Polycythemia vera in pregnancy represents a challenge for a multidisciplinary collaboration: A case report and literature review

Bohîlțea, Roxana Elena; Niculescu-Mizil, Emilia; Mihai, Bianca Margareta; Furtunescu, Florentina; Ducu, Ioniță; Munteanu, Octavian; Georgescu, Tiberiu Augustin; Grigoriu, Corina

doi:10.3892/etm.2021.10941

Cited by 4 publications

(6 citation statements)

References 27 publications

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“…Notwithstanding, considering cytoreductive therapy in low‐risk patients, most said that the desire for paternity/maternity would be a criterion for IFN use. This indicates that there is clear regard for therapeutic choice in pregnant women and those of childbearing potential as noted by other authors 20–22 …”

Section: Discussionsupporting

confidence: 61%

“…This indicates that there is clear regard for therapeutic choice in pregnant women and those of childbearing potential as noted by other authors. [20][21][22] Regarding leukocytosis, this is typically defined as >15.0 Â 10 9 /L, although a clear cutoff value is lacking. According to a recent systematic review, cutoffs used for the definition of leukocytosis ranged from 9.5 Â 10 9 /L to 25.0 Â 10 9 /L in PV, as further evidence that there is no well-established cutoff value.…”

Section: Discussionmentioning

confidence: 99%

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Management of polycythemia vera: A survey of treatment patterns in Italy

Palumbo

Breccia

Baratè

et al. 2022

European J of Haematology

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Objectives: Polycythemia vera (PV) is an acquired clonal hematopoietic stem cell disorder characterized by the overproduction of red blood cells. It has long been underlined that there are differences in treatment patterns in routine practice. Therapeutic strategies have also expanded, and in recent years the JAK1/JAK2 inhibitor ruxolitinib has emerged as a second-line therapeutic option in patients who are intolerant to or resistant to hydroxyurea. Determining the impact of changes on practice patterns is of interest, especially for aspects that lack detailed guidance for management. Methods:To gain insights into treatment patterns by clinicians treating patients with PV in Italy, we carried out a survey of 60 hematologists and transfusion specialists.The questions covered: treatment of low-risk patients, definition of significant leukocytosis, splenomegaly and excessive phlebotomies, resistance/intolerance to hydroxyurea, use of ruxolitinib, cytoreductive therapy, and vaccines.Results: In general, the results of the survey indicate that there is a large heterogeneity in management of patients with PV across these areas.Conclusions: While helping to provide greater understanding of treatment patterns for patients with PV in Italy, our survey highlights the need for additional clinical studies to obtain more precise guidance for the routine care of patients with PV.

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Section: Discussionsupporting

confidence: 61%

Section: Discussionmentioning

confidence: 99%

Management of polycythemia vera: A survey of treatment patterns in Italy

Palumbo

Breccia

Baratè

et al. 2022

European J of Haematology

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“…Chronic maternal hypoxia retards foetal growth and leads to the delivery of a newborn with intrauterine growth restriction, 13 while polycythaemia will increase the risk of thrombosis 14 and abortion. 15 …”

Section: Discussionmentioning

confidence: 99%

Iatrogenic diversion of inferior vena cava to the left atrium presented as recurrent foetal loss: a case report

Darwazah

Ibrahim

Nairat

et al. 2022

European Heart Journal - Case Reports

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Background Iatrogenic diversion of inferior vena cava (IVC) to the left atrium (LA) after atrial septal defect repair (ASD) is an unusual complication. It rarely occurred nowadays due to trans-esophageal echocardiography (TEE) check during surgery, but there are still few numbers of patients who survived from an old operation during childhood and reached adulthood undiagnosed. Case Summary We present a 27 years old female post ASD repair in childhood with a unique presentation of recurrent abortion in adulthood besides exertional dyspnea. A full workup of investigations were normal except for hemoglobin of 21 and oxygen saturation of 70%. TEE revealed abnormal drainage of IVC to the LA. Surgical correction was done to release the IVC opening to drain in the right atrium and the oxygen saturation reached 99% after weaning from the bypass machine. The postoperative course was uneventful and the patient was discharged five days later. Two years later, she got pregnant twice and completed her pregnancies to term with well-developed infants. Discussion The diversion of the IVC may be either complete or partial. Such complications may result in intraoperative death on the table or may present as early desaturation, shortness of breath, cyanosis, and clubbing or it may present with such symptoms in adulthood. Rarely, it may present with cerebral stroke. Our case presented with the unexpected presentation of recurrent abortion. So, even if it is rare, echocardiography should be considered as a workup for recurrent abortion in a patient with a history of congenital heart surgery.

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“…PV, along with essential thrombocytosis, chronic myeloid leukemia and idiopathic myelofibrosis, constitute a group of stem cell disorders that lack the Philadelphia chromosome marker, demonstrating similar cytogenetic and molecular characteristics, and can be attributed to several genetic alterations [ 4 ]. In the majority of patients (97%), the mutation responsible for the abnormal proliferation of PV is the highly specific JAK2V617F, with the rest having the JAK2 exon 12 type, both activating the JAK-STAT pathway leading to a defective erythropoiesis and therefore polycythemia [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…PV has a 0.8 to 7.8% thrombosis incidence rate, as well as an increased risk for bleeding, especially in patients with JAK2V617F mutation [ 3 ]. Because of the physical effects in the coagulation cascade that occur during pregnancy in order to prepare for delivery, gestation is a high-risk condition that may result in thrombosis [ 4 ]. In the event of co-existence of a myeloproliferative condition, the risk of thromboembolism is six times higher in pregnant women than in non-pregnant women [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

Portal Vein Thrombosis after C-Section in a Patient with Polycythemia Vera (PV) Due to Pregnancy and Iron Deficiency Anemia (IDA)

Ntounis

Zioutos²,

Koutras

et al. 2022

Clinics and Practice

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Polycythemia vera (PV) is one of the three main classic disorders of Philadelphia-negative myeloproliferative neoplasms (MPNs), with the other two being essential thrombocythemia (ET) and primary myelofibrosis (PMF). PV may develop (15%) in women of childbearing age (15–45 years), with an anticipated rate of roughly 0.3 per 100,000 people, although maintaining a male to female ratio predominance of about 2:1 and a peak prevalence in the sixth and seventh decades of life. Without always being presented with its actual clinical manifestations due to pregnancy itself, and most commonly due to iron deficiency, PV can be frequently missed and therefore belatedly diagnosed. We describe the case of a primipara woman in her 40s, without risk factors for thrombosis, who developed a portal vein occlusion 1.5 month postpartum after C-section and who had a delayed diagnosis of PV.

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Polycythemia vera in pregnancy represents a challenge for a multidisciplinary collaboration: A case report and literature review

Cited by 4 publications

References 27 publications

Management of polycythemia vera: A survey of treatment patterns in Italy

Management of polycythemia vera: A survey of treatment patterns in Italy

Iatrogenic diversion of inferior vena cava to the left atrium presented as recurrent foetal loss: a case report

Portal Vein Thrombosis after C-Section in a Patient with Polycythemia Vera (PV) Due to Pregnancy and Iron Deficiency Anemia (IDA)

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