EPIDEMIOLOGICAL, CLINICAL, MOLECULAR AND GENETIC CHARACTERISTICS OF PRIMARY MYELOFIBROSIS IN THE RUSSIAN FEDERATION.
Author(s): ,
Anait Melikyan
Affiliations:
National Research Center for Hematology,Moscow,Russian Federation
,
Irina Subortseva
Affiliations:
National Research Center for Hematology,Moscow,Russian Federation
,
Darya Baryshniikova
Affiliations:
Regional Clinic Hospital,Omsk,Russian Federation
,
Elena Volodicheva
Affiliations:
Tula Regional Clinical Hospital,Tula,Russian Federation
,
Elena Gilyazitdinova
Affiliations:
National Research Center for Hematology,Moscow,Russian Federation
,
Natal'ya Glonina
Affiliations:
Regional Clinical Hospital №1 named after Professor S.I. Sergeev,Khabarovsk,Russian Federation
,
Georgij Dimov
Affiliations:
Chelyabinsk Regional Clinical Hospital,Chelyabinsk,Russian Federation
,
Mariya Ivanova
Affiliations:
First St. Petersburg Medical University named after Academician I.P. Pavlov,St. Petersburg,Russian Federation
,
Kamil' Kaplanov
Affiliations:
Volgograd Regional Clinical Oncologic Dispensary №1,Volgograd,Russian Federation
,
Anna Kaplina
Affiliations:
Rostov Regional Clinical Hospital,Rostov on Don,Russian Federation
,
Elena Kirillova
Affiliations:
Omsk Regional Clinical Hospital,Omsk,Russian Federation
,
Marina Kiseleva
Affiliations:
Chelyabinsk Regional Clinical Hospital,Chelyabinsk,Russian Federation
,
Tat'yana Klitochenko
Affiliations:
Volgograd Regional Clinical Oncologic Dispensary №1,Volgograd,Russian Federation
,
Tamara Koloshejnova
Affiliations:
National Research Center for Hematology,Moscow,Russian Federation
,
Elena Komarceva
Affiliations:
Rostov Regional Clinical Hospital,Rostov on Don,Russian Federation
,
Sergey Kulikov
Affiliations:
National Research Center for Hematology,Moscow,Russian Federation
,
Elza Lomaia
Affiliations:
National Medical Research Center named after V.A. Almazov,St. Petersburg,Russian Federation
,
Michail Mihalev
Affiliations:
Krasnoyarsk Interdistrict Clinical Hospital №7,Krasnoyarsk,Russian Federation
,
Olga Senderova
Affiliations:
Irkutsk Regional Clinical Hospital of the Order 'Badge of Honor',Irkutsk,Russian Federation
,
Mikhail Fominykh
Affiliations:
Russian Research Institute of Hematology and Transfusiology of the Federal Medical and Biological Agency,St. Petersburg,Russian Federation
,
Anna Hazieva
Affiliations:
Regional Clinic Hospital,Krasnoyarsk,Russian Federation
,
YUliya CHabaeva
Affiliations:
National Research Center for Hematology,Moscow,Russian Federation
,
YUliya CHernyh
Affiliations:
Moscow Regional Research and Clinical Institute named after M.F. Vladimirsky,Moscow region,Russian Federation
,
Kristina Shashkina
Affiliations:
National Research Center for Hematology,Moscow,Russian Federation
Vasilij SHuvaev
Affiliations:
Russian Research Institute of Hematology and Transfusiology of the Federal Medical and Biological Agency,St. Petersburg,Russian Federation
EHA Library. Subortseva I. May 16, 2019; 268126; PB2217
Dr. Irina Subortseva
Dr. Irina Subortseva
Contributions
×
Abstract

Abstract: PB2217

Type: Publication Only

Background
To date studies have not been conducted to assess the epidemiological characteristics of Ph-negative myeloproliferative neoplasms, in particular primary myelofibrosis (PMF) in the Russia.

Aims
Creation of the Russian registry of primary myelofibrosis to assess the epidemiological clinical molecular characteristics of the PMF.

Methods
In 2017 initiated a multicenter prospective observational cohort study involving 14 research centers from 11 regions of Russia. The study included 188 patients: 177 (94%) with PMF, 10 patients with post-PV MF and 1 patient with post-ET MF. Men: women ratio – 1 : 1,3.

Results
The incidence of PMF in various regions of the Russia ranges from 0.05 to 2.12 per 100000 (Table 1, 2). Among patients with PMF, post-PV MF and post-ET MF, 44% (83) are patients in the age group 60-74 years, 28% (53) in the age group 45-59 years, 17% (32) - patients aged 18-44 years, 11% (20) - in the age group 75-90 years. Constitutional symptoms were recorded in the majority of patients: fatigue - 78% (147), lethargy - 65 (122), headache - 40% (75), discomfort in the stomach - 38% (72), discomfort in the left hypochondrium - 38% ( 71), night sweats - 32% (61), feeling of early satiety - 30% (56), weight loss - 26% (48), bone pain - 23% (43), sweating - 15% (28), skin itching - 11% (20), fever - 6% (12). No symptoms mediated by the disease - 5% (10). Splenomegaly at the time of diagnosis was recorded in 76% (143 patients). Molecular analysis performed at 94% of patients (176): JAKV617F mutation detected in 122 patients, JAK2exon12 - 1, CALR - 34, MPL - 7, triple negative variant - 13. Allelic burden of JAK2V617F defined in 90 patients with PMF, median - 34% (1 - 100%). Data on allelic burden JAK2V617F was in 7 patients with post-PV MF, the median was 57% (3 - 88%). Histological examination of bone marrow performed by 91% (171 patients). Histochemical studies with an assessment of the degree of reticulin fibrosis - 81% (153). Confirmation of the diagnosis according to the full set of criteria in patients with PMF registered in 54% of patients. The distribution of patients by risk groups in accordance with the IPSS prognostic scale: low - 26%, intermediate-1 - 33%, intermediate-1 - 23%, high - 18%. The first line of therapy: hydroxyurea - 58%, ruxolitinib - 14%, interferon-alpha - 10%, glucocorticosteroids - 5%, erythropoietin 3%, anagrelide - 1%, other therapy - 9%. Blood transfusions performed in 70% of patients, thromboconcentrate transfusions - 6%. Concomitant therapy is given to 63% (119 patients): acetylsalicylic acid - 78, allopurinol - 49, erythropoietin - 11, clopidogrel - 6, NSAID - 3, sulodexide - 3, tranexamic acid - 1, etamzilat - 1, other (dipyridamole, iron preparations , folic acid, vitamin B12) - 30. The efficacy of therapy assessed for 52 patients: complete clinical and hematological remission - 23% (12), partial clinical and hematological remission - 48% (25), stabilization of the disease - 29% (15). 5 deaths reported. The 2-year overall survival rate was 97% (Figure 1).

Conclusion
In general the registry of patients with PMF allows integrating information into a common system and obtaining information characterizing the population of patients with this disease in Russia.

Session topic: 16. Myeloproliferative neoplasms - Clinical

Keyword(s): Epidemiology, Myelofibrosis

By clicking “Accept Terms & all Cookies” or by continuing to browse, you agree to the storing of third-party cookies on your device to enhance your user experience and agree to the user terms and conditions of this learning management system (LMS).

Cookie Settings
Accept Terms & all Cookies