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eJHaem,2022年

Lia C. Scott, Juan Li, Lorraine A. Cafuir, Manila Gaddh, Christine L Kempton

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Introduction : Limited data exists on the safety and efficacy of direct-acting oral anticoagulants (DOAC) use in morbidly obese patients with venous thromboembolism (VTE). Given the benefits of DOAC use over vitamin K antagonists (VKAs), in terms of monitoring requirements, and dietary and drug interactions, it is important to evaluate whether this is consistent in the higher risk for VTE recurrence morbidly obese group body mass index (BMI ≥ 40 kg/m 2 ). Materials and methods : This retrospective, single-center cohort study included patients with a BMI of at least 40 kg/m 2 who were admitted to Emory University Hospital from 1 st January 2012 to 31 st May 2020 with acute VTE, and subsequently initiated on anticoagulation treatment with either DOAC or VKA (warfarin). Univariate and bivariate analyses were used to evaluate differences in demographics by treatment type and BMI. Multivariate Cox proportional hazard regression was used to assess the risk of VTE recurrence by type of treatment among morbidly obese patient subgroup. Results : There were 247 (11.8%) morbidly obese (≥ 40 kg/m 2 ) patients who were more likely than non-obese patients to be younger, female, and of non-white race. Thirty percent of the study population (n=74) had a BMI > 50 kg/m 2 . T ime-to-event analysis confirmed that the hazard of experiencing a recurrent thrombosis was not statistically significantly different among morbidly obese patients treated with a DOAC compared with VKA (hazard ratio [HR]: 0.28, confidence interval [CI] 0.07-1.11, p  = 0.07). Conclusions : This study aligns with previous literature and confirms that morbidly obese patients receiving DOAC or VKA have similar risks of recurrent VTE.

    eJHaem,2022年

    Siba El Hussein, Mariko Yabe, Wei Wang, Naveen Pemmaraju, Sanam Loghavi, Fatima Zahra Jelloul, Hong Fang, L. Jeffrey Medeiros, W. Richard Burack, Andrew G. Evans, Jane L. Liesveld, John M. Bennett

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    This report highlights the value of flow cytometry analysis, particularly in the setting of myeloproliferative neoplasms showing features of progression, as neoplastic plasmacytoid dendritic cell (PDC) proliferations may be present, representing either a clonal expansion of mature PDCs related to the underlying myeloproliferative neoplasm or transformation to blastic plasmacytoid dendritic cell neoplasm (BPDCN). BPDCN should always be considered in patients with myeloid neoplasms in progression and/or who develop new cutaneous findings, as it may prompt change of management.

      eJHaem,2022年

      Jorge J. Castillo, Shayna Sarosiek, Catherine A. Flynn, Carly Leventoff, Megan Little, Timothy White, Kirsten Meid, Steven P. Treon

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      The hematopoietic cell kinase (HCK) regulates BTK activation and represents a potential therapeutic target in Waldenstrom macroglobulinemia (WM). We investigated dasatinib, a potent HCK inhibitor, in patients with WM progressing on ibrutinib. Study treatment consisted of dasatinib administered at 100 mg by mouth once daily in four-week cycles for up to 24 cycles. This study was registered under ClinicalTrials.Gov ID NCT04115059. Three participants were enrolled and received at least one cycle of dasatinib. The best response was stable disease. Two patients received 5 months and one patient received 1 month of therapy. The dose of dasatinib was decreased in one participant due to volume overload. Based on the lack of responses observed, the study was terminated. Dasatinib might not be effective in patients with WM progressing on ibrutinib.

        eJHaem,2022年

        Conor McCafferty, Leo Lee, Tengyi Cai, Slavica Praporski, Julian Stolper, Vasiliki Karlaftis, Chantal Attard, David Myint, Leeanne M. Carey, David W. Howells, Geoffrey A. Donnan, Stephen Davis, Henry Ma, Sheila Crewther, Vinh A. Nguyen, Suelyn Van Den Helm, Natasha Letunica, Ella Swaney, David Elliott, Kanta Subbarao, Vera Ignjatovic, Paul Monagle

