Essential thrombocythemia - basic analyzes in children and adolescents

Маматкулова Феруза Хайдаровна

Journal of cardiorespiratory research/Kardiorespirator tadqiqotlar jurnali · 2023-yil

Annotatsiya

Essential thrombocythemia is an extremely rare disorder in childhood. This disease is characterized by a persistent increase in the peripheral blood platelet count, associated with a proliferation of atypical megakaryocytes in the bone marrow. Aim — to analyze the clinical features of the course of essential thrombocythemia (ET) and the response to therapy in pediatric and adolescent patients Materials and methods. Thirty-one patients with ET under the age of191 years were analyzed. All patients were diagnosed with ET in accordance with WHO criteria on the basis of an examination, including assessment of clinical data, laboratory tests (general clinical tests; morphological, genetic, and histological examinations of bone marrow), instrumental studies, and an assessment of response to treatment. The average age of disease onset was 9 years 9 months, with a median of 9 years 6 months. Organomegaly was recorded in 13 (52 %) patients, of whom 7 (37.5 %) had isolated splenomegaly and 6 (37.5 %) had hepatosplenomegaly. Bleeding was noted in 6 (19.4 %) patients with a deep decrease in vWF:RCo (no more than 15 %) and an extreme increase in platelets (PLT) (more than 2000 × 109 /L). Twelve (38.7 %) patients suffered from microcirculation disorders (headaches, dizziness, melalgia), half of them had a platelet count of 1000–2000 × 109 /L, which is comparable to asymptomatic patients. No thrombosis was registered in our group. The JAK2V617F mutation was detected in 3 (9.7 %) patients, a mutation in the CALR gene was found in 9 (29.0 %) of patients, there was a mutation in the MPL gene in one (3.2 %) patient, and in the remaining cases (18 (58.1 %) patients), there was no damage to typical driver genes. Translocation t(12;12) was revealed in 1 (3.2 %) patient. The response to one-component cytoreductive therapy (CR+PR) was found to be quite high in young patients and constituted about 70–80 %. The complete response rate (CR) was as follows, respectively: 42.9 % (3) — to anagrelide therapy (ANA), 47.4 % (9) — to interferon therapy (INF), and 0 % — to hydroxycarbamide (HU). However, HU was not used in the first line of therapy for the children in our group. In the pediatric population, ET patients are dominated by the group of “triple-negative” disease, which somewhat complicates the differential diagnosis with secondary thrombocytosis. Compared to the adult population, the risk of bleeding is higher for pediatric patients, which is associated with the large number of patients with extremely high levels of platelets. In the case of hemorrhagic syndrome development or microcirculatory disorders that cannot be stopped by taking antiplatelet agents, we recommend giving preference to INF and HU as fi rst-line therapy, due to the peculiarities of pharmacokinetics and the potential risk of progression of myelofi brosis during ANA therapy.

Maqola ma’lumotlari
MualliflarМаматкулова Феруза Хайдаровна
JurnalJournal of cardiorespiratory research/Kardiorespirator tadqiqotlar jurnali
NashriyotСамарқанд давлат тиббиёт университети
Nashr sanasi2023-10-31
Jild4
Son3
TilIngliz
Ilmiy sohaTibbiyot
Indeksga qo‘shilgan2026-08-26

Maqolaning asl sahifasi

Kalit so‘zlar

эссенциальная тромбоцитемия, дети, подростки, молодой возраст, активность фактора Виллебранда, хронические миелопролиферативные новообразования, JAK2, MPL, CALR, essential thrombocythemia, children, adolescents, young age, von Willebrand factor activity, chronic myeloproliferative neoplasms, JAK2, MPL, CALR, essenтsial trombotsitemiya, bolalar, o‘smirlik, fon Villebrand omil faolligi, surunkali mieloproliferativ kasallik, JAK 2, MPL, CALR

Ilmiy soha

Tibbiyot

Iqtibos keltirish

APAМаматкулова Феруза Хайдаровна (2023). ESSENTIAL THROMBOCYTHEMIA - BASIC ANALYZES IN CHILDREN AND ADOLESCENTS. Journal of cardiorespiratory research/Kardiorespirator tadqiqotlar jurnali, 4(3). https://www.tadqiqot.uz/index.php/cardio/article/view/8241
GOST (OAK)Маматкулова Феруза Хайдаровна ESSENTIAL THROMBOCYTHEMIA - BASIC ANALYZES IN CHILDREN AND ADOLESCENTS // Journal of cardiorespiratory research/Kardiorespirator tadqiqotlar jurnali. – 2023. – T. 4, № 3.
MLAМаматкулова Феруза Хайдаровна. “ESSENTIAL THROMBOCYTHEMIA - BASIC ANALYZES IN CHILDREN AND ADOLESCENTS.” Journal of cardiorespiratory research/Kardiorespirator tadqiqotlar jurnali, 4(3), 2023. https://www.tadqiqot.uz/index.php/cardio/article/view/8241
ChicagoМаматкулова Феруза Хайдаровна. “ESSENTIAL THROMBOCYTHEMIA - BASIC ANALYZES IN CHILDREN AND ADOLESCENTS.” Journal of cardiorespiratory research/Kardiorespirator tadqiqotlar jurnali, 4(3) (2023). https://www.tadqiqot.uz/index.php/cardio/article/view/8241
HarvardМаматкулова Феруза Хайдаровна (2023) ‘ESSENTIAL THROMBOCYTHEMIA - BASIC ANALYZES IN CHILDREN AND ADOLESCENTS’, Journal of cardiorespiratory research/Kardiorespirator tadqiqotlar jurnali, 4(3). https://www.tadqiqot.uz/index.php/cardio/article/view/8241
BibTeX
@article{article2023,
  title = {ESSENTIAL THROMBOCYTHEMIA - BASIC ANALYZES IN CHILDREN AND ADOLESCENTS},
  author = {Маматкулова Феруза Хайдаровна},
  journal = {Journal of cardiorespiratory research/Kardiorespirator tadqiqotlar jurnali},
  year = {2023},
  volume = {4},
  number = {3},
  url = {https://www.tadqiqot.uz/index.php/cardio/article/view/8241}
}

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