Hematology
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Review Comparative Study
The rebirth of the cool: a narrative review of the clinical outcomes of cold stored low titer group O whole blood recipients compared to conventional component recipients in trauma.
There has been renewed interest in the use of low titer group O whole blood (LTOWB) for the resuscitation of civilian casualties. LTOWB offers several advantages over conventional components such as providing balanced resuscitation in one bag that contains less additive/preservative solution than an equivalent volume of conventional components, is easier and faster to transfuse than multiple components, avoids blood product ratio confusion, contains cold stored platelets, and reduces donor exposures. The resurgence in its use in the resuscitation of civilian trauma patients has led to the publication of an increasing number of studies on its use, primarily amongst adult recipients but also in pediatric patients. ⋯ The published studies to date on mortality have shown conflicting results with some demonstrating a reduction following LTOWB transfusion while most others have not shown a reduction; there have not been any studies to date that have found significantly increased overall mortality amongst LTOWB recipients. Similarly, when other clinical outcomes, such as venous thromboembolism, sepsis, hospital or intensive care unit lengths of stay are evaluated, LTOWB recipients have not demonstrated worse outcomes compared to conventional component recipients. While definitive proof of the trends in these morbidity and mortality outcomes awaits confirmation in randomized controlled trials, the evidence to date indicates the safety of transfusing LTOWB to injured civilians.
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Introduction: RUNX1 mutations in acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS) are associated with distinct clinicopathologic features. However, the clinical and laboratory characteristics of the myeloid malignancies may be influenced by the presence of more concomitant mutations. The aim of this study is to provide a further understanding of mutational landscape in the context of RUNX1 mutation in AML/MDS. ⋯ RUNX1-mutated AML patients with 3, or ≥4 co-mutations showed much lower CR rate than that with 2 additional mutations (p = 0.0247, 0.00919). Conclusion: RUNX1-mutated AML and MDS are associated with a different complex co-mutation cluster. Some co-mutations have certain influence on the clinical feature and CR rate in the context of RUNX1 mutation.
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Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy that can have high mortality rates without prompt treatment. Standard treatment is urgent plasma exchange (PLEX), which leads to disease remission in the vast majority of patients. Deficiency of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) alone is not sufficient to cause the clinical manifestations characteristic of TTP. We present a case of acquired TTP, where spontaneous recovery was observed prior to initiation of any TTP-specific therapy. ⋯ TTP is a haematologic emergency that requires urgent therapy to reduce morbidity and mortality. However, it is well documented that individuals with hereditary TTP and a proportion with acquired TTP in clinical remission can have low or nearly absent ADAMTS13 activity levels without evidence of microangiopathic haemolytic anaemia (MAHA) or thrombotic manifestations. Our patient represents a unique case of confirmed ADAMTS13 deficiency due to a documented inhibitor, leading to mild haemolytic anaemia and thrombocytopenia both of which recovered spontaneously. We propose that this scenario could represent a 'subclinical' TTP state that precedes the development of clinically significant disease.
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Background: Venous thromboembolism (VTE) is a common complication in cancer patients. We aim to evaluate the effect and safety of direct oral anticoagulants (DOACs) as primary prophylaxis in ambulatory cancer patients. Methods: We conducted a literature search in PubMed, EMBASE and ClinicalTrials for studies that evaluated DOACs for thromboprophylaxis in cancer patients. ⋯ DOACs may be recommended in selected patients at high risk of VTE. More high-quality studies are needed to further validate our results. Abbreviations: CAT: cancer-associated thrombosis; CI: confidence interval; DOAC: direct oral anticoagulant; DVT: deep vein thrombosis; LMWH: low molecular weight heparin; NNH: number needed to harm; NNT: number needed to treat; PE: pulmonary embolism; RCT: randomized controlled trials; RR: risk ratio; RD: rate difference; VTE: venous thromboembolism.
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Clinical Trial
Malignancy-associated hemophagocytic lymphohistiocytosis in children: a 10-year experience of a single pediatric hematology center.
Objective: Malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH) in children is a relatively rare but life-threatening secondary hemophagocytic lymphohistiocytosis (sHLH). Until now, only a limited number of cases regarding children with M-HLH has been reported. Methods: We conducted a retrospective study of 27 children with M-HLH, who admitted to our center between July 2007 and October 2019. ⋯ Patients with prolonged APTT had a significantly poorer OS than other patients (p = 0.012). Conclusions: The M-HLH children with EBV infection are more likely to have prolonged APTT and more hemophagocytosis in BM. The M-HLH children had a poor prognosis, especially those with prolonged APTT.