Patients with Chronic Myeloid Leukemia Diagnosed with Predominant Thrombocytosis without Marked Leukocytosis: Case Series

Öz

Chronic myeloid leukemia (CML) is a clonal hematopoietic pluripotent stem cell disease characterized by excessive and uncontrolled proliferation of myeloid lineage cells. Platelet count is increased in more than half of patients and the appearance of platelets is variable. When patients present with predominant thrombocytosis without marked leukocytosis, they should be tested for the Philadelphia chromosome or (breakpoint cluster region- Abelson) BCR-ABL to distinguish cases of CML. In this study, the data of 215 patients diagnosed with CML between 2010 and 2023 were retrospectively evaluated. The study enrolled patients aged ≥18 years with leukocyte count <40 x109/L and platelet count >500 x109/L at the time of diagnosis. While investigating the etiology of predominant thrombocytosis and no significant leukocytosis, 13 patients diagnosed with CML were identified. The proportion of these patients among all patients with CML was 6%. This study showed that CML should be considered in the differential diagnosis of patients with predominant thrombocytosis without marked leukocytosis. In these patients, the Ph chromosome should definitely be checked before ET is diagnosed. Making a correct diagnosis in this patient group is important in order to start tyrosine kinase inhibitor treatment before it progresses to accelerated or blastic phases.

Anahtar Kelimeler

• chronic myeloid leukemia
• myeloproliferative disorders
• essential thrombocythemia
• thrombosis
• tyrosine kinase inhibitor

Belirgin Lökositoz Olmaksizin Baskin Trombositozla Tani Alan Kronik Miyeloid Lösemili Hastalar: Vaka Serisi

Öz

Kronik miyeloid lösemi (KML), miyeloid seri hücrelerinin aşırı ve kontrolsüz çoğalmasıyla karakterize klonal hematopoietik pluripotent bir kök hücre hastalığıdır. Hastaların yarısından fazlasında trombosit sayısı artmıştır ve trombositlerin görünümü değişkendir. Hastalarda belirgin lökositoz olmaksızın baskın trombositoz mevcutsa, KML vakalarını ayırt etmek için Philadelphia kromozomu veya (breakpoint cluster region -Abelson) BCR-ABL testi yapılmalıdır. Bu çalışmada 2010-2023 yılları arasında KML tanısı almış 215 hastanın verileri retrospektif olarak değerlendirildi. Çalışmaya tanı anında lökosit sayısı <40 x109/L ve trombosit sayısı >500 x109/L olan ≥18 yaş hastalar dahil edildi. Belirgin lökositoz olmadan baskın trombositozla tetkik edilirken KML tanısı alan 13 hasta belirlendi. Bu hastaların tüm KML hastaları içindeki oranı %6 bulundu. Bu çalışma, belirgin lökositoz olmaksızın baskın trombositozu olan hastaların ayırıcı tanısında KML'nin düşünülmesi gerektiğini göstermektedir. Bu hastalarda ET tanısı konulmadan önce mutlaka Ph kromozomuna bakılmalıdır. Bu hasta grubunda doğru tanı koymak, hastalığın akselere veya blastik faza ilerlemeden tirozin kinaz inhibitörü tedavisine başlanması açısından önemlidir.

Anahtar Kelimeler

• kronik miyeloid lösemi
• miyeloproliferatif hastalık
• esansiyel trombositoz
• tromboz
• triozin kinaz inhibitörü

Kaynakça

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