ISSN: 1300-7777 E-ISSN: 1308-5263
Refractory Thrombocytopenia with Multilineage Dysplasia: A Rare Type of Myelodysplastic Syndrome [Turk J Hematol]
Turk J Hematol. 2003; 20(1): 25-30

Refractory Thrombocytopenia with Multilineage Dysplasia: A Rare Type of Myelodysplastic Syndrome

Fatih Demırkan1, G. Hayri Özsan1, Mehmet Ali Özcan1, Filiz Vural1, İlhan Öztop1, Erdinç Yüksel2, Bülent Ündar1
1Division Of Hematology-oncology, Department Of Internal Medicine, School Of Medicine, Dokuz Eylül University, İzmir, Turkey
2Department Of Cytogenetics And Molecular Biology, School Of Medicine, Dokuz Eylül University, İzmir, Turkey

Thrombocytopenia may be the presenting cytopenia of myelodysplastic syndrome (MDS) and is named as refractory thrombocytopenia (RT) and categorized in the refractory cytopenia with multilineage dysplasia (RCMD) group according to the recent World Health Organization (WHO) classification of the acute leukemias and MDS. Abnormal cytogenetics can be found in 60% to 80% of patients with MDS. Most common cytogenetic abnormalities include monosomy 5, 5q-, monosomy 7, trisomy 8, deletion 20q and loss of X or Y chromosome. Here we report clinical features and outcomes of nine patients with RT. Cytogenetic abnormalities were detected in seven. Among two patients who have a normal karyotype at diagnosis, one of them transformed to acute myeloid leukemia (AML). During a median follow-up of 29 months, two patients died of hemorrhagia and one of AML. The features and prognosis of patients with RT needs to be determined by larger series.

Keywords: Myelodysplastic syndrome, Refractory thrombocytopenia, Refractory cytopenia with multilineage dysplasia, Cytogenetics.


Çok Dizinli Displaziye Eşlik Eden Refrakter Trombositopeni: Seyrek Rastlanan Bir Myelodisplastik Sendrom Tipi

Fatih Demırkan1, G. Hayri Özsan1, Mehmet Ali Özcan1, Filiz Vural1, İlhan Öztop1, Erdinç Yüksel2, Bülent Ündar1
1Division Of Hematology-oncology, Department Of Internal Medicine, School Of Medicine, Dokuz Eylül Universitesi, İzmir, Turkiye
2Department Of Cytogenetics And Molecular Biology, School Of Medicine, Dokuz Eylül Universitesi, İzmir, Turkiye

Trombositopeni myelodisplastik sendromu (MDS)’nun ilk bulgusu olabilir ve refrakter trombositopeni (RT) adı altında Dünya Sağlık Örgütü (WHO)’nün akut lösemi ve MDS sınıflandırmasında multilinaj displaziıe e.lik eden refrakter sitopeni (RCMD) grubuna dahil edilmektedir. MDS’li olguların %60-80’inde anormal sitogenetik bulgular olur. En sık görülen sitogenetik anomaliler arasında monozomi 5, 5q-, monozomi 7, trizami 8, 20q delesıonu ve X ya da Y kromozom kaybı vardır. Biz bu yazıda RT’li dokuz olgunun klinik özelliklerini bildirdik. yedi olguda sitogenetik anomali saptandı. Tanı sırasında normal karyotipe sahip iki olgudan bir tanesi AML’ye dönüştü. Median 29 aylık bir izleme sonunda, iki olgu hemorajiden kaybedilirken, bir olgu AML’ye dönüşerek kaybedildi. RT’li olguların özellikleri ve prognozlarının daha geniş serilerde değerlendirilmesi gerekmektedir.

Anahtar Kelimeler: Myelodisplastik, Refrakter trombositopeni, Refrakter sitopeni, Sitogenetik. Turk J Haematol 2003, 20(1): 25-30


Fatih Demırkan, G. Hayri Özsan, Mehmet Ali Özcan, Filiz Vural, İlhan Öztop, Erdinç Yüksel, Bülent Ündar. Refractory Thrombocytopenia with Multilineage Dysplasia: A Rare Type of Myelodysplastic Syndrome. Turk J Hematol. 2003; 20(1): 25-30

Corresponding Author: Fatih Dem&253;rkan, Türkiye


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