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Background: Aplastic anaemia is a rare bone marrow failure syndrome and is defined by pancytopenia associated with a hypo-cellular bone marrow with no increase in reticulin and the absence of any abnormal infiltrate. The objective of the study was to determine the frequency of Aldehyde Dehydrogenase type 2 (ALDH2) deficiency in patients with Aplastic Anaemia and investigate its correlation with patient and disease characteristics. It was a descriptive cross-sectional study conducted at Armed Forces Bone Marrow Transplant Centre Rawalpindi from 01-08-2022-01-02-2023, over 6 months., Methods: A total of 56 patients who were diagnosed with aplastic anaemia during this period, fulfilling inclusion criteria were enrolled. Patients were genotyped as GG (homozygous) and GA (heterozygous). GG had normal ALDH2, while GA were patients with ALDH2 deficiency. Data was collected on the patient's demographics, type and severity of anaemia, type of hematopoietic stem cell transplant (HSCT) and frequency of ALDH2 deficiency. Results were analyzed for ALDH2 deficiency and its correlation with patient and disease characteristics was investigated., Results: A total of 56 patients were included in the study. The median age of the patients was 28 years (20-39). According to the type of aplastic anaemia, 2 (3.6%) had Fanconi anaemia and 54 (96.4%) had acquired aplastic anaemia. In our study, 18 (32.1%) patients had undergone HSCT while the remaining 38 (67.9%) could not undergo HSCT. The frequency of the presence of ALDH2 deficiency was 2 (3.6%). There was no statistically significant correlation between the frequency of ALDH2 deficiency with variables like gender, age distribution, type of aplastic anaemia, the severity of aplastic anaemia and hematopoietic stem cell transplant., Conclusions: We concluded from our study the frequency of ALDH2 was rare in patients with aplastic anaemia. There was no statistically significant correlation between the frequency of ALDH2 deficiency with variables like gender, age distribution, type of aplastic anaemia, the severity of aplastic anaemia and hematopoietic stem cell transplant.

Background: Aplastic Anaemia (AA) is characterized by pancytopenia and hypocellular marrow. Immunosuppressive therapy (IST) SHOWS impressive haematological response; however, risk of relapse and clonal evolution persists. The objective of the study is to assess response to IST in patients with aplastic anaemia., Methods: A retrospective single centre study at AFBMTC / NIBMT for patients of acquired AA was conducted from January 2005 to December 2019.Inclusion criteria included diagnosed cases of acquired AA receiving IST for at least 12 weeks and age >2 years. IST included cyclosporine (CsA) alone, CsA + androgens, CsA + rabbit anti thymocyte globulin (rATG), CsA + horse anti thymocyte globulin (hATG). Primary outcome measure was response to IST; secondary outcome measure was overall survival (OS)., Results: A total of 513 patients received IST. Median age was 23 years (range 2-97 years). In study cohort, 155 (30.2%) patients responded to the IST, 63 (12.3%) achieved complete response (CR) while 92 (17.9%) achieved partial response (PR). The ORR of CsA in NSAA, SAA and VSAA was 52.6%, 28.10% and 10% respectively; whereas ORR of CsA + rATG in NSAA, SAA and VSAA was 50%, 35.1% and 22.5% respectively. OS was 38% at a median follow up of 36 months. There was a significant difference in the survival distributions of different treatment modalities (p=0.016). Median survival time 60 months (CsA), 9 months (CsA+ androgens) and 39 months (CsA+ rATG/hATG.) ., Conclusion: In resource constrained settings, single agent CsA remains a reasonable alternative with modest activity and acceptable side effect profile.

Background: Coronavirus disease 2019 (COVID-19) is a multisystem disorder and haematological abnormalities are frequently documented in affected patients., Methods: This retrospective study included 549 patients hospitalized with COVID-19 from 1st June to 15th July 2020 at Pak Emirates hospital, Rawalpindi Pakistan. p<0.05 was considered statistically significant., Results: Median age was 60 years (range 12-94 years), males 442 (80.5%) and females 107 (19.5%). There was no patient with mild illness, 181 (32.9%) had moderate, 158 (28.7%) severe and 210 (38.2%) patients had critical disease. Patients with severe and critical disease had lower absolute lymphocyte count (ALC) and platelets (p<0.001 for both) while higher white blood cell count (WBC), neutrophil lymphocyte ratio (NLR), C-reactive protein (CRP), interleukin-6 (IL-6) and lactate dehydrogenase levels (LDH) levels (all p<0.001). Overall survival of study cohort was 83.2% (n=457). Median haemoglobin and platelet count were significantly lower (p<0.001) while WBC, ANC, NLR, prothrombin time (PT), activated partial thromboplastin time (APTT), ferritin, IL-6, LDH were significantly higher (p<0.001) for patients who died. On multivariate logistic regression analysis WBC count>10x109/l (odds ratio [OR] 2.19 [95% CI 1.3-4.2] p=0.01), NLR>9 (OR 3.4 [95% CI 0.87-6.8], p<0.001), platelets<150x109/l (OR 3.9 [95% CI 1.4-9.8] p<0.001), CRP >100; (OR 4.1[95% CI 0.78-10.9] p<0.001) and ferritin >1000 (OR 5.3 [95% CI 1.9- 13.5], p<0.001) were associated with increased risk of death in patients with COVID-19., Conclusion: Monitoring of haematological, coagulation and inflammatory parameters provide reliable, convenient, rapid and cost-effective method for predicting disease severity, complications and prognosis of COVID-19 patients.

Background: Patients with haematological malignancies and stem cell transplant recipients are at high risk of opportunistic infections. Little international and no national data is available comparing noble metal coated versus uncoated central venous catheters (CVC) in this special population of severely immunocompromised patients. Objective of the study is to compare infectious and non-infectious complications of noble metal coated versus uncoated central venous catheters in patients undergoing stem cell transplantation and receiving chemotherapy for acute myeloid leukaemia., Methods: This is a prospective, cross-sectional, randomized study (January to December 2016), enrolling 45 consecutive patients undergoing stem cell transplantation or chemotherapy for acute myeloid leukaemia. Patients were randomized in 2 groups. Twenty 23 patients received standard CVC and 22 patients received CVC catheters coated with three noble metals (Gold, Silver, Palladium). Patients were observed for catheter related infectious and noninfectious complications. Data was analysed using SPSS., Results: Mean age was 24.3 (±4.91) in uncoated and 25.09 (±5.22) in coated CVL group. CRBSI infection was detected in 2 (8.6%) and 3 (13.6%) patients in uncoated and coated group respectively with p-value of .279. There was no statistically significant difference in febrile episodes between coated (95.4%) and uncoated (91.3%) group. While we considered non-infectious complications, 2 patients in coated (8.6%) and 1 in uncoated CVCs group (4.3%) had CVC thrombosis which was not significant statistically., Conclusion: There was no efficacy of BG-thin noble metal coated CVCs in reducing infectious and non-infectious complications (thrombosis) in our study.