Advanced SearchSearch Tips
Spectrum of the WHO Classification De Novo Myelodysplastic Syndrome: Experience from Southern Pakistan
facebook(new window)  Pirnt(new window) E-mail(new window) Excel Download
 Title & Authors
Spectrum of the WHO Classification De Novo Myelodysplastic Syndrome: Experience from Southern Pakistan
Sultan, Sadia; Irfan, Syed Mohammed; Jawed, Syeda Narisa;
  PDF(new window)
Background: Myelodysplastic syndrome (MDS) is a clonal disorder of hemopoeitic stem cells, characterized by infective hematopoiesis, peripheral cytopenias along with hypercellularity of marrow and marked dysplastic features. Our aim was to study the spectrum of the WHO classification in adult Pakistani patients with MDS at disease presentation. Materials and Methods: This retrospective descriptive study was conducted at Liaquat National Hospital and Medical College, extending from January 2010 to December 2014. Patient data were retrieved from the maintained archives. Results: Overall, 45 patients were diagnosed at our institution with de novo MDS during the study period. There were 28 males and 17 females. Age ranged between 18 and 95 years with a mean of . The male to female ratio was 1.7:1. According to the WHO classification, 53.3% had refractory cytopenia with multilineage dysplasia, 22.2% had refractory cytopenia with unilineage dysplasia, 4.4% each had refractory anemia with excess of blasts-1 and II and 15.5% had MDS unclassified. The main presenting complaints were generalized fatigue (60%), fever (33.3%), dyspnea (15.5%), bleeding (13.3%) and weight loss (11.1%). Physical examination revealed pallor in 37.7%, followed by petechial and purpuric rashes in 20% of patients. Hemoglobin was <10 g/dl in 41 (91.1%). Pancytopenia and bicytopenia were noted in 18 (40%) and 14 (31.1%) respectively. Conclusions: MDS in our patients presents at a relatively young age. Refractory c ytopenia with multilineage dysplasia was the dominant disease variant in our setting.
Myelodysplastic syndrome;WHO classification;adults;Pakistan;
 Cited by
Akiba M, Matsuda A, Misumi M, ET AL (2001). Clinical significance of WHO classification and MDS 2000 classification in myelodysplastic syndromes. Rinshu Ketsuk, 42, 162-9.

Akinci S, Silay K, Ulas A, et al (2014). Neutrophil to lymphocyte ratio--not an independent prognostic factor in patients with the myelodysplastic syndrome. Asian Pac J Cancer Prev, 15, 10883-5.

Bernasconi P, Klersy C, Boni M, et al (2005). Incidence and prognostic significance of karyotype abnormalities in de novo primary myelodysplastic syndromes: a study on 331 patients from a single institution. Leukemia, 19, 1424-31. crossref(new window)

Brunning RD, Orazi A, Germing U, Le Beau MM (2008). Myelodysplastic syndromes/neoplasms, overview. In: Swerdlow HS, Campo E, Haris LN, et al. WHO Classification of Tumours of haemopoietic and lymphoid tissues. Lyon: International agency for research on cancer, 89.

Chevassut TJ, Mufti GJ (2011). The myelodysplastic syndromes. In:Hoffbrand AV, Catovsky D, Tuddenham EG, Green AR. Post Graduate Hematology, 6th edition, 503.

Chaubey R, Sazawal S, Dada R, Mahapatra M, Saxena R (2011). Cytogenetic profile of Indian patients with de novo myelodysplastic syndromes. Indian J Med Res, 134, 452-7.

Chatterjee T, Dixit A, Mohapatra M, et al (2004). Clinical, haematological and histomorphological profile of adult myelodysplastic syndrome: study of 96 cases in a single institute. Eur J Haematol, 73, 93-7. crossref(new window)

de Hollanda A, Beucher A, Henrion D, et al (2011). Systemic and immune manifestations in myelodysplasia: a multicenter retrospective study. Arthritis Care Res, 63, 1188-94. crossref(new window)

Ehsan A, Aziz M (2010). Clinico-haematological characteristics in Pakistani patients of primary myelodysplastic syndrome according to World Health Organization classification. J Coll Physicians Surg Pak, 20, 232-6.

Fenaux P, Haase D, Sanz GF, et al(2014). ESMO Guidelines Working Group. Myelodysplastic syndromes: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol, 25, 57-69. crossref(new window)

Gao S, Li Z, Fu JH, et al (2015). Decitabine in the treatment of acute myeloid leukemia and myelodysplastic syndromes, which combined with complex karyotype respectively. Asian Pac J Cancer Prev, 16, 6627-32. crossref(new window)

Irfan M, Kakepoto GN, Khursheed M (1998).Primary myelodysplastic syndrome: clinical spectrum of 53 cases. J Pak Med Assoc, 48, 69-73.

Kar R, Rao S, Saxena R (2009). Myelodysplastic syndromes: classification and prognostic scoring systems and their applicability in Indian scenario-experience from a tertiary care center. Hematol, 14, 145-9. crossref(new window)

Malcovati L, Hellstrom-Lindberg E, Bowen D, et al (2013). Diagnosis and treatment of primary myelodysplastic syndromes in adults: recommendations from the European Leukemia Net. Blood, 122, 2943-64. crossref(new window)

Newman K, Maness-Harris L, El-Hemaidi I, Akhtari M (2012). Revisiting use of growth factors in myelodysplastic syndromes. Asian Pac J Cancer Prev, 13, 1081-91. crossref(new window)

Neukirchen J, Schoonen WM, Strupp C, et al (2011). Incidence and prevalence of myelodysplastic syndromes: data from the Dusseldorf MDS-registry. Leuk Res, 35, 1591-6. crossref(new window)

Rodrigues EF, de Souza DC, Camargo A, et al (2007). Cytogenetic biclonality in a child with hypocellular primary myelodysplastic syndrome. Cancer Genet Cytogenet, 178, 70-2. crossref(new window)

Rashid A, Khurshid M, Shaikh U, Adil S (2014). Chromosomal abnormalities in primary myelodysplastic syndrome. J Coll Physicians Surg Pak, 24, 632-5.

Shah NM, Prajapati SG, Adesara RP, Patel AP (2009). An analysis of 30 cases of myelodysplastic syndrome. Indian J Pathol Microbiol, 52, 206-9. crossref(new window)

Wang XQ. Sino-US Shanghai Leukemia Cooperative Group (2008).WHO classification and cytogenetic analysis of 435 cases with myelodysplastic syndrome. Zhonghua Nei Ke Za Zhi, 47, 464-7.

Yan X, Wei J, Wang J, et al (2015). Analysis of the karyotype abnormalities and its prognostic in 298 patients with myelodysplastic syndrome. Zhonghua Xue Ye Xue Za Zhi, 36, 297-301.