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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. [ABSTRACT FROM AUTHOR]

BACKGROUND: This study aimed to determine the association of single-nucleotide polymorphisms (SNPs) of interleukin-6 (IL-6) gene with the survival and disease status of patients after allogeneic hematopoietic stem cell transplantation. MATERIALS AND METHODS: It was a prospective cohort study of total 102 participants, 51 patients along with their donors who had human leukocyte antigen-identical-matched allogeneic hematopoietic stem cell transplantation (aHSCT). Their pretransplant and posttransplant blood samples were collected for extraction of DNA for genotyping by sequence-specific primers polymerase chain reaction and gel electrophoresis followed by sequencing. The pre- and posttransplant levels of IL-6 were measured using enzyme-linked immunosorbent assay technique. RESULTS: The mean age of our patients was 19.83 ± 12.5 years having males 65 (63.7%) and females 37 (36.3%). The frequency of −174G/C SNP among acute graft-versus-host disease (aGVHD) group was GG = 53.8%, GC = 34.6%, and CC = 11.5%, and for −597G/A SNP, it was GG = 69.2%, GA = 15.4%, and AA = 15.4%. Our results showed that the presence of G allele in both homozygous and heterozygous forms was associated with increased aGVHD incidence, while the homozygous CC and AA mutant genotypes correlated with the lowest number of cases of aGVHD. The polymorphism −597G/A was significantly associated with the incidence of aGVHD with P = 0.04. The GG genotype in −174G/C and −597G/A was found to be significantly associated with aGVHD with P < 0.0001. High serum levels of IL-6 pre- and posttransplant were found to be significantly associated with the incidence of aGVHD with P < 0.0001. CONCLUSION: We concluded that the two SNPs − 174G/C and − 597G/A in the promoter region of IL-6 gene present either in donors or recipients predisposed to increase the risk of development of aGVHD following aHSCT. Their evaluation in our transplant setting may help in the risk stratification of transplant recipients early in time and predict the onset of aGVHD and mortality. [ABSTRACT FROM AUTHOR]

Corona virus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome corona virus 2 (SARSCoV2) and has currently affected 11 million people worldwide with 0.53 million deaths. While there is ongoing trials and research for newer therapeutic options including antivirals (remdesivir), anti-interleukin 6 (tocilizumab), vaccines and antimalarials; there is renewed interest in the use of old therapeutic tools as well. Convalescent plasma has been used historically for measles, poliomyelitis, Spanish flu pandemic, severe acute respiratory syndrome (SARS1) and Ebola epidemics with conflicting results. With widespread use of convalescent plasma throughout the world as investigational drug for COVID-19, it is important to review past experience with its use in various outbreaks, potential role in COVID-19, and clinical application during current pandemic and challenges faced by resource constrained countries. [ABSTRACT FROM AUTHOR]

Objective: To determine the clinical/haematological manifestations and frequency of different subtypes of Acute Myeloid Leukaemia (AML) according to the French-American-British (FAB) classification. Study Design: Descriptive study. Place and Duration of Study: The study was carried out at haematology department of Armed Forces Institute of Pathology (AFIP), Rawalpindi from January 2011 to September 2012. Material and Methods: Retrospective review of documents of patient diagnosed to have acute myeloid leukaemia on bone marrow aspiration was done. Patient's age, gender, major signs and symptoms at time of presentation and haematological parameters of peripheral blood and bone marrow were noted. The subtype of AML according to FAB classification was also documented. Data was entered and analyzed in SPSS 16.0. Results: During the selected study duration acute myeloid leukaemia was diagnosed in 173 patients on bone marrow e×amination. Out of these 123 (71.1%) were males and 50 (28.9%) were females. Thirty (17.3%) of the patients fell in paediatric age group (< 15 years) while the remaining 143 (82.7%) were in adult age category (> 15 years). The mean age of presentation was 9 years among paediatric patients and 44.5 years among adults. The overall mean age of both these two groups was 38.4 years (3-84 years). Fever (71.6%), generalized weakness (34.1%) and pallor (23.7%) were the three main complaints of the patients, followed by splenomegaly and lymphadenopathy. The mean total leukocyte count, haemoglobin and platelet count were 57.4 × 109/L, 7.9 g/dL and 54 × 109/L respectively. AML-M2 was found to be the most frequent FAB AML subtype among 72 (41.6%) paediatric and adult patients. Conclusion: The main signs and symptoms of the patients of AML presenting to our centre were fever, generalized weakness and pallor. AML-M2 was found to be the most common FAB subtype among AML in children and adults. [ABSTRACT FROM AUTHOR]

Hairy cell leukaemia is a clonal B cell lymphoproliferative disorder. Patient usually presents to clinician with cytopenias and splenomegaly. Common symptoms include abdominal discomfort, fatigue, generalized weakness, recurrent infections, bruising and bleeding complaints. We present a case of Hairy cell leukaemia with only 25% neoplastic cells in which BRAFV600E mutation was detected. Cytochemical stain, trephine biopsy and immunophenotyping were also consistent with Hairy cell leukaemia. [ABSTRACT FROM AUTHOR]