Primary myelofibrosis: own experience and news from diagnostic and treatment

Абдулкадыров, К М; Shuvaev, Vasiliy; Martynkevich, Irina

doi:10.17650/1818-8346-2015-10-2-26-36

Cited by 4 publications

(7 citation statements)

References 30 publications

(45 reference statements)

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“…Primary myelofibrosis (PMF) refers to Ph-negative myeloproliferative diseases in accordance with the WHO classification (2016). Myeloproliferative diseases (MPD) are clonal tumors that occur at the level of a hematopoietic stem cell and are characterized by the proliferation of one or more myelopoiesis cell lines in the bone marrow with signs of preserved terminal differentiation [1,3]. The discovery of the JAK2 V617F mutation (2005) contributed to the revision of the diagnostic criteria for MPD.…”

Section: Discussionmentioning

confidence: 99%

Transformation of primary myelofibrosis into acute myeloblastic leukemia: clinical case

Baidurin,

Bekenova,

Tkachev

et al. 2023

BIO Web Conf.

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Primary myelofibrosis (PMF) is a disease from the group of Ph–negative myeloproliferative tumors, which is characterized by bone marrow fibrosis, splenomegaly and extramedullary hematopoiesis. The mean life in PMF ranges from 7.6 to 10 years and varies widely depending on the appearance of additional mutations and a higher degree of malignancy. Most patients have an indolent flow, and in some patients with PMF, the disease progresses rapidly with the development of acute myeloblastic leukemia. The international stratification system DIPSS-plus allows predicting the occurrence time of the PMF blast transformation phase. The article presents a case of early PMF transformation into acute myeloblastic leukemia in a patient with a heterozygous mutation in the JAK2 gene and an intermediate risk-2 established according to the International Prognostic Scoring DIPSS-plus. The patient received standard cytoreductive therapy.

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Section: Discussionmentioning

confidence: 99%

Transformation of primary myelofibrosis into acute myeloblastic leukemia: clinical case

Baidurin,

Bekenova,

Tkachev

et al. 2023

BIO Web Conf.

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“…We have applied different treatment algorithms to every Ph-MPN form (ET, PV, PMF), based on risks for survival and/ or thrombosis [7][8][9]. .…”

Section: Ocimum Scientific Publishersmentioning

confidence: 99%

“…The risk-based approach was used in PMF management too [9]. The basis for the treatment modality choice was stratificaton by scoring systems: IPSS , DIPSS, DIPSS+, MIPSS information that we had.…”

Section: Submit Manuscriptmentioning

confidence: 99%

Implementation of Algorithm-Based Approach for Ph-Negative Myeloproliferative Neoplasms in Routine Clinical Practice in Russia

Ltd¹

2019

DTHJ

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Background: In recent years there has been significant progress in the diagnostic and treatment of Ph-negative myeloproliferative neoplasms. By the way, they remain heterogenous with great variability in clinical course for every patient. The individual choice from several available treatment methods should be based on balance between potential benefit and risk of adverse events and disease progression. There are some prognostic models for every classic Ph-negative myeloproliferative disease (Essential Thrombocythemia, Polycythemia Vera and Primary Myelofibrosis) that based on retrospective analysis. The implications of treatment algorithms based on such prognostic scales could apply for Phnegative myeloproliferative neoplasms in routine clinical practice.Aim: To compare results of diagnostic and treatment algorithms for Ph-MPN patients management with previous experience. Materials and methods:A database of Ph-MPN patients study group (97 ET patients, 174 PV patients, 94 PMF patients) and control group (116 ET patients, 265 PV patients, 182 PMF patients). Characteristics of the groups of patients were similar. The diagnosis and treatment of study group patients was conducted according to own risk-based algorithms since year 2012. We have applied different treatment algorithms to every Ph-MPN form (ET, PV, PMF), based on risks for survival and/or thrombosis. Results:The implementation of diagnostic algorithm diminished the period between first hematologist referral to diagnosis established from 57 days in control group up to 16 days in study group. The use of risk-adapted approach led to two-times more frequent ET patient's management without cytoreductive therapy (32% in study group vs 14% in control group). Five-year thrombosisfree survival was 91% in study group and 79% in control group. Relative risk for thrombosis was 0.34; 95% C.I. 0.15-0.81; p=0.01). Our ET management algorithm allowed to decrease thrombosis probability from 0.039 event per patient-year in control group to 0.032 event per patient-year in study group. Overall 5-year survival was 93% in study group and 80% in control group. The death probability was decreased from 0.026 event per patient-year in control group to 0.018 event per patient-year. The PV management was also improved with implementation of risk-based algorithm. The cytoreduction in total (drug + excessive red cell removal) were significantly (p=0.0001) rarer in study group (87%) than in control group (98%). HU was used significantly (p=0.0001) more frequent in control group (83%) than in study group (67%). HU as only monotherapy was significantly (p=0.0001) more frequently used in study group (24%) than in control group (11%). Despite of the rarer use of cytoreduction the complete responses almost two times were significantly (p=0.004) frequently

