Severe acquired platelet dysfunction because of primary myelofibrosis with full functional and morphological recovery after allogeneic hematopoietic cell transplantation

In this report, we document restoration of megakaryocyte and platelet structure and function in PMF after allogeneic hematopoietic cell transplantation (HCT). A 59-year-old man presented with recurrent episodes of postoperative bleeding preceding a diagnosis of primary myelofibrosis (PMF). Platelet aggregation and secretion studies showed abnormal responses to all agonists tested (epinephrine, ADP, arachidonic acid, U46619, collagen, ristocetin) despite the presence of thrombocytosis. After an allogeneic HCT, platelet morphology and function studies were all normal. The pathophysiology of platelet dysfunction in myeloid neoplasia is not well understood but, as highlighted in our report, restoration of platelet function by HCT supports a clonal process involving an early hematopoietic progenitor cell.
Source: Blood Coagulation and Fibrinolysis - Category: Hematology Tags: CASE REPORTS Source Type: research

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This study was prospectively registered on Current Controlled Trials on 25 March 2015 (ISRCTN73545489) and is also registered on ClinicalTrials.gov (NCT03136445).
Source: Trials - Category: Research Source Type: clinical trials
Authors: Gilani JA, Ashfaq MA, Mansoor AE, Abdul Jabbar A, Siddiqui T, Khan M Abstract Primary Myelofibrosis is a BCR-ABL negative myeloproliferative neoplasm with a variety of hematologicalpresentations, including thrombosis, bleeding diathesis and marrow fibrosis. It is estimated to have an incidence of1.5 per 100,000 people each year. Although JAK2 or MPL mutations are seen in PMF, several other mutations haverecently been documented, including mutations in CALR, epigenetic regulators like TET, ASXL1, and 13q deletions.The identification of these mutations has improved the ability to develop novel treatment opti...
Source: Asian Pacific Journal of Cancer Prevention - Category: Cancer & Oncology Tags: Asian Pac J Cancer Prev Source Type: research
Conclusion The impact of sepsis on the gut is manifold, e.g., sepsis mediated alteration of the gut-blood barrier and increase in the intestinal permeability, which may correlate with the phenomena of bacterial translocation and lymphatic activation (“toxic-lymph”). Systemic consequences of sepsis are widespread and concern to the coagulative system, the microbiome as well as enzymes, such as pancreatic proteases, MMPs and IAPs. Nevertheless, the therapeutic approaches for modulating the mucosal immune system are still rarely effective in daily routine. Recent published studies showing that treatment with FMT,...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Maria R. Gancheva1*, Karlea L. Kremer1, Stan Gronthos2,3 and Simon A. Koblar1,3,4 1Stroke Research Programme Laboratory, Adelaide Medical School, Faculty of Health and Medical Sciences, University of Adelaide, Adelaide, SA, Australia 2Mesenchymal Stem Cell Laboratory, Adelaide Medical School, Faculty of Health and Medical Sciences, University of Adelaide, Adelaide, SA, Australia 3South Australian Health and Medical Research Institute, Adelaide, SA, Australia 4Central Adelaide Local Health Network, Adelaide, SA, Australia Stroke is a leading cause of permanent disability world-wide, but aside from rehabilitatio...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Fight Aging! provides a weekly digest of news and commentary for thousands of subscribers interested in the latest longevity science: progress towards the medical control of aging in order to prevent age-related frailty, suffering, and disease, as well as improvements in the present understanding of what works and what doesn't work when it comes to extending healthy life. Expect to see summaries of recent advances in medical research, news from the scientific community, advocacy and fundraising initiatives to help speed work on the repair and reversal of aging, links to online resources, and much more. This content is...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
CONCLUSION: GPIbα does not significantly impact platelet gene therapy of HA with inhibitors. 2bF8 gene therapy restores hemostasis and promotes immune tolerance in HA mice with pre-existing immunity. This article is protected by copyright. All rights reserved. PMID: 30609275 [PubMed - as supplied by publisher]
Source: Thrombosis and Haemostasis - Category: Hematology Authors: Tags: J Thromb Haemost Source Type: research
Background: While novel agents have allowed for successful outpatient treatment of patients with multiple myeloma (MM), multi-agent infusional chemotherapy regimens continue to have a role, particularly in high burden disease states. However, the use of platinum-based regimens is limited in patients with renal insufficiency and renal failure. We have been using a modified hyperCVAD regimen, termed HyperCD (Table 1), with omission of vincristine, and inclusion of a proteasome inhibitor (PI, either bortezomib or carfilzomib) in these aggressive clinical presentations for rapid tumor reduction. Here we present our experience ...
Source: Blood - Category: Hematology Authors: Tags: 653. Myeloma: Therapy, excluding Transplantation: Poster II Source Type: research
CONCLUSIONSOur incidence approaches the literature, albeit the series of published cases are heterogeneous and with variable differences. Although the incidence of thrombosis in these patients is a cause of marked morbidity and mortality, the risk of bleeding also increases, therefore routine prophylaxis is not recommended in all patients. We must undergo an exhaustive analysis of the data to identify individually which patients may be candidates for prophylaxis, with the aim of reducing the incidence without raising the hemorrhagic risk of our patients.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - Category: Hematology Authors: Tags: 332. Antithrombotic Therapy Source Type: research
ConclusionsIn this pilot study of 50 patients, low-dose apixaban was safe and well tolerated as thromboprophylaxis for patients with MM receiving IMiDs. No patients experienced VTE, major hemorrhage, stroke, or MI. Further randomized studies are needed to validate apixaban as a standard primary prevention anti-thrombotic strategy for patients with MM receiving IMiDs.DisclosuresMoslehi: Bristol-Myers Squibb: Consultancy, Research Funding. Jagasia: Incyte Corporation: Membership on an entity's Board of Directors or advisory committees.
Source: Blood - Category: Hematology Authors: Tags: 332. Antithrombotic Therapy: Poster I Source Type: research
Overall incidence of hemostatic complications in pediatric recipients of Hematopoietic Stem Cell Transplant (HSCT) is scarcely studied. This retrospective review explored the incidence and underlying risk factors of bleeding and thrombotic complications in children.
Source: Thrombosis Research - Category: Hematology Authors: Tags: Full Length Article Source Type: research
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