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. 2018 May 25;35(2):109-115.
doi: 10.4274/tjh.2017.0130. Epub 2017 Jun 7.

Myelodysplastic Syndrome in Pakistan: Clinicohematological Characteristics, Cytogenetic Profile, and Risk Stratification

Rafia Mahmood  1 , Chaudry Altaf  1 , Parvez Ahmed  1 , Saleem Ahmed Khan  1 , Hamid Saeed Malik  1
Affiliations

Myelodysplastic Syndrome in Pakistan: Clinicohematological Characteristics, Cytogenetic Profile, and Risk Stratification

Rafia Mahmood et al. Turk J Haematol. .

Abstract
in English, Turkish

Objective: Myelodysplastic syndrome (MDS) is a group of bone marrow diseases that not only have variable morphological presentation and heterogeneous clinical courses but also have a wide range of cytogenetic abnormalities. Clinicohematological parameters have a significant role in diagnosis and along with identification of cytogenetic abnormalities are important for prognostic scoring and risk stratification of patients to plan management and make treatment decisions. This study aimed to determine the clinicohematological characteristics, cytogenetic abnormalities, and risk stratification of newly diagnosed de novo MDS patients.

Materials and methods: This cross-sectional study was conducted in the Department of Hematology, Armed Forces Institute of Pathology, Rawalpindi, from January 2013 to January 2017. Patients were diagnosed on the basis of World Health Organization criteria for MDS, clinicohematological parameters were noted, and cytogenetic analysis was performed. Risk stratification was done using the Revised International Prognostic Scoring System.

Results: A total of 178 cases of MDS were analyzed, including 119 males (66.9%) and 59 females (33.1%). The median age was 58 years. The most common presenting feature was anemia in 162 (91%) of the patients. MDS with multilineage dysplasia was the most common diagnosis, seen in 103 (57.9%) patients. A normal karyotype was seen in 95 (53.4%), while 83 (46.6%) showed clonal karyotypic abnormalities at diagnosis. Of these, the common abnormalities found were trisomy 8, complex karyotype, and del 5q. Risk stratification revealed low-risk disease in 73 (41%) patients.

Conclusion: Cytogenetic analysis showed the normal karyotype to be the most common while risk stratification revealed a predominance of low-risk disease at the time of presentation.

Amaç: Myelodisplastik sendrom (MDS) sadece değişken morfolojik prezentasyona ve heterojen klinik seyre değil geniş sitogenetik anormallikler yelpazesine de sahip olan bir grup kemik iliği hastalığıdır. Klinikohematolojik parametreler tanıda önemli role sahiptir ve sitogenetik anormalliklerin tanımlanması ile birlikte prognostik skorlamada ve yönetimi planlamak ve tedavi kararlarını vermek için risk stratifikasyonunda önemlidir. Bu çalışma, yeni tanı de novo MDS hastalarında klinikohematolojik özellikler, sitogenetik anormallikler ve risk stratifikasyonunu belirlemeyi amaçlamıştır. Gereç ve Yöntemler: Bu kesitsel çalışma Rawalpindi Silahlı Kuvvetler Patoloji Enstitüsü Hematoloji Departmanı’nda Ocak 2013’ten Ocak 2017 tarihine kadar sürdürülmüştür. Hastalar Dünya Sağlık Örgütü MDS kriterlerine göre teşhis edildi, klinikohematolojik parametreler not edildi ve sitogenetik analiz yapıldı. Risk stratifikasyonu Revize Uluslararası Prognostik Skorlama Sistemi kullanılarak yapıldı. Bulgular: Toplam 178 MDS olgusu, 119 erkek (%66,9) ve 59 kadın (%33,1) analiz edildi. Medyan yaş 58 idi. Başvuruda en sık görülen belirti olguların 162’sinde (%91) anemi idi. En sık tanı MDS çoklu seride displazi olup 103 (%57,9) hastada görüldü. Teşhiste normal karyotip 95 (%53,4) olguda görülürken 83 (%46,6) olgu klonal karyotipik anormallikler gösterdi. Bunlar arasında, en sık görülenler trizomi sekiz, kompleks karyotip ve del5q idi. Risk stratifikasyonu 73 (%41) hastada düşük-risk hastalık ortaya koydu. Sonuç: Sitogenetik analiz en sık normal karyotipi gösterirken risk stratifikasyonu tanı sırasında düşük-risk hastalığın çoğunlukta olduğunu ortaya koymuştur.

Keywords: Cytogenetics Revised International Prognostic Scoring System.; Myelodysplastic syndrome.

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Conflict of interest statement

Conflict of Interest: The authors of this paper have no conflicts of interest, including specific financial interests, relationships, and/or affiliations relevant to the subject matter or materials included.

Figures

Figure 1
Figure 1. Cytogenetics of the patients.
Figure 2
Figure 2. Common karyotypic abnormalities of the patients.
See this image and copyright information in PMC

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