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        Coronavirus disease 2019 (COVID-19) patients have increased thrombosis risk. With increasing age, there is an increase in COVID-19 severity. Additionally, adults with a history of vasculopathy have the highest thrombotic risk in COVID-19. The mechanisms of these clinical differences in risk remain unclear. Human umbilical vein endothelial cells (HUVECs) were infected with SARS-CoV-2, influenza A/Singapore/6/86 (H1N1) or mock-infected prior to incubation with plasma from healthy children, healthy adults or vasculopathic adults. Fibrin on surface of cells was observed using scanning electron microscopy, and fibrin characteristics were quantified. This experiment was repeated in the presence of bivalirudin, defibrotide, low-molecular-weight-heparin (LMWH) and unfractionated heparin (UFH). Fibrin formed on SARS-CoV-2 infected HUVECs was densely packed and contained more fibrin compared to mock-infected cells. Fibrin generated from child plasma was the thicker than fibrin generated in vasculopathic adult plasma ( p  = 0.0165). Clot formation was inhibited by LMWH (0.5 U/ml) and UFH (0.1–0.7 U/ml). We show that in the context of the SARS-CoV-2 infection on an endothelial culture, plasma from vasculopathic adults produces fibrin clots with thinner fibrin, indicating that the plasma coagulation system may play a role in determining the thrombotic outcome of SARS-CoV-2 infection. Heparinoid anticoagulants were most effective at preventing clot formation.

          eJHaem,2022年

          Nobuhiko Imahashi, Mikiko Arakawa, Akari Iwakoshi, Mikiko Mori, Hirokazu Nagai, Hiroatsu Iida

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          A 50-year-old man developed acute myeloid leukemia while receivingantiretroviral therapy for human immunodeficiency virus (HIV) infection. Following three courses of chemotherapy, he received cord bloodtransplantation (CBT) in the first complete remission. The patientdeveloped mild skin acute graft-versus-host disease after CBT. In addition, he developed organizing pneumonia 1 month after CBT, whichresponded to steroid therapy, but repeatedly relapsed during steroidtapering. Thus, he had been receiving prolonged immunosuppressivetherapy. Thirteen months after CBT, bluish-red elevated lesions developed on the hard palate of the oral cavity (Figure 1A). Biopsy samplesobtained from the palate lesions confirmed the diagnosis of Kaposisarcoma (KS). His HIV viral load was undetectable, and there were nosigns of leukemia relapse. To improve the immunosuppression status,immunosuppressive therapy was attenuated. This led to the increasein CD4+ count from 0.26 × 109/L to 0.68 × 109/L and regression of theKS (Figure 1B, 18 months after CBT). However, the patient succumbedto heart and kidney failure 18 months after CBT. Despite its rarity, KSshould be considered as a differential diagnosis when hematopoieticstem cell transplantation recipients develop bluish-red or purplelesions.

            eJHaem,2022年

            Fatima Khadadah, Natasha Rupani, Jordan Scott, Martina Trinkaus, Jerome Teitel, Michelle Sholzberg

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            A previously healthy 33-year-old female presented with a large hematoma over her right knee after kneeling. She was found to have pancytopenia and massive splenomegaly. Von Willebrand Factor (VWF) antigen level was 0.38 units/ml, ristocetin cofactor activity 0.13 units/ml, and VWF multimeric distribution was normal. Bone marrow examination revealed an indolent B-cell lymphoma. Diagnosis was consistent with acquired von Willebrand syndrome as an autoimmune epiphenomenon of a lymphoma. Diagnostic and therapeutic splenectomy under hemostatic coverage was performed. VWF antigen levels and activities immediately normalized postoperatively and remained within the normal range several months later. Splenic pathology confirmed hairy cell leukemia with a BRAF mutation.