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“…Первичный миелофиброз (ПМФ) -миелопролиферативное новообразование (МПН), характеризующееся клональной пролиферацией трансформированной гемопоэтической стволовой клетки (ГСК) и сопровождающееся процессами фиброза, остеосклероза и ангиогенеза в костном мозге, а также формированием очагов экстрамедуллярного гемопоэза [1][2][3][4].…”

Section: Introductionunclassified

“…Хотя инициирующее событие в ГСК до настоящего времени достоверно не установлено, считают, что к формированию фенотипа («запуску») МПН у 90 % больных приводят повреждения одного из генов JAK2, CALR или MPL, продукты которых прямо или косвенно вовлечены в JAK-STAT-путь передачи сигнала [1,5]. Пациенты с ПМФ без пусковых (драйверных) мутаций составляют группу с тройным негативным статусом (ТНС).…”

Section: Introductionunclassified

Current Genetic Models for Prediction of Primary Myelofibrosis

Polushkina¹,

Transfusiology²,

Shuvaev³

et al. 2019

Клиническая онкогематология

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1 ФГБУ «Российский НИИ гематологии и трансфузиологии ФМБА», ул. 2-я Советская, д. 16, Санкт-Петербург, Российская Федерация, 191024 2 ФГБОУ ВО «Северо-Западный государственный медицинский университет им. И.И. Мечникова» Минздрава России, ул. Кирочная, д. 41, Санкт-Петербург, Российская Федерация, 191015 3 ФГБВОУ ВО «Военно-медицинская академия им. С.М. Кирова» Минобороны России, ул. Академика Лебедева, д. 6, Санкт-Петербург, Российская Федерация, 194044 РЕФЕРАТ Цель. Изучить связь кариотипа, драйверной мутации в генах JAK2, CALR, MPL и мутационного статуса гена ASXL1 с особенностями течения и прогнозом первичного миелофиброза (ПМФ). Материалы и методы. В исследование включено 110 пациентов с диагнозом ПМФ (38 мужчин, 72 женщины), медиана возраста составила 59 лет (диапазон 18-82 года) с медианой срока наблюдения после установления диагноза 2,6 года (диапазон 0,1-23 года). Пациенты обследованы на наличие мутаций в генах JAK2, CALR, MPL и ASXL1. Замену V617F в гене JAK2 и мутации кодона 515 в гене MPL анализировали методом полиморфизма длин рестрикционных фрагментов. Исследование на наличие мутаций в генах CALR (экзон 9), ASXL1 (экзон 12) проводили методом прямого секвенирования по Сэнгеру. У 48 (44 %) из 110 пациентов был определен кариотип клеток костного мозга. Проанализированы клинико-гематологические показатели и медианы общей выживаемости (ОВ) больных с учетом выявленных генетических аберраций и их сочетаний. Результаты. Мутации в генах JAK2, CALR, MPL обнаружены у 55 (50 %), 28 (25,5 %) и 7 (6,4 %) из 110 пациентов соответственно. Тройной негативный статус (ТНС) имели 20 (18,2 %) из 110 обследованных больных. Мутации в гене ASXL1 выявлены у 22 (20 %) из 110 пациентов. Из 48 больных нормальный кариотип обнаружен у 32 (66,7 %), благоприятный -у 3 (6,3 %), промежуточного прогноза -у 4 (8,3 %), неблагоприятный -у 9 (18,7 %). При сравнении клинико-гематологических показателей выявлен ряд статистически значимых отличий. У JAK2позитивных больных отмечался более высокий уровень MYELOID TUMORS

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Primary myelofibrosis: own experience and news from diagnostic and treatment

Cited by 4 publications

References 30 publications

Transformation of primary myelofibrosis into acute myeloblastic leukemia: clinical case

Transformation of primary myelofibrosis into acute myeloblastic leukemia: clinical case

Implementation of Algorithm-Based Approach for Ph-Negative Myeloproliferative Neoplasms in Routine Clinical Practice in Russia

Current Genetic Models for Prediction of Primary Myelofibrosis

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