Hematology
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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.
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Comparative Study
Hematologic predictors of mortality in hospitalized patients with COVID-19: a comparative study.
Background: The first cases of proved COVID-19 in Iran were reported in February 2020 and has since rapidly spread worldwide. We aimed to clarify the clinical significance of hematologic parameters alteration in COVID-19. Methods: Different hematologic parameters were measured in 225 hospitalized COVID-19 patients in a tertiary care university hospital, during the peak of COVID-19 outbreak and their association with duration of hospitalization, ICU admission and especially mortality was analyzed. ⋯ Platelet-to-lymphocyte ratio (PLR) also correlated with mortality and was significantly higher in non-survivors (P = .034). Conclusions: Hematologic laboratory parameters have always been a crucial component of diagnostic and therapeutic strategies in infectious disease. Hematologic predictors of a fatal outcome in COVID19 hospitalized patients in our series include elevated NLR and PLR, lower than normal Hb and Plt, elevated d-dimer and prolonged prothrombin time (PT), together with elevated inflammatory indicators in the blood.