Myelodysplastic Syndrome

.: PREFACE
ÖN SÖZ
PREFACE
Prof. Dr. Mustafa ÇETİNER
Acıbadem Maslak Hastanesi, Hematoloji Kliniği, İstanbul, Türkiye
Article Language: TR
Miyelodisplastik Sendrom (MDS), özellikle yaşlı popülasyonda en sık rastlanan hematolojik malignitelerden biridir.

MDS bir ileri yaş hastalığıdır. MDS tanısı alan hastaların %86'sı 60 yaş ve üstüdür. Yapılan çalışmalar, hastaların sadece %6'sının 50 yaşın altında olduğunu göstermektedir.

Modern çağda ortalama yaşam süresinin uzaması ile MDS sıklığı önümüzdeki yıllarda daha da artacaktır.

MDS hastalığının insidans ve prevalansı, tanı sırasında genellikle gözden kaçması ve ileri ülkelerde bile düzenli kayıt sistemlerinin bulunmaması nedeniyle net olarak bilinmiyor.

ABD'de yılda 12.000-20.000, dünya genelinde ise her yıl 87.000 hasta MDS tanısı alıyor. Günümüzde sadece ABD'de 170.000 MDS hastasının yaşadığı tahmin ediliyor.

MDS bir klonal hematopoetik kök hücre hastalığıdır. Etkisiz hematopoezis ve displazi ile karakterize bir seyre sahip olan MDS hastalığı, oldukça heterojen bir klinik fenotipi sergiler. Bu kompleks seyir nedeniyle günümüzde kullanılan prognostik skorlama sistemleri prognoz öngörmede çoğu zaman son derece yetersiz kalmaktadır.

Buna ek olarak tedavi yanıtlarında da çok büyük farklılıklar izlenebilmektedir. MDS'nin bir ileri yaş hastalığı olması, aynı zamanda hastaların büyük bölümünün kırılgan ve ciddi komorbiditelere sahip olduğu anlamına da gelir.

Son yıllarda yapılan çalışmalar MDS'nin sadece bir kök hücre hastalığı değil, aynı zamanda kemik iliği mikroçevresinin de MDS patogenezinde önemli rol oynadığını ortaya koydu.

Hem hastalığın heterojen yapısı, hem de kemik iliği mikroçevresinin patogenezde önemli rol alması, hayvan ve laboratuvar çalışmalarında modellemeler yapmayı neredeyse imkânsız hâle getirmektedir.

Bu zorluklar elbette hastalığın tedavisindeki gelişmeleri de olumsuz etkilemektedir.

Yukarıda saydığım sebeplerden dolayı ilaç sektörünün hastalığa uzak durması onu neredeyse yalnız ve çaresiz bir hastalık hâline getirmiştir.

MDS tedavisinde, 2000'li yılların başından itibaren yakın bir geçmişe kadar sadece lenalidomid ve hipometile edici iki ilaç (azasitidin ve desitabin) FDA onayı alabildi. En son 2006 yılında desitabin onay aldıktan sonra, 2020 yılına kadar hiçbir yeni tedavi seçeneği FDA'dan onay alamadı.

Ancak son yıllarda durum değişiyor. 2020 yılında hem luspatersept, hem de desitabin/sedazuridin tedavisi FDA onayı aldı.

Özellikle genetik alanındaki baş döndürücü ilerlemeler, hastalığı daha iyi anlamamıza, prognoz kestirimini daha iyi yapmamıza ve yeni tedavi seçeneklerini denememize olanak verdi. Günümüzde MDS alanında yepyeni bir çağın eşiğinde duruyoruz.

Türkiye Klinikleri tarafından basılan bu kitabı, MDS'deki temel konuları ve yeni gelişmeleri sizlere aktarmak için hazırladık.

Kitabın hazırlanmasında katkılarından dolayı Türkiye Klinikleri'ne ve tüm yazar kadrosuna en içten dileklerimi sunarım.

Prof. Dr. Mustafa ÇETİNER
Editör
Myelodysplastic Syndrome (MDS) is one of the most common hematological malignancies, especially in the elderly population. MDS is a disease of the elderly and 86% of patients diagnosed as MDS are over 60 years of age. Studies show that only 6% of MDS patients are under the age of 50.

With the increase in life expectancy in the modern times, the frequency of MDS will be expected to increase more in the forthcoming years.

The incidence and prevalence of MDS are not exactly known because they are often overlooked at the time of diagnosis and there is no proper registry system and database even in the developed countries.

Every year, 12,000 up to 20,000 patients in the USA and 87,000 patients worldwide are diagnosed with MDS. It is estimated that 170,000 MDS patients has been living only in the USA.

MDS is a clonal hematopoietic stem cell disease. MDS characterized by an ineffective hematopoiesis and dysplasia, exhibits a highly heterogeneous clinical phenotype. Due to its complex course, the prognostic scoring systems commonly used in clinical practice are usually inadequate to predict the exact prognosis.

In addition, in terms of treatment response, a huge difference can be observed among patients. Since MDS is a disease of the elderly, it means that the majority of patients are fragile and have serious and multiple comorbidities.

Studies conducted in the recent years have revealed that MDS is not only a stem cell disease, but also the bone marrow microenvironment plays an important role in the pathogenesis of the MDS.

Both the heterogeneous nature of the disease and the important role of the bone marrow microenvironment in the pathogenesis make it almost impossible to model in animal and laboratory studies.

Of course, these difficulties negatively affect the developments in the treatment of the disease. Because of the reasons I listed above, the pharmaceutical companies are not willing to conduct a research and costly clinical trials in MDS. This has caused MDS to be almost an orphan and desperate disease.

From the treatment perspective, only lenalidomide and two hypomethylating agents (azacitidine and decitabine) were approved by the FDA since the early 2000s. After the last approval of decitabine in 2006, no new treatment option was approved by the FDA until 2020.

However, this gloomy situation has started to change in a positive way in the last couple of years. In 2020, both luspatercept and decitabine/cedazuridine treatments were approved by FDA which was a promising sign.

Especially the immense advances in the era of genetics have allowed us to understand the disease, predict prognosis and develop new treatment options much better. Today, we stand on the brink of a new era in the MDS.

We have prepared this book printed by Türkiye Klinikleri for you to document basic concepts and new developments in MDS.

I would like to thank to Türkiye Klinikleri and all writers for their priceless and outstanding efforts.

Prof. Dr. Mustafa ÇETİNER
Editor

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