Myelodysplastic Syndrome

.: CHAPTERS
Miyelodisplastik Sendromda Klinik: Tanı, Ayırıcı Tanı ve Sınıflandırma
Clinical Features of Myelodysplastic Syndrome: Diagnosis, Differantial Diagnosis and Classification
Fahir ÖZKALEMKAŞa
aBursa Uludağ Üniversitesi Tıp Fakültesi, Hematoloji BD, Bursa, TÜRKİYE
Özkalemkaş F. Miyelodisplastik sendromda klinik: Tanı, ayırıcı tanı ve sınıflandırma. Çetiner M, editör. Miyelodisplastik Sendrom. 1. Baskı. Ankara: Türkiye Klinikleri; 2021. p.1-7.
Article Language: TR
ÖZET
Miyelodisplastik sendromlar (MDS), displastik hematopoez ve değişen derecelerde akut miyeloid lösemi (AML)'ye dönüşüm riski ile karakterli heterojen bir grup klonal hematopoetik kök hücre bozukluğunu ifade eder. İnsidansı yılda 100.000'de yaklaşık 4,9'dur. Tanıda ortanca yaş 70-75'dir. MDS'nin belirti ve bulguları nonspesifiktir. Birçok hastada tanı sırasında ya çok az semptom olur ya da hiç semptom bulunmaz, bu olgular rutin kan sayımlarında bulunan anormallikler nedeniyle tanı alırlar. Diğerleri açıklanamamış sitopenilerin yol açtığı semptomlarla gelirler. Anemi tanıda neredeyse tüm olgularda vardır; lökopeni hastaların yaklaşık %50'sinde, trombositopeni kabaca %25'inde bulunur. Klinisyenler, sitopenilerin zamanı, şiddeti ve hızı, önceki enfeksiyonlar ve kanama epizotları ve transfüzyonları içeren bir anemnez almalıdırlar. Çevre kanının değerlendirilmesi tanıda anahtar unsurlardan biridir; çevre kanı incelemesinde eritrositlerde anizositoz ve hafif makroovalositoz, nötrofillerde hipogranülasyon ve hiposegmentasyon gibi displazi bulguları veya az sayıda miyeloblast saptanabilir. Kemik iliği aspirasyon ve biyopsisi hematopoetik hücre dizilerindeki maturasyon anormalliklerinin derecesini, kemik iliği blast oranını, kemik iliği sellülaritesini, ring sideroblastların bulunup bulunmadığını ve fibrozisi değerlendirmek için gereklidir. MDS'de sitogenetik büyük öneme sahiptir, standart karyotipik yöntemlerle elde edilemezse MDS ilişkili bir floresan in situ hibridizsayon (FİSH) paneli yapılmalıdır. Karyotip normalse bazı somatik mutasyonları saptamak içim moleküler testlerin yapılması düşünülmelidir. Akım sitometri bulguları MDS için tanısal olarak düşünülmemektedir, fakat akım sitometri bulguları şüpheli olgularda tanıyı destekleyebilir veya ayırıcı tanıda yararlı olabilir. MDS, indolen miyeloid bozukluklar, miyelodisplastik/miyeloproliferatif neoplazi (MDS/MPN) sendromları, akut lösemiler, aplastik anemi, miyelofibroz, megaloblastik anemiler ve bazı ilaç etkileri vb. displazi ve/veya sitopeni ile prezente olan bozukluklardan ayrılmalıdır. Bugünkü MDS WHO sınıflaması son olarak 2016 yılında güncellenmiştir. Bu sınıflandırma 6 antite tanımlamaktadır: tek seri displazili MDS (MDS-SLD); ring sideroblastlı MDS (MDS-RS); artmış blastlı MDS (MDS-EB); çoklu dizge displazili MDS (MDS-MLD); izole del(5q)'lu MDS+-7/del7q hariç diğer bir anormalik ve sınıflandırılamayan MDS (MDS-U).

Anahtar Kelimeler: Miyelodisplastik sendromlar; anemi, dirençli; lösemi, miyeloid, akut
ABSTRACT
The myelodysplastic syndromes (MDS) express a heterogeneous group of clonal hematopoietic stem cell disorders characterized by dysplastic hematopoesis and a variable risk of transformation to acute myeloid leukemia (AML). The incidence rate of MDS is approximately 4.9 per 100.000 people per year. Median age at diagnosis is 70- 75 years. Signs and symptoms of MDS are nonspesific. Many patients have minimal or no symptom at time of diagnosis; they are diagnosed based upon abnormalities found on routine blood counts. Other patients present with symptoms arise from unexplained cytopenias. Anemia is almost uniformly found at diagnosis; leukopenia are present approximately 50 persent of the patients; and thrombocytopenia are present roughly 25 persent of all cases. Clinicians should have a history of the patient which include timing, severity and tempo of cytopenias, prior infections and bleeding episodes, and transfusions. Evaluation of peripheral blood smear is a key component of diagnosis which usually demonstrates evidence of dysplasia like anisocytosis, mild macro-ovalocytosis of red blood cells and hyposegmentation and hypogranulation of neutrophils or it revealed rare myeloblasts. Bone marrow aspiration and biopsy are needed to evaluate the degree of maturation abnormalities of hematopoetic cell lineages, percentage of marrow blasts, marrow cellularity, presence or absence of ring sideroblasts and fibrosis. Cytogenetics are of major prognostic importance in MDS; if it can not be obtained by standart karyotyping methods a MDS-related fluorescence in situ hybridization (FISH) panel should be performed. If karyotype is normal molecular tests should be considered to detect some somatic mutations. Flow cytometry findings are not considered diagnostic for MDS, but they can provide further support for the diagnosis in suspected cases and they can be usefull for differantial diagnosis. MDS must be distinguished from other disorders that may also present cytopenias and/or dysplasia such as indolen myeloid disorders, myelodysplastic/myeloproliferative neoplasms (MDS/MPN) syndromes, acute leukemia, aplastic anemia, myelofibrosis, megaloblastic anemias, and some drug effects etc. The current WHO classification of MDS has been updated lastly in 2016. This diagnostic classification identify six entities: MDS with single lineage dysplasia (MDS-SLD); MDS with ring sideroblasts (MDS-RS); MDS with excess blast (MDS-EB); MDS with isolated del(5q )+one other abnormality except - 7/del(7q) and MDS unclassifiable (MDS-U).

Keywords: Myelodysplastic syndromes; anemia, refractory; leukemia, myeloid, acute

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