Adult Primary Myelodysplastic Syndrome: Experience from a Tertiary Care Center in Pakistan

Abstract

Background: Primary myelodysplastic syndrome (MDS) is an acquired clonal disorder of myeloid progenitor cells, characterized by peripheral cytopenias in the presence of hypercellular marrow with dysplastic features. Our aim was to study the demographical and clinicopathological features of adult Pakistani patients with MDS at disease presentation. Materials and Methods: This single centre study was conducted at Liaquat National Hospital and Medical College, extending from January 2010 to December 2014. Data were retrieved from the patient 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 age of 57.6±17.4 years and median of 64 years. The male to female ratio was 1.7:1. The main presenting complaints were generalized fatigue (60%), fever (33.3%), dyspnea (15.5%), bleeding (13.3%) and weight loss (11.1%). Examination was unremarkable in 42.2% of patients. Physical examination revealed pallor in 37.7%, followed by petechial and purpuric rashes in 20%. The commonest laboratory finding was anemia (hemoglobin < 10 g/dl in 41 (91.1%) patients. Out of these, 27 (60%) patients had normocytic anemia, followed by macrocytic (22.2%) and microcytic (8.8%). Conclusions: Primary MDS in Pakistani patients demonstrates a male preponderance. The proportion of anemic patients was high in our series with predominance of normocytic anemia. However, other clinico-hematological features appear comparable to published data.

Clinicohematological and Cytogenetic Profile of Myelodysplastic Syndromes in Pakistan – Compare and Contrast

“…The most common presenting complaint in our patients was loss of appetite followed by weakness. In our study mean Hb was 7.7 g/dl which is in concordance with a previous national study [1] and higher as compared to other studies done in Pakistan, China and India [2,6,8] and lower as compared to Turkey, Greece and Poland [9,10,15]. In our study, mean platelet count was 82 ×10 9 /l, which is in concordance with a national study and also seen in India [1,8].…”

“…MDS is generally considered a preleukemic disorder and is prevalent in elder individuals in western world [5]. However, median age at diagnosis in our study was younger which concurs with other studies done in Asia [1][2][3][4][6][7][8] as compared to the studies from west with higher median age in MDS (Greece, Germany, Poland) [10,14,15]. In our study we observed male predominance which is universally seen in this disorder [1][2][3][4][5][6][7][8].…”

“…2). Mean hemoglobin (Hb), total leukocyte count (TLC), platelets, MCV at baseline and subtypes of MDS as per WHO classification in comparison with other national and international studies is shown in Table 1 [1][2][3][6][7][8][9][10][11][12][13][14][15].…”

“…Myelodysplastic syndromes are characterized by ineffective hematopoiesis with blood cytopenias and morphological dysplasia [1][2][3][4][5]. MDS is generally considered a preleukemic disorder and is prevalent in elder individuals in western world [5].…”

“…Myelodysplastic syndromes (MDS) are group of clonal hematopoietic stem cell disorders exhibiting ineffective hematopoiesis, morphological dysplasia and progressive tendency to evolve into acute myeloid leukemia (AML) [1][2][3][4][5]. The exact pathogenesis is not completely understood [3].…”