Successful pregnancy outcome in a case of JAK2 positive essential thrombocytemia after treatment with peginterferon alpha-2b

M.-V. Teleanu1, S. Jaramillo 1, S. Schönsteiner2, S. Estenfelder3, P. Schwarzwälder2, C. Langer2, M. Kull1 (1Ulm, Germany , 2Ulm, Germany, 3Ulm , Germany)

Women issues in thrombosis and hemostasis
Date: 17.02.2017,
Time: 17:15 - 18:15

Objective: Diagnosis of myeloproliferative neoplasms (MPN) puts women in childbearing age at high risk for pregnancy complications. Here we present the case of a pregnant woman with Essential Thrombocytemia (ET).

Methods: A 33-year-old woman presented in our clinic in 12/2015 for counseling and treatment of ET during pregnancy. She was at her 3rd pregnancy, 10 weeks of gestation. Her 1st pregnancy (in 2011) was complicated by intrauterine fetal death at 28 weeks of gestation. At her 2nd pregnancy (in 05/2012) due to intrauterine growth restriction, cesarean section was performed at 29 weeks of gestation and she delivered a female newborn weighing 720 g. In 08/2015 she was diagnosed with ET with JAK2 V617F mutation. Her platelet count was 660 G/L. Additionally type 2A von Willebrand disease (VWD) was diagnosed. At a retrospective analysis of blood tests, elevated platelet counts up to 600 G/L were traced back to 2012. The patient was considered at high risk for pregnancy complications and we started cytoreductive treatment with Peginterferon alpha-2b (PEG-IFNα-2b) at a dose of 35 µg weekly and low dose acetylsalicylic acid (ASA), 100 mg daily.

Results: After one month, platelet counts dropped within normal ranges and remained stable during the course of the pregnancy. No bleeding complications occurred in relation to ASA or VWD. Treatment with PEG-IFNα-2b was stopped 2 weeks and ASA 1 week prior to delivery. In 07/2016 cesarean section was performed at 36 weeks of gestation and she delivered a healthy male baby. Prophylactic treatment with LMWH was given for 3 weeks after delivery. The patient presented in our ambulatory in 10/2016 and she and the baby were in good clinical condition. Her platelet counts rose again to 610 G/L. Until now, the patient is being monitored and we did not restart the cytoreductive treatment.

Conclusion: This case emphasizes the need for carefully investigation in patients with pregnancy complications. Treatment for ET with PEG-IFNα-2b and ASA resulted in successful pregnancy outcome. Another challenge was the associated VWD that could prone the patient to bleeding complications. A clear relation between platelet counts and VWF parameters has not been defined. Yet, an inverse relationship between platelet counts and VWF:RCo and VWF:CB has been suggested, underlining the importance of maintaining platelets within normal ranges