Trombositozun Bir Nedeni; 5q Sendromu

Yıl 2021, Cilt: 7 Sayı: 1, 155 - 157, 01.01.2021

Elif Selvıoğlu İrfan Yavaşoğlu

Öz

Miyelodisplastik sendromlar MDS periferik kanda değişik derecelerde sitopeni, hematopoetik hücrelerde morfolojik ve fonksiyonel anormalliklerle karakterize, akut lösemiye ilerleme riski yüksek, kemik iliğinin klonal bir grup hematolojik malignitesidir. Trombositoz; trombosit sayısının 450.000/mm3 üzerinde olması olarak tanımlanır. Aşırı trombositoz ise trombosit sayısının 1.000.000/mm3olmasıdır. Miyelodisplastik sendromlar çoğunlukla sitopeni ile görülmektedir. Trombositoz, MDSʼde trombositopeniden daha az görülür. Trombositoz; 5q sendromunda, 3q21q26 sendromunda ve MDS / MPNʼde, genellikle JAK2ʼde ve ayrıca SF3B1ʼin aktive edici mutasyonları ile ilişkili halka sideroblastları ve trombositozu daha önce RARS-T olarak adlandırılır bulunan MDS / MPNʼde tarif edilmiştir. Burada aşırı trombositozun bir nedeninin de 5q sendromu olabileceğini vurgulamak üzere olgumuzu yayınlamak istedik

Anahtar Kelimeler

Miyelodisplastik sendromlar, 5q sendrom, Trombositoz, Anemi, Displazi

A Cause of Thrombocytosis; 5q Syndrome

Öz

Myelodysplastic syndromes MDS are hematological malignancies of bone marrow with a high risk of progression to acute leukemia and characterized by morphological and functional abnormalities in hematopoietic cells. thrombocytosis; is defined as the number of platelets above 450.000/mm3. Excessive thrombocytosis is a platelet count of 1.000.000/mm3. Myelodysplastic syndromes are mostly presented with cytopenia. Thrombocytosis is less commonly seen in MDS than thrombocytopenia. Thrombocytosis has been described in 5q- syndrome, 3q21q26 syndrome, and in MDS/MPN with ring sideroblasts and thrombocytosis previously called RARS-T which is often associated with activating mutations in JAK2 as well as SF3B1. We wanted to publish our case to emphasize that 5q syndrome may be a cause of extrem thrombocytosis

Anahtar Kelimeler

Myelodysplastic syndromes, 5q syndrome, Thrombocytosis, Anemia, Dysplasia

Kaynakça

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