title: Diagnosis, prevention, and management of bleeding episodes in Philadelphia-negative myeloproliferative neoplasms: recommendations by the Hemostasis Working Party of the German Society of Hematology and Medical Oncology (DGHO) and the Society of Thrombosis and Hemostasis Research (GTH).
abstract: Philadelphia-negative myeloproliferative neoplasms (Ph-negative MPN) comprise a heterogeneous group of chronic hematologic malignancies. The quality of life, morbidity, and mortality of patients with MPN are primarily affected by disease-related symptoms, thromboembolic and hemorrhagic complications, and progression to myelofibrosis and acute leukemia. Major bleeding represents a common and important complication in MPN, and the incidence of such bleeding events will become even more relevant in the future due to the increasing disease prevalence and survival of MPN patients. This review discusses the causes, differential diagnoses, prevention, and management of bleeding episodes in patients with MPN, aiming at defining updated standards of care in these often challenging situations.
authors.affiliation: Department of Hematology, Oncology, Hemostaseology, and Stem Cell Transplantation, Faculty of Medicine, RWTH Aachen University, 52074, Aachen, Germany.
authors.forename: Iris
authors.initials: I
authors.lastname: Appelmann
authors.affiliation: Department of Hematology and Oncology, Charite Berlin, Berlin, Germany.
authors.forename: Stephan
authors.initials: S
authors.lastname: Kreher
authors.affiliation: Department of Hematology, Oncology, and Palliative Medicine, Rems-Murr-Klinikum Winnenden, Winnenden, Germany.
authors.forename: Stefani
authors.initials: S
authors.lastname: Parmentier
authors.affiliation: Department of Internal Medicine IV, Martin-Luther-University Halle-Wittenberg, Halle (Saale), Germany.
authors.forename: Hans-Heinrich
authors.initials: HH
authors.lastname: Wolf
authors.affiliation: Department of Medicine I, Mathias Spital Rheine, Rheine, Germany.
authors.forename: Guido
authors.initials: G
authors.lastname: Bisping
authors.affiliation: Department of Hematology, Oncology, Hemostaseology, and Stem Cell Transplantation, Faculty of Medicine, RWTH Aachen University, 52074, Aachen, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Kirschner
authors.affiliation: Medizinisches Versorgungszentrum Wagnerstibbe, Hannover, Germany.
authors.forename: Frauke
authors.initials: F
authors.lastname: Bergmann
authors.affiliation: Department of Hematology and Oncology, University Hospital Jena, Jena, Germany.
authors.forename: Kristina
authors.initials: K
authors.lastname: Schilling
authors.affiliation: Department of Hematology, Oncology, Hemostaseology, and Stem Cell Transplantation, Faculty of Medicine, RWTH Aachen University, 52074, Aachen, Germany.
authors.forename: Tim H
authors.initials: TH
authors.lastname: Brümmendorf
authors.affiliation: Hematology Oncology Centre, Ludwig Maximilians University of Munich Medical School, Munich, Germany.
authors.forename: Petro E
authors.initials: PE
authors.lastname: Petrides
authors.affiliation: Department of Haematology, Haemostasis, Oncology and Stem-Cell Transplantation, Hannover Medical School, Hannover, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Tiede
authors.affiliation: Clinic for Internal Medicine II, Dept. of Hematology, Oncology, Asklepios Clinic Uckermark, Schwedt/Oder, Germany.
authors.forename: Axel
authors.initials: A
authors.lastname: Matzdorff
authors.affiliation: Johannes Wesling Academic Medical Center, Minden, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Griesshammer
authors.affiliation: Department of Hematology and Oncology, Charite Berlin, Berlin, Germany.
authors.forename: Hanno
authors.initials: H
authors.lastname: Riess
authors.affiliation: Department of Hematology, Oncology, Hemostaseology, and Stem Cell Transplantation, Faculty of Medicine, RWTH Aachen University, 52074, Aachen, Germany. skoschmieder@ukaachen.de.
authors.forename: Steffen
authors.initials: S
authors.lastname: Koschmieder
mesh_terms: D000925:Anticoagulants / Q000009:adverse effects / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D016063:Blood Loss, Surgical / Q000517:prevention & control; D001803:Blood Transfusion; D001808:Blood Vessels / Q000187:drug effects / Q000473:pathology; D002986:Clinical Trials as Topic; D000075202:Contraindications; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D019468:Disease Management; D017558:Elective Surgical Procedures; D006470:Hemorrhage / Q000175:diagnosis / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy; D006489:Hemostatic Techniques; D006801:Humans; D054438:Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative / Q000150:complications / Q000188:drug therapy / Q000235:genetics; D008099:Liver / Q000503:physiopathology; D015337:Multicenter Studies as Topic; D009196:Myeloproliferative Disorders / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D010975:Platelet Aggregation Inhibitors / Q000009:adverse effects / Q000627:therapeutic use; D010979:Platelet Function Tests; D017713:Platelet Transfusion; D019106:Postoperative Hemorrhage / Q000209:etiology / Q000517:prevention & control / Q000628:therapy; D019851:Thrombophilia / Q000188:drug therapy / Q000209:etiology; D014148:Tranexamic Acid / Q000627:therapeutic use; D014842:von Willebrand Diseases / Q000209:etiology; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D000925:Anticoagulants; D000970:Antineoplastic Agents; D001779:Blood Coagulation Factors; D010975:Platelet Aggregation Inhibitors; D014841:von Willebrand Factor; D014148:Tranexamic Acid; D003894:Deamino Arginine Vasopressin
keywords: Bleeding; Essential thrombocythemia; Hemorrhage; MPN; Management; Myeloproliferative neoplasms; Polycythemia vera; Review