Primary myelofibrosis (PMF) is a rare myeloproliferative neoplasm characterized by clonal proliferation of mature myeloid lineages derived from stem cells (erythrocytes, leukocytes and magakaryocytes) with variable megakaryocyte atypia associated with reticulin and / or collagen bone marrow fibrosis, osteosclerosis, ineffective erythropoiesis, angiogenesis, extramedullary hematopoiesis and abnormal expression of cytokines. Allogeneic hemopoietic stem cell transplantation (alloHSCT) is currently the only curative approach for patients with myelofibrosis, and for this reason the number of allografts for this indications have been growing over the past years. Unfortunately relapse of myelofibrosis (MF) after an alloHSCT occurs in 10-40% of cases: patients usually present with a declining donor chimerism, and a reappearance of driver mutations if present; a bone marrow (BM) biopsy is usually consistent with typical megakaryocyte abnormalities and stromal fibrosis. Ultimately BM cells exhibit progressive loss of donor chimerism, and the relapse is therefore of recipient origin. Here we report two allografted MF patients who relapsed in donor cells

Sora', F., Donor cell-derived myelofibrosis relapse after allogenic stem cell transplantation, <<HAEMATOLOGICA>>, 2019; (104): 77-78 [http://hdl.handle.net/10807/217950]

Donor cell-derived myelofibrosis relapse after allogenic stem cell transplantation

Sora', Federica
Penultimo
Membro del Collaboration Group
2019

Abstract

Primary myelofibrosis (PMF) is a rare myeloproliferative neoplasm characterized by clonal proliferation of mature myeloid lineages derived from stem cells (erythrocytes, leukocytes and magakaryocytes) with variable megakaryocyte atypia associated with reticulin and / or collagen bone marrow fibrosis, osteosclerosis, ineffective erythropoiesis, angiogenesis, extramedullary hematopoiesis and abnormal expression of cytokines. Allogeneic hemopoietic stem cell transplantation (alloHSCT) is currently the only curative approach for patients with myelofibrosis, and for this reason the number of allografts for this indications have been growing over the past years. Unfortunately relapse of myelofibrosis (MF) after an alloHSCT occurs in 10-40% of cases: patients usually present with a declining donor chimerism, and a reappearance of driver mutations if present; a bone marrow (BM) biopsy is usually consistent with typical megakaryocyte abnormalities and stromal fibrosis. Ultimately BM cells exhibit progressive loss of donor chimerism, and the relapse is therefore of recipient origin. Here we report two allografted MF patients who relapsed in donor cells
Inglese
Sora', F., Donor cell-derived myelofibrosis relapse after allogenic stem cell transplantation, <<HAEMATOLOGICA>>, 2019; (104): 77-78 [http://hdl.handle.net/10807/217950]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10807/217950
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