JAK2V617F negative essential thrombocytosis with pregnancy
Amalia NK*, Mediarty**
*Resident of Internal Medicine Specialist Sriwijaya University, Palembang, Indonesia
** Medical Hematology Oncology Division of Internal Medicine Department, Universitas
Sriwijaya, Doctor Mohammad Hoesin Hospital, Palembang, Indonesia
Abstract
Introduction. Essential thrombocythemia (ET) is generally considered as a chronic
myeloproliferative disorder characterized by a markedly elevated platelet count in the
peripheral blood due to an excessive proliferation of bone marrow megakaryocytes. When
the disease affects women during pregnancy an adverse obstetric outcome is possible. The
objective of this clinical case report is to evaluate the clinical impact of essential
thrombocythemia on the outcome of pregnancy.
Case report. A 28-year-old-woman presented to the hospital with tingling sensation in the
tip of the fingers since one day before admission, with additional complain is headache
since two months. She’s been diagnosed with ET since two years ago. She has
splenomegaly. Now she become pregnant at 15th weeks. Complete blood count examination
revealed thrombocytosis (platelet count: 1.175x109/L). No leukoerythroblastosis or
erythropoietic cells were seen. Her biochemical examination was normal. Bone marrow
examination revealed presence increases of clusters of megakaryocytes. Mutational studies
were done and she was found to be negative for JAK2V617F kinase mutation as well as for
BCR-ABL gene mutation. All the other ultrasounds performed during the course of
pregnancy (at sixth and thirteen weeks) revealed a fetal size appropriate for gestational age.
Discussion. This case illustrates the potential for thrombosis and bleeding event for
maternal and fetal and the importance of comprehensive treatment from hematologist and
obstetriciant. Some research have proposed not to treat the pregnant patients because the
low-risk of thromboembolic disease. In fact pregnancy increases the risk of
thromboembolism. Indeed we believe to treat these women, to prevent multiple infarc of
plasenta. Among the drugs proposed the use of hydroxyurea and anagrelide in the first
trimester, although really effective, is often discouraged. The plateletpheresis was done for
this patient, but the result is not very significant to decrease the platelet count.
Conclusion. In conclusion we consider the aspirin treatment safe and effective since the
preconceptional time, unless contraindicated and still be considered the first choice to treat
ET in pregnancy. In this case, aspirin is contraindicated because of platelet aggregation test
shown hypoaggregation. In the second trimester we try to give the hydroxyurea to decrease
the platelet count, while we evaluate the fetal growth.
Keywords : Essential thrombocytosis, pregnancy, JAK2V617F negative