Contributions
Abstract: PB1725
Type: Publication Only
Session title: Myeloproliferative neoplasms - Clinical
Background
Essential thrombocythaemia (ET) is a clonal stem cell disorder presented with elevated platelet counts. ET in young women can be challenging due to their childbearing potential. There can be increased risk of fetal and maternal complications. The most common pregnancy-related complication is spontaneous abortion during the first trimester.
Aims
We present here a high-risk ET patient 38y old with successful pregnancy and with gestational thrombocytopenia
Methods
Chart review of the case records of the hospital
Results
The patient has been followed by Hematologist since 2014 due to four miscarriages (2013, 2015, 2017 and 2018) but had normal pregnancy with healthy boy born in 2002. Her platelet (PLT) count started to increase from 2010 and in 2014 JAK2 positive ET was diagnosed. In 2017 patient reported heavy metrorrhagia caused by acquired von Willebrand disease with loss of high weight vWF multimers. Hemostasis analyses: APTT 47.7s (28-40), PT-INR 1.28 (0.8-1.2), fibrinogen 1.3 g/L (1.6-3.5), D-dimers 0.28 µg/l (<0.5), FVIII 37% (60-150), vWFAg 61% (50-160), vWFac 34% (46-146), vWFAg/ac ratio 0.55 (>0.7). Patient was consulted by genetics, prothrombin and factor V Leiden mutation were negative.
The highest PLT count was 1604x109/L before the last 6th pregnancy in 2019. During the first and second trimester PLT count was in range of 600x109/L and decreased to normal reference range before delivery (PLT 398x109/L). Patient was treated with LMW heparin Enoxaparin sodium and low-dose Aspirin (75mg) during the 2nd-3rd trimester and after delivery for 10 days. PLT count at week (w) 12 was 1122 x109/L, w22 600 x109/L, w36 475 x109/L and w39 398 x109/L. Hemostasis analyses in w36: APTT 31.6s (28-40), PT-INR 1.1 (0.8-1.2), fibrinogen 3.6 g/L (1.6-3.5), D-dimers 0.64 µg/l (<0.5), FVIII 122% (60-150), vWF 257% (50-160), vWFac 182% (46-146). Five weeks after the delivery PLT count increased again to 1099x109/L Patient had no bleeds during pregnancy and blood loss was 300 ml during delivery.
Conclusion
We conclude that the patient had pregnancy associated thrombocytopenia due to PLT count decrease of the 70% from base value. It has been reported PLT decrease up to 20% in patients with ET during pregnancy. However, PLT count decrease could also be a side effect of Enoxaparin treatment but PLT decrease was profound, more than 30-50% in which observed PLT change was unlikely caused by Enoxaparin treatment. Furthermore, clinical findings do not support drug-induced thrombocytopenia. Pregnancy outcome was successful without initially planned alfa-interferon therapy. VWF level normalized and during pregnancy increased to thrombosis state, but VWF was still dysfunctional with lack of high molecular weight multimers. Clinically there was no reports of bleeding during delivery and Enoxaparin therapy after the childbirth. Our patient's case refers to the need of individual person and situation specific guiding of pregnancy in patients with myeloproliferative neoplasms which can be challenging.
Keyword(s): Essential Thrombocytemia, Myeloproliferative disorder, Platelet count, Pregnancy
Abstract: PB1725
Type: Publication Only
Session title: Myeloproliferative neoplasms - Clinical
Background
Essential thrombocythaemia (ET) is a clonal stem cell disorder presented with elevated platelet counts. ET in young women can be challenging due to their childbearing potential. There can be increased risk of fetal and maternal complications. The most common pregnancy-related complication is spontaneous abortion during the first trimester.
Aims
We present here a high-risk ET patient 38y old with successful pregnancy and with gestational thrombocytopenia
Methods
Chart review of the case records of the hospital
Results
The patient has been followed by Hematologist since 2014 due to four miscarriages (2013, 2015, 2017 and 2018) but had normal pregnancy with healthy boy born in 2002. Her platelet (PLT) count started to increase from 2010 and in 2014 JAK2 positive ET was diagnosed. In 2017 patient reported heavy metrorrhagia caused by acquired von Willebrand disease with loss of high weight vWF multimers. Hemostasis analyses: APTT 47.7s (28-40), PT-INR 1.28 (0.8-1.2), fibrinogen 1.3 g/L (1.6-3.5), D-dimers 0.28 µg/l (<0.5), FVIII 37% (60-150), vWFAg 61% (50-160), vWFac 34% (46-146), vWFAg/ac ratio 0.55 (>0.7). Patient was consulted by genetics, prothrombin and factor V Leiden mutation were negative.
The highest PLT count was 1604x109/L before the last 6th pregnancy in 2019. During the first and second trimester PLT count was in range of 600x109/L and decreased to normal reference range before delivery (PLT 398x109/L). Patient was treated with LMW heparin Enoxaparin sodium and low-dose Aspirin (75mg) during the 2nd-3rd trimester and after delivery for 10 days. PLT count at week (w) 12 was 1122 x109/L, w22 600 x109/L, w36 475 x109/L and w39 398 x109/L. Hemostasis analyses in w36: APTT 31.6s (28-40), PT-INR 1.1 (0.8-1.2), fibrinogen 3.6 g/L (1.6-3.5), D-dimers 0.64 µg/l (<0.5), FVIII 122% (60-150), vWF 257% (50-160), vWFac 182% (46-146). Five weeks after the delivery PLT count increased again to 1099x109/L Patient had no bleeds during pregnancy and blood loss was 300 ml during delivery.
Conclusion
We conclude that the patient had pregnancy associated thrombocytopenia due to PLT count decrease of the 70% from base value. It has been reported PLT decrease up to 20% in patients with ET during pregnancy. However, PLT count decrease could also be a side effect of Enoxaparin treatment but PLT decrease was profound, more than 30-50% in which observed PLT change was unlikely caused by Enoxaparin treatment. Furthermore, clinical findings do not support drug-induced thrombocytopenia. Pregnancy outcome was successful without initially planned alfa-interferon therapy. VWF level normalized and during pregnancy increased to thrombosis state, but VWF was still dysfunctional with lack of high molecular weight multimers. Clinically there was no reports of bleeding during delivery and Enoxaparin therapy after the childbirth. Our patient's case refers to the need of individual person and situation specific guiding of pregnancy in patients with myeloproliferative neoplasms which can be challenging.
Keyword(s): Essential Thrombocytemia, Myeloproliferative disorder, Platelet count, Pregnancy