TY - JOUR
T1 - Donor cell-derived myelofibrosis relapse after allogeneic stem cell transplantation
AU - Sica, Simona
PY - 2023
Y1 - 2023
N2 - 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 (BM) 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 these 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; 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
AB - 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 (BM) 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 these 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; 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
KW - Donor cell-derived myelofibrosis relapse after allogeneic stem cell
KW - Donor cell-derived myelofibrosis relapse after allogeneic stem cell
UR - http://hdl.handle.net/10807/229400
U2 - 10.3324/haematol.2022.281564
DO - 10.3324/haematol.2022.281564
M3 - Article
SN - 0390-6078
VL - 108
SP - 278
EP - 282
JO - Haematologica
JF - Haematologica
ER -