scholarly journals Haploidentical hematopoietic stem cell transplantation in a myelofibrosis patient with primary graft failure

2018 ◽  
Vol 9 (4) ◽  
Author(s):  
Cristina Tecchio ◽  
Angelo Andreini ◽  
Claudio Costantini ◽  
Alberto Zamò ◽  
Donata De Sabata ◽  
...  

The prognosis of patients affected by myelofibrosis (MF) is usually dismal and allogeneic hematopoietic stem cell transplantation (HSCT) remains the only cure. The number of HSCTs in MF patients has recently increased. However, a major obstacle is still represented by primary graft failure (PGF). Currently there are no definitive guidelines for the treatment of PGF and a second HSCT can be performed only when an allogeneic donor is rapidly available. Herein we report on a MF patient with PGF after an unrelated HSCT, who was rescued by a non-myeloablative, unmanipulated, haploidentical HSCT that resulted in persistent engraftment and bone-marrow fibrosis regression, but not in a long-term disease control. Based on this experience we briefly review the role of different conditioning regimens and hematopoietic stem cell sources in the setting of HSCT for MF patients with PGF. The role of haploidentical donors in MF patients lacking HLAmatched relatives is also discussed.

Haematologica ◽  
2019 ◽  
Vol 104 (11) ◽  
pp. 2314-2323 ◽  
Author(s):  
Pietro Merli ◽  
Ignazio Caruana ◽  
Rita De Vito ◽  
Luisa Strocchio ◽  
Gerrit Weber ◽  
...  

2018 ◽  
Vol 25 (35) ◽  
pp. 4535-4544 ◽  
Author(s):  
Annalisa Ruggeri ◽  
Annalisa Paviglianiti ◽  
Fernanda Volt ◽  
Chantal Kenzey ◽  
Hanadi Rafii ◽  
...  

Background: Circulating endothelial cells (CECs), originated form endothelial progenitors (EPCs) are mature cells not associated with vessel walls and detached from the endothelium. Normally, they are present in insignificant amounts in the peripheral blood of healthy individuals. On the other hand, elevated CECs and EPCs levels have been reported in the peripheral blood of patients with different types of cancers and other diseases. Objective: This review aims to provide an overview on the characterization of CECs and EPCs, to describe isolation methods and to identify the potential role of these cells in hematological diseases and hematopoietic stem cell transplantation. Methods: We performed a detailed search of peer-reviewed literature using keywords related to CECs, EPCs, allogeneic hematopoietic stem cell transplantation, and hematological diseases (hemoglobinopathies, hodgkin and non-hodgkin lymphoma, acute leukemia, myeloproliferative syndromes, chronic lymphocytic leukemia). Results: CECs and EPCs are potential biomarkers for several clinical conditions involving endothelial turnover and remodeling, such as in hematological diseases. These cells may be involved in disease progression and in the neoplastic process. Moreover, CECs and EPCs are probably involved in endothelial damage which is a marker of several complications following allogeneic hematopoietic stem cell transplantation. Conclusion: This review provides information about the role of CECs and EPCs in hematological malignancies and shows their implication in predicting disease activity as well as improving HSCT outcomes.


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