Evidence Of Hematopoietic Stem Cell Dysfunction In Murine Bmpr2-Related Pulmonary Arterial Hypertension

Author(s):  
Rizwan Hamid ◽  
Ling Yan ◽  
James D. West ◽  
James E. Loyd ◽  
Linda Robinson ◽  
...  
2016 ◽  
Vol 6 (4) ◽  
pp. 611-613 ◽  
Author(s):  
Saadia A. Faiz ◽  
Cezar Iliescu ◽  
Juan Lopez-Mattei ◽  
Bela Patel ◽  
Lara Bashoura ◽  
...  

We present the case of a 62-year-old man with myelofibrosis-associated pulmonary arterial hypertension (PAH) who underwent allogeneic hematopoietic stem cell transplantation with subsequent resolution of disease and PAH. Right heart catheterization was used to guide PAH therapy before and after transplantation. Drug interactions, adverse effects, and renal insufficiency posed clinical challenges for the management of PAH-specific medications after transplantation. PAH improved soon after transplantation, and vasoactive medications were tapered off. Resolution of PAH was confirmed with repeat measurement of pulmonary hemodynamic characteristics. Although the etiology and pathophysiology for the resolution of PAH was unclear, the myelopulmonary pathophysiologic link was likely to have contributed. This is the first report describing resolution of myelofibrosis-associated PAH after allogeneic hematopoietic stem cell transplantation.


2021 ◽  
pp. 204589402110504
Author(s):  
Gretchen Louise Hackett ◽  
Farrah Munir ◽  
Teresa Shapiro ◽  
Robert Greiner ◽  
Daniel J. McKeone ◽  
...  

Pulmonary arterial hypertension (PAH) in pediatric patients is associated with significant morbidity and mortality. Few studies exist to evaluate the incidence of PAH in pediatric oncology patients treated with carboplatin and thiotepa followed by hematopoietic stem cell transplant (HSCT). We describe two pediatric patients who developed PAH following high dose chemotherapy with carboplatin and thiotepa followed by autologous HSCT. These cases highlight the need for a surveillance protocol and reinforce the need for cardiologist-oncologists or close collaboration between providers in these sub-specialties who provide care to patients undergoing this type of chemotherapy regimen.


Blood ◽  
2014 ◽  
Vol 124 (10) ◽  
pp. 1622-1625 ◽  
Author(s):  
Serine Avagyan ◽  
Michael Churchill ◽  
Kenta Yamamoto ◽  
Jennifer L. Crowe ◽  
Chen Li ◽  
...  

Key Points XLF-deficient mice recapitulate the lymphocytopenia of XLF-deficient patients. Premature aging of hematopoietic stem cells underlies the severe and progressive lymphocytopenia in XLF-deficient mice.


CHEST Journal ◽  
2012 ◽  
Vol 142 (4) ◽  
pp. 118A
Author(s):  
Hala Moukhachen ◽  
Prabalini Rajendram ◽  
Sanjay Chawla ◽  
Nina Raoof ◽  
Kaye Hale ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document