis. We report a retrospective review of 13 patients with ET between the ages of 22 and 35 in which 11 were symptomatic at diagnosis, with only one remaining asymptomatic during follow-up. Three patients presented with potentially life-threatening complications (two myocardial infarctions, one stroke), although no deaths were observed. The majority of the nonlife-threatening complications were vaso-occlusive in nature, including erythromelalgia and transient neurologic symptoms. We conclude that ET in young adults is not always a benign disease and that potentially life-threatening complications are not rare. The optimum approach to treatment in this or any other age group remains uncertain.
70 citations
TL;DR: It is postulate that the spontaneous abortions, fetal growth retardation, and pre‐eclampsia may be related to the thrombocytosis and suggest that the use of antiplatelet agents throughout pregnancy should be seriously considered in such cases.
Abstract: Essential thrombocythemia (ET) is one of the myeloproliferative disorders characterized by an elevated platelet count, usually greater than 1,000 X 10(9)/L. It may be associated with either hemorrhagic or thrombotic tendencies. Although it usually affects older people, we have recently encountered two young women with ET who had a total of six spontaneous abortions before the end of the first trimester. During her most recent pregnancy, one of the patients underwent plateletpheresis on four separate occasions before the 12th week. She reached 34 weeks but demonstrated evidence of fetal growth retardation and pre-eclampsia. At delivery there were scattered villus infarcts in the placenta. Based on this patient and previous reports in the literature, we postulate that the spontaneous abortions, fetal growth retardation, and pre-eclampsia may be related to the thrombocytosis and suggest that the use of antiplatelet agents throughout pregnancy should be seriously considered in such cases.
69 citations