scholarly journals A case of primary nonleukemic myeloid sarcoma of the spleen, successfully treated by surgery and hematopoietic stem cell transplantation

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Asuka Ono ◽  
Yuki Kitano ◽  
Katsunori Imai ◽  
Takashi Matsumoto ◽  
Shinya Endo ◽  
...  

Abstract Background Myeloid sarcoma (MS) is a rare disease, mostly found in conjunction with acute myelogenous leukemia or other diseases, and primary nonleukemic MS of the spleen is particularly rare. Case presentation We report a 57-year-old male who presented with a spleen mass that was found incidentally, and was enlarged. As a result of various examinations, he was diagnosed with primary MS of the spleen with suspected involvement of the transverse colon, left kidney, pancreatic tail, and left diaphragm. He underwent a total splenectomy, partial pancreatectomy, partial colectomy, left nephrectomy, and left diaphragm partial resection. Histological examination revealed splenic primary MS. Bone marrow biopsy and immunophenotypic flow cytometry revealed no