Chronic myeloid leukemia during pregnancy complicated by dengue infection and pulmonary embolism.

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by the rearrangement of long arm of chromosomes 9 and 22, resulting in the Philadelphia chromosome, creating the fusion oncogene BCR-ABL1. Median age of onset of CML is 67 years. However, approximately a third of patients are of reproductive age at diagnosis.

Haemotoxicity of snakes: a review of pathogenesis, clinical manifestations, novel diagnostics and challenges in management.

Haemotoxicity is the most common complication of systemic envenoming following snakebite, leading to diverse clinical syndromes ranging from haemorrhagic to prothrombotic manifestations. Key haematological abnormalities include platelet dysfunction, venom-induced consumption coagulopathy, anticoagulant coagulopathy and organ-threatening thrombotic microangiopathy. Diagnostic methods include the bedside whole blood clotting test, laboratory coagulation screening and other advanced methods such as thromboelastogram and clot strength analysis.

Snakebite-associated acute kidney injury in South Asia: narrative review on epidemiology, pathogenesis and management.

Snakebite-associated acute kidney injury (AKI) poses a significant health burden in the South Asia region, resulting in considerable morbidity and mortality. Multiple factors contribute to the pathogenesis of AKI following snakebites, including hypotension, intravascular haemolysis, disseminated intravascular coagulation, rhabdomyolysis, thrombotic microangiopathy (TMA) and direct nephrotoxicity. Clinical features manifest as anuria, oliguria, haematuria, abdominal pain and hypertension.

"Bone marrow-liver-spleen-type diffuse large B-cell lymphoma" presenting with cold autoimmune hemolytic anemia: a case report.

Introduction: Primary bone marrow diffuse large B-cell lymphoma is a rare clinical entity, and the "bone marrow-liver-spleen" type of diffuse large B-cell lymphoma is rarer, with only a few published cases in literature. Though bone marrow-liver-spleen-type diffuse large B-cell lymphoma has unique presentations such as fever, cytopenias, and hemophagocytic lymphohistiocytosis, no cases with cold autoimmune hemolytic anemia have been reported.

Case Presentation: A 39-year-old Sri Lankan woman, previously healthy, presented with shortness of breath, productive cough, and fever for 4 days.

Donor-type bone marrow aplasia following hematopoietic stem cell transplantation in a child with a novel variant.

Pathogenic variants in the genes ( and cause bone marrow failure with characteristic syndromic features. We report a case of a previously healthy, 3-year-old boy with no dysmorphology, who presented with severe aplastic anemia and a novel variant in the gene. His father, elder brother and sister who harbored the same variant were completely healthy.

Hirudin versus citrate as an anticoagulant for ROTEM whole blood impedance aggregometry in thrombocytopenic patients.

Citrate is widely used as an anticoagulant for platelet function tests (PFTs). Due to an intrinsic inhibitory effect of citrate on platelet function, hirudin is used as an alternative. However, studies comparing the effect of these anticoagulants on rotational thromboelastometry (ROTEM) platelet whole blood impedance aggregometry in thrombocytopenic patients are scant.

The use of rotational thromboelastometry parameters in understanding the coagulopathy following hump-nosed viper (Hypnale spp) bites: a preliminary study.

Background: Hump-nosed vipers (HNV; Hypnale spp) are one of the medically important venomous snakes in Sri Lanka and South-Western regions of India. The haemostatic dysfunction due to HNV bites is poorly characterized by standard diagnostic tests performed to identify coagulopathy. We aimed to determine the usefulness of rotational thromboelastometry (ROTEM) parameters compared to 20-minute whole blood clotting test (WBCT20) and prothrombin time (PT) in understanding the coagulopathy of HNV bites.

Assessment of iron overload in a cohort of Sri Lankan patients with transfusion dependent beta thalassaemia and its correlation with pathogenic variants in HBB, HFE, SLC40A1, and TFR2 genes.

Background: Iron overload (IO) is a complication in transfusion dependent beta thalassaemia (TDT). Pathogenic variants in genes involving iron metabolism may confer increased risk of IO. The objective of this study was to determine the magnitude of the cardiac and hepatic IO and determine whether pathogenic variants in HFE, SLC40A1 and TFR2 genes increase the risk of IO in a cohort of TDT patients in Sri Lanka.

Rotational thromboelastometry in critical phase of dengue infection: Association with bleeding.

Background: The critical phase of dengue carries a high risk of bleeding. Associations of coagulation test parameters and the risk of bleeding in the critical phase is unclear. This study examines the association of rotational thromboelastometry (ROTEM and ROTEM ) with bleeding risk of patients with dengue in the critical phase.

A report of haploidentical allogeneic haematopoietic stem cell transplantation for inherited bone marrow failure in Sri Lanka.

Bone marrow failure (BMF) in children can be idiopathic (70-80%) or inherited. Haematopoietic stem cell transplantation (HSCT) is the only cure for both causes. Allogeneic HSCT requires a suitable donor.

A Sri Lankan girl with a new genetic variant in the PKLR gene causing pyruvate kinase deficiency: a case report.

Background: Erythrocyte pyruvate kinase is expressed under the control of the PKLR gene located on chromosome 1q21. Pyruvate kinase catalyzes the final steps of the glycolytic pathway and creates 50% of the red cell total adenosine triphosphate. Pyruvate kinase deficiency is the commonest glycolytic defect causing congenital non-spherocytic hemolytic anemia inherited in an autosomal recessive trait in which homozygotes and compound heterozygotes are common.

Comparison of rotational thromboelastometry parameters with 20-minute whole blood clotting test as a predictor of envenoming in Russell's viper bite patients.

Background: Coagulopathy is an important and common systemic clinical syndrome caused by snake envenoming. The major clinical effect of Russell's viper (RV) envenoming is haematotoxicity. The 20-min whole blood clotting test (WBCT20) is the standard test for identification of envenoming in resource-limited settings.

Hemostatic derangement in leptospirosis: A prospective cross-sectional study.

Context: Coagulation abnormalities have been observed among leptospirosis patients. However, coagulopathy in severe leptospirosis has not been further characterized.

Aims: The aim of this study was to evaluate conventional coagulation and rotational thromboelastometry (ROTEM) parameters in leptospirosis patients.

Pharmacokinetic, pharmacodynamic, and pharmacogenetic assays to monitor clopidogrel therapy.

Clopidogrel is the most common and widely used antiplatelet agent for patients with coronary artery disease following confirmation by electrocardiographic studies. The nonresponsiveness of patients to clopidogrel and the possibility of testing for clopidogrel resistance by platelet function assays (PFA) are contentious issues. Light transmission aggregometry (LTA) is considered as the gold standard test among all PFA.

Severe acute kidney injury following Sri Lankan spp. envenoming is associated with thrombotic microangiopathy.

Context: Acute kidney injury (AKI) is the most serious clinical manifestation of the Sri Lankan hump-nosed pit viper ( spp.) bites. Thrombotic microangiopathy (TMA) is increasingly recognized in association with AKI in cases of spp envenomation.

Immunophenotypic characterization of acute lymphoblastic leukemia in a flowcytometry reference centre in Sri Lanka.

Background: Acute lymphoblastic leukemia (ALL), is a biologically heterogeneous disease where diagnosis, treatment and prognosis is heavily dependent on the correct characterization of the immunophenotype of each case.

Objectives: To describe the immunophenotypic and laboratory features of a cohort of Sri Lankan children and adults with ALL and to compare them with those reported in other series.

Methods: Records of 229 patients who were suspected of having acute leukaemia and referred for flow cytometry to the Asiri Hospital Sri Lanka, between August 2009 and October 2013 were reviewed.

Transient elevation of serum ferritin in a Sri Lankan with homozygosity for H63D mutation in the HFE gene: a case report.

Introduction: Hereditary hemochromatosis is an inherited disorder of iron metabolism, characterized by excessive iron deposition in major organs of the body, leading to multi-organ dysfunction. It is a genetically heterogeneous disease caused by mutations in one or more different genes, the most common being mutations in the HFE gene. HFE hereditary hemochromatosis is mostly found in Europeans and is almost always a result of two mutations: C282Y and H63D.

Thrombotic microangiopathy and acute kidney injury following Sri Lankan and species envenoming.

Thrombotic microangiopathy (TMA) resulting in acute kidney injury (AKI) is an important complication of venomous snakebites. We aimed to describe TMA secondary to Russell's viper () and hump-nosed viper ( spp.) bites and assess the effect of different treatments.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin (POEMS) changes syndrome presenting with a pseudosensory level: a case report.

Introduction: Polyneuropathy is a key feature of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome, which is a paraneoplastic manifestation of an underlying lymphoproliferative neoplasm. We report the first case of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome presenting with a pseudosensory level.

Case Presentation: A 59-year-old Tamil woman with long-standing diabetes mellitus and hypertension developed painless, progressive inguinal lymphadenopathy.

Advances in hematopoietic stem cell transplantation in the Asia-Pacific region: the second report from APBMT 2005-2015.

Between 2005 and 2015, 138,165 hematopoietic stem cell transplantation (HSCT) were reported in 18 countries/regions in the Asia-Pacific region. In this report, we describe current trends in HSCT throughout the Asia-Pacific region and differences among nations in this region and various global registries. Since 2008, more than 10,000 HSCTs have been recorded each year by the Asia-Pacific Blood and Marrow Transplantation Group Data Center.

The role of haematological indices in predicting early iron deficiency among pregnant women in an urban area of Sri Lanka.

Background: Early detection and treatment of iron deficiency during pregnancy is crucial for optimum pregnancy outcomes. Anaemia is a late indictor of iron deficiency measured as Hb < 11 g/dL, and is widely used as a proxy for iron deficiency. We aimed to evaluate the role of red cell indices as a screening tool for early detection of iron deficiency among pregnant women in an urban area of Sri Lanka.

Can findings on peripheral blood smear differentiate leptospirosis from other infections? A preliminary comparative study.

Background: Blood smear changes have never been investigated as a potential tool to aid in the diagnosis of leptospirosis.

Methods: Blood smears prepared from patients with leptospirosis, dengue and sepsis within the first 5 days of illness were reported by haematologists blinded to the diagnosis.

Results: A total of 20, 28 and 22 patients with leptospirosis, dengue and sepsis, respectively, were analysed.

ATG vs thiotepa with busulfan and cyclophosphamide in matched-related bone marrow transplantation for thalassemia.

Matched-related bone marrow transplantation (BMT) may cure >80% of low-risk children with severe thalassemia (ST). Very long-term follow-up studies have shown how the standard busulfan-cyclophosphamide (BuCy) regimen may be associated with normalization of health-related quality of life, no second malignancies in the absence of chronic graft-versus-host disease, and fertility preservation in many patients. However, because BuCy may be associated with high rejection rates, some centers incorporate thiotepa (Tt) in busulfan- or treosulfan-based regimens, a combination that may increase the risk of permanent infertility.