Contributions
Abstract: PB2108
Type: Publication Only
Background
Myelodysplastic syndromes (MDS) represent a diverse group of hematopoietic stem cell disorders characterizing ineffective hematopoiesis, morphological dysplasia and leukemic transformation. Although the international prognostic scoring system (IPSS) represents gold standard for risk assessment, it is still difficult to predict survival and median time to leukemic transformation in the majority. MDS being considered a relatively uncommon disorder in our region having no national registry for evaluation of disease burden is presumed to be an incurable disease with majority of patients conventionally being treated by only blood product support. Most of the patients are unable to avail standard treatment and those who are treated with recommended options are presumed to have hopeless outcomes. Hence, the study was planned to evaluate the fate of this disorder in our region with respect to various given treatment options.
Aims
The aim of the study was to assess disease outcome in the region with best available treatment option given and establish local data on it.
Methods
It was an analytical cross sectional study conducted at National Institute of Blood Diseases and Bone Marrow Transplantation, Karachi Pakistan from June 2010 to January 2018. Baseline investigations done including complete blood counts, bone marrow biopsy and cytogenetics. Clinical parameters of all patients were recorded. Patients were classified according to world health organization (WHO) 2008 classification and IPSS was calculated. Approval from the institutional ethics committee was obtained prior to the study. Differences in clinical measurements were evaluated using SPSS version 23. Moreover overall survival (OS) was also observed.
Results
A total of 186 patients were included in the study. The median age was 55 (range 3-90) years. There were 139 males and 47 females. In our patients the most common presenting complaint was loss of appetite 110(59%) followed by weakness and fever in 76(41%). The mean hemoglobin (Hb%) was 7.94±2.17g/dl, total leucocyte count (WBC) 7.51±13.3 × 109/l, platelet count 81±93.7 × 109/l. Cytogenetic data was available for 104 patients which revealed normal karyotype in 58 (56%) and abnormal karyotype in 46 (44%). The mean IPSS of the patients was 01. Out of total, supportive treatment was offered to (n=70,38%), hematinics (vitamin B12 and folic acid) (n=45,24%), recombinant erythropoietin (n=25,14%), lenalidomide (n=19,10%), thalidomide (n=11,06%), hypomethylating agents in (n=06,03%), steroids (n=06,03%) and growth factors (GCSF) in (n=04,02%). Death of 124 patients occurred during the study period. Cause of death was ascertained in 84 patients. Septicemia was the most common cause of death and found in (n=27, 32%) followed by shortness of breath (n=26, 31%), severe anemia (n=21, 25%) and intracranial bleeding in (n=10, 12%). The overall survival was 33% analyzed by using Kaplan meier curve. Risk of progression to AML was found in 06 patients with median time of 15 months.
Conclusion
Although MDS is considered as an uncommon disease yet the numbers of patients are increasing in Pakistan. There is a need to develop disease registry in our region to understand and approximate better measures which will help to treat patients with definitive treatment. Moreover, further studies with large sample size are needed to convince state’s health department to finance treatment and research in the context so that better treatment options are made available.
Session topic: 10. Myelodysplastic syndromes – Clinical
Keyword(s): MDS, Outcome, Treatment
Abstract: PB2108
Type: Publication Only
Background
Myelodysplastic syndromes (MDS) represent a diverse group of hematopoietic stem cell disorders characterizing ineffective hematopoiesis, morphological dysplasia and leukemic transformation. Although the international prognostic scoring system (IPSS) represents gold standard for risk assessment, it is still difficult to predict survival and median time to leukemic transformation in the majority. MDS being considered a relatively uncommon disorder in our region having no national registry for evaluation of disease burden is presumed to be an incurable disease with majority of patients conventionally being treated by only blood product support. Most of the patients are unable to avail standard treatment and those who are treated with recommended options are presumed to have hopeless outcomes. Hence, the study was planned to evaluate the fate of this disorder in our region with respect to various given treatment options.
Aims
The aim of the study was to assess disease outcome in the region with best available treatment option given and establish local data on it.
Methods
It was an analytical cross sectional study conducted at National Institute of Blood Diseases and Bone Marrow Transplantation, Karachi Pakistan from June 2010 to January 2018. Baseline investigations done including complete blood counts, bone marrow biopsy and cytogenetics. Clinical parameters of all patients were recorded. Patients were classified according to world health organization (WHO) 2008 classification and IPSS was calculated. Approval from the institutional ethics committee was obtained prior to the study. Differences in clinical measurements were evaluated using SPSS version 23. Moreover overall survival (OS) was also observed.
Results
A total of 186 patients were included in the study. The median age was 55 (range 3-90) years. There were 139 males and 47 females. In our patients the most common presenting complaint was loss of appetite 110(59%) followed by weakness and fever in 76(41%). The mean hemoglobin (Hb%) was 7.94±2.17g/dl, total leucocyte count (WBC) 7.51±13.3 × 109/l, platelet count 81±93.7 × 109/l. Cytogenetic data was available for 104 patients which revealed normal karyotype in 58 (56%) and abnormal karyotype in 46 (44%). The mean IPSS of the patients was 01. Out of total, supportive treatment was offered to (n=70,38%), hematinics (vitamin B12 and folic acid) (n=45,24%), recombinant erythropoietin (n=25,14%), lenalidomide (n=19,10%), thalidomide (n=11,06%), hypomethylating agents in (n=06,03%), steroids (n=06,03%) and growth factors (GCSF) in (n=04,02%). Death of 124 patients occurred during the study period. Cause of death was ascertained in 84 patients. Septicemia was the most common cause of death and found in (n=27, 32%) followed by shortness of breath (n=26, 31%), severe anemia (n=21, 25%) and intracranial bleeding in (n=10, 12%). The overall survival was 33% analyzed by using Kaplan meier curve. Risk of progression to AML was found in 06 patients with median time of 15 months.
Conclusion
Although MDS is considered as an uncommon disease yet the numbers of patients are increasing in Pakistan. There is a need to develop disease registry in our region to understand and approximate better measures which will help to treat patients with definitive treatment. Moreover, further studies with large sample size are needed to convince state’s health department to finance treatment and research in the context so that better treatment options are made available.
Session topic: 10. Myelodysplastic syndromes – Clinical
Keyword(s): MDS, Outcome, Treatment