Contributions
Abstract: PB1833
Type: Publication Only
Background
Aplastic anemia are acquired or congenital anemias associated with hypocellular bone marrow. The exact etiology is unknown but several factors are considered to be causative for suppression of hematopoitic cell production resulting in aplastic anemia.
Aims
This entity considered to be uncommon is frequently prevalent with rising trend seen especially in Pakistan. This is quite contrast to what is observed in west. Moreover, there is scarce local data. Hence the study was done to assess baseline clinical and cytogenetics features of patients presenting with aplastic anemia.
Methods
This study was approved by the Institutional Ethics Committee of National Institute of Blood Diseases and Bone Marrow Transplantation. In this cross sectional study,122 patients with aplastic anemia were enrolled during the period of June 2016 to January 2018. Informed consent was obtained prior to the study. Data collected included demographic information, laboratory information, includinggender, symptoms, treatments, blood counts and chemistry parameters including urea, creatinine and liver function tests.Viral profile included Anti-HCV,Anti-HBsAg, HIV I/II and Cytomegalovirus (CMV) performed. Cytogenetic analysis was performed on bone marrow samples and karyotypes were described according to the International System for Human Cytogenetic Nomenclature (ISCN) 2013, karyogram were made using Meta system. Variables were evaluated using SPSS version 23.
Results
A total of 122 patients were included in the study. The median age of the patients was 14±12.59. A slight predominance of males were observed which were (n=76, 62%). Fever and weakness were the most complain found in (n=56, 46%) followed by colitis (n=24, 20%), gum bleeding (n=20, 16%) bruises (n=12, 10%) and shortness of breath in (n=10, 8%) patients. All patients were grouped according to camitta classification. Non severe aplastic anemia were observed in (n=100, 82%) followed by very severe aplastic anemia (n=12, 10%) and severe aplastic anemia (n=10, 8%). The mean hemoglobin was 7.61±2.11g/dl, red blood cells 3.17±3.4 × 1012/l, MCV 89.2±11.56fl, total leucocytes counts (tlc) 3.09±2.03 × 109/l, absolute neutrophils count 0.7±0.99×109/l and platelets counts were 27±52.29 ×109/l. The mean total bilirubin was 0.76±0.38mg/dl, direct bilirubin 0.37±0.27mg/dl, alanine aminotransferase (SGPT) 61±89.5u/l and alkaline phosphatase was 201±116.5u/l. Out of 122 patients, chromosomal breakage was observed in (n=10, 8%) patients. Anti HbsAg was positive in (n=04, 3%) and anti HCV in (n=02, 1.6%).CMV was positive in (n=02, 1.6%) patients.
Conclusion
In our study we have observed lower median age andmale predominance.Non severe aplastic anemia was most common.Raised SGPT was seen in many indicating liver damage. To overcome adverse prognostic implications,early identification of such patients with close clinical follow up and upfront stem cell transplant must be considered. This study was done retrospectively yet represents a large cohort of aplastic anemia in the country. In future, prospective studies are needed to be done to elaborate disease biology and clinical outcome of the baseline adverse disease characteristics observed in our study.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): Aplastic anemia, Cytogenetics
Abstract: PB1833
Type: Publication Only
Background
Aplastic anemia are acquired or congenital anemias associated with hypocellular bone marrow. The exact etiology is unknown but several factors are considered to be causative for suppression of hematopoitic cell production resulting in aplastic anemia.
Aims
This entity considered to be uncommon is frequently prevalent with rising trend seen especially in Pakistan. This is quite contrast to what is observed in west. Moreover, there is scarce local data. Hence the study was done to assess baseline clinical and cytogenetics features of patients presenting with aplastic anemia.
Methods
This study was approved by the Institutional Ethics Committee of National Institute of Blood Diseases and Bone Marrow Transplantation. In this cross sectional study,122 patients with aplastic anemia were enrolled during the period of June 2016 to January 2018. Informed consent was obtained prior to the study. Data collected included demographic information, laboratory information, includinggender, symptoms, treatments, blood counts and chemistry parameters including urea, creatinine and liver function tests.Viral profile included Anti-HCV,Anti-HBsAg, HIV I/II and Cytomegalovirus (CMV) performed. Cytogenetic analysis was performed on bone marrow samples and karyotypes were described according to the International System for Human Cytogenetic Nomenclature (ISCN) 2013, karyogram were made using Meta system. Variables were evaluated using SPSS version 23.
Results
A total of 122 patients were included in the study. The median age of the patients was 14±12.59. A slight predominance of males were observed which were (n=76, 62%). Fever and weakness were the most complain found in (n=56, 46%) followed by colitis (n=24, 20%), gum bleeding (n=20, 16%) bruises (n=12, 10%) and shortness of breath in (n=10, 8%) patients. All patients were grouped according to camitta classification. Non severe aplastic anemia were observed in (n=100, 82%) followed by very severe aplastic anemia (n=12, 10%) and severe aplastic anemia (n=10, 8%). The mean hemoglobin was 7.61±2.11g/dl, red blood cells 3.17±3.4 × 1012/l, MCV 89.2±11.56fl, total leucocytes counts (tlc) 3.09±2.03 × 109/l, absolute neutrophils count 0.7±0.99×109/l and platelets counts were 27±52.29 ×109/l. The mean total bilirubin was 0.76±0.38mg/dl, direct bilirubin 0.37±0.27mg/dl, alanine aminotransferase (SGPT) 61±89.5u/l and alkaline phosphatase was 201±116.5u/l. Out of 122 patients, chromosomal breakage was observed in (n=10, 8%) patients. Anti HbsAg was positive in (n=04, 3%) and anti HCV in (n=02, 1.6%).CMV was positive in (n=02, 1.6%) patients.
Conclusion
In our study we have observed lower median age andmale predominance.Non severe aplastic anemia was most common.Raised SGPT was seen in many indicating liver damage. To overcome adverse prognostic implications,early identification of such patients with close clinical follow up and upfront stem cell transplant must be considered. This study was done retrospectively yet represents a large cohort of aplastic anemia in the country. In future, prospective studies are needed to be done to elaborate disease biology and clinical outcome of the baseline adverse disease characteristics observed in our study.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): Aplastic anemia, Cytogenetics