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

Linnik, Yevgeniy A.a; Salvatore, Lauren T.a; Lowrey, Christopher H.b,c; Ornstein, Deborah L.a,b,c

Blood Coagulation & Fibrinolysis: December 2019 - Volume 30 - Issue 8 - p 419–422
doi: 10.1097/MBC.0000000000000850
CASE REPORTS
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Primary myelofibrosis (PMF) is a clonal hematopoietic stem cell disorder characterized by fibrosis of the marrow cavity, marked megakaryocyte atypia and progressive cytopenias. Although thrombosis predominates, bleeding is the primary manifestation in up to 20% of patients and may be life-threatening. 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.

aDepartment of Pathology & Laboratory Medicine

bDepartment of Medicine (Hematology/Oncology) Dartmouth-Hitchcock Medical Center, Norris Cotton Cancer Center, Lebanon

cGeisel School of Medicine at Dartmouth, Hanover, New Haven, USA

Correspondence to Yevgeniy A. Linnik, MD, Department of Pathology & Laboratory Medicine, Dartmouth Hitchcock Medical Center and Norris Cotton Cancer Center, One Medical Center Drive, Lebanon, NH 03756, USA Tel: +1 603 650 8523; fax: +1 603 650 7214; e-mail: Yevgeniy.Linnik@vumc.org

Received 25 March, 2019

Accepted 14 August, 2019

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