Multicenter Retrospective Analysis of Turkish Patients with Chronic Myeloproliferative Neoplasms

Soyer, Nur; Haznedaroğlu, İbrahim C.; Cömert, Melda; Çekdemir, Demet; Yılmaz, Mehmet; Ünal, Ali; Çağlıyan, Gülsüm Akgün; Bilgir, Oktay; İlhan, Osman; Özdemirkiran, Füsun; Kaya, Emin; Şahin, Fahri; Vural, Fılız; Saydam, Güray

doi:10.4274/tjh.2016.0005

Cited by 16 publications

(19 citation statements)

References 37 publications

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“…The results demostrated that leukemic and fibrotic transformations occurred at rates of 0.6% and 13.2. 21 The rates of leukemic and fibrotic transformation were similar to those of our study. Frederiksen et al showed that the patients with different CMPD were at increased risk of developing both additional hematologic and non-hematologic malignancies.…”

Section: Discussionsupporting

confidence: 89%

Fibrosis Development, Leukemic Transformation and Secondary Malignancies Complicating the Clinical Course of Essential Thrombocythemia

Çiftçiler¹

2019

UHOD

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Essential thrombocythemia (ET) is a myeloproliferative neoplastic disease characterized by abnormal proliferation of megakaryocytes in the bone marrow leading to elevated platelet counts in the peripheral blood. In the present study, we aim to assess the long-term complications of ET and its treatments in terms of the development of secondary malignancies, bone marrow fibrosis and leukemic transformation. One hundred and twenty four patients with ET were included into the study. Retrospective data were collected from our database of myeloproliferative disorders. There were 75 (60.5%) men and 49 (39.5%) women with a median age of 53 (range, 20-80) years. The data indicated that 3 patients treated with hydroxyurea (HU) had suffered of bladder cancer, non-small cell lung cancer and thyroid papillary cancer, 2 patients without treatment suffered of breast cancer and neuroendocrine cancer and 2 patients treated with combination (HU and anagrelide) suffered of acute myeloid leukemia (AML) and prostate cancer. In total, 16/124 patients (12.9%) developed bone marrow fibrosis. Nine of the patients (7.2%) who developed bone marrow fibrosis were receiving HU, 5 of them (4%) were using HU and anagrelide and 2 of them (1.6%) were followed without treatment (p= 0.44). The age (p= 0.03), hemoglobin level at diagnosis (p< 0.001), white blood cell level at diagnosis (p= 0.02), CRP level (p= 0.01), pre-treatment hemorrhage rate (p< 0.001) were statistically significant different between the patients who developed myelofibrosis and patients who did not develop myelofibrosis. The results of this study disclosed that there was no statistically significant difference regarding the development of bone marrow fibrosis, leukemic transformation or secondary malignancies with regard to the treatment options of ET. Moreover, ET patients who had received HU and anagrelide treatment has better OS than the other patient cohorts. ÖZET Esansiyel Trombositozun Klinik Seyrini Oluşturan Fibrozis Gelişimi, Lösemik Dönüşüm ve Sekonder Malignitelerin DeğerlendirilmesiEsansiyel trombositemi (ET), periferik kanda trombosit sayısında artışa yol açan kemik iliğinde anormal megakaryosit proliferasyonu ile karakterize miyeloproliferatif bir neoplastik hastalıktır. Bu çalışmada ET'nin uzun süreli komplikasyonlarını ve tedavilerini sekonder maligniteler, kemik iliği fibrozu ve lösemi transformasyonu açısından değerlendirmeyi amaçladık. ET'li 124 hasta çalışmaya dahil edildi.Retrospektif veriler, myeloproliferatif hastalıklar veritabanımızdan edinilmiştir. Hastaların ortanca yaşı 53 (20-80) saptandı. Çalışmaya 75 (%60.5) erkek ve 49 (% 39,5) kadın dahil edildi. Hidroksiüre (HU) ile tedavi edilen 3 hastada mesane kanseri, küçük hücreli dışı akciğer kanseri ve tiroid papiller kanseri gözlendi. Tedavi almayan 2 hastada meme kanseri ve nöroendokrin kanseri gelişti. Hidroksiüre ve anagrelide alan 2 hastada AML ve prostat kanseri izlendi. Toplamda 16 hastada (%12.9) kemik iliği fibrozu gelişti. Kemik iliği fibrozisi gelişen hastaların 9'u (%7.2) HU, 5'i (% 4) HU ve ...

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Section: Discussionsupporting

confidence: 89%

Fibrosis Development, Leukemic Transformation and Secondary Malignancies Complicating the Clinical Course of Essential Thrombocythemia

Çiftçiler¹

2019

UHOD

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“…Hemorrhages due to treatment (antiplatelet and anticoagulant drugs, thrombocytopenia secondary to HU), disease evolution or complications (thrombocytopenia resulting from disease transformation or splanchnic thrombosis and/or extramedullary hematopoiesis-related portal hypertension/hypersplenism, coagulopathy of liver failure caused by BCS, etc. ), acquired von Willebrand syndrome (AVWS), and intrinsic platelet dysfunctions are also possible during the disease course (51). Diagnosis and management of these specific scenarios involve some specific details.…”

Section: Specific Recommendationsmentioning

confidence: 99%

Polycythemia vera: diagnosis, clinical course, and current management

Büyükaşık¹,

Ali²,

Ar³

et al. 2018

Turk J Med Sci

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Very important developments related to polycythemia vera (PV) have occurred during the last two decades. The discovery of Janus kinase (JAK) 2 mutations has changed both the diagnosis and clinical management of PV. Currently JAK2 molecular testing is essential in the diagnostic work-up and JAK2 mutation positivity is a major diagnostic criterion. The discovery of JAK2 mutations suggested that abnormal JAK-STAT signaling was a pivotal feature in the pathogenesis of Philadelphia-negative myeloproliferative neoplasms. This idea led to the development of JAK inhibitors. Currently ruxolitinib, a JAK1/JAK2 inhibitor, is also approved for PV patients with hydroxyurea resistance or intolerance. International collaborations have made it possible to describe disease characteristics and evolution better. Presently it is possible to quantify the symptomatic burden of the disease and to estimate prognosis. In spite of these developments, management of PV still largely depends on estimation of thromboembolic risk and trying to decrease the risk with or without cytoreductive medications. Different approaches have been proposed by international disease experts for the diagnosis, thromboembolic risk estimation, and drug selection. This paper aims to review clinical aspects of PV and propose a management algorithm. The authors also point to still unresolved questions and unmet needs in diagnosis and management.

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“…In ET, PV and PMF are BCR/ABL-negative CMPDs. The effects of a few genetic mutations have been reported recently in BCR/ABL-negative CMPDs (2).…”

Section: Introductionmentioning

confidence: 98%

“…In ET, typical increases in megakaryocyte and platelet counts are observed. In idiopathic myelofibrosis, bone marrow fibrosis is more prominent (2). In ET, PV and PMF are BCR/ABL-negative CMPDs.…”

Section: Introductionmentioning

confidence: 99%

Retrospective analysis of patients with chronic myeloproliferative neoplasms: a single center experience

Çekdemir¹,

Gündüz²

2018

Med J Islamic World Acad Sci

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Chronic myeloproliferative diseases (CMPD) are clonal diseases that may cause hemostatic and thrombotic abnormalities. They progress to acute leukemia and are characterized by increases in the number of mature and immature cells in the peripheral blood as a result of uncontrolled proliferation of one or more than one type of myeloerythroid cells in the bone marrow. This study aimed to determine demographic features, incidence of Janus kinase 2 (JAK2) mutations, disease characteristics, and treatment strategies in patients diagnosed with CMPD. A total of 100 patients with CMPD [essential thrombocytosis (ET), n = 52; primary myelofibrosis (PMF), n = 31; and polycythemia vera (PV), n = 17] having JAK mutations admitted to outpatient clinics of Hematology Department of Sakarya University Faculty of Medicine between February 2006 and February 2013 were included with the diagnosis of BCR/ABL (The ABL gene from chromosome 9 joins to the BCR gene on chromosome 22, to form the BCR-ABL fusion gene)-negative CMPD based on the 2008 World Health Organization criteria. Age, gender, family history, secondary cancer, bleeding, history of thrombosis, whole blood cell counts, and presence of hepatomegaly, splenomegaly, and other symptoms and signs at the time of diagnosis were evaluated. Besides, a history of thrombosis and hemorrhage was assessed. The presence of JAK mutations in DNA samples was analyzed using real-time polymerase chain reaction. Age and gender distribution, family history, previous incidents of bleeding, thrombosis, secondary cancer, blood hemoglobin, lactate dehydrogenase values, platelet and white blood cell counts, constitutional symptoms, minor neurologic symptoms, and presence of hepatomegaly and splenomegaly at the time of diagnosis were assessed. The incidence of JAK2 mutations was highest among patients with PMF (70.9%), followed by patients with PV (70.6%) and ET (51.9%), in this study. The incidence of JAK2 mutations has offered a different perspective in BCR/ABL-negative patients with CMPD and served as an acceptable diagnostic factor. The present study had a small sample size. Hence, large-sample studies should be conducted to confirm the relationship between this mutation and CMPD.

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Multicenter Retrospective Analysis of Turkish Patients with Chronic Myeloproliferative Neoplasms

Cited by 16 publications

References 37 publications

Fibrosis Development, Leukemic Transformation and Secondary Malignancies Complicating the Clinical Course of Essential Thrombocythemia

Fibrosis Development, Leukemic Transformation and Secondary Malignancies Complicating the Clinical Course of Essential Thrombocythemia

Polycythemia vera: diagnosis, clinical course, and current management

Retrospective analysis of patients with chronic myeloproliferative neoplasms: a single center experience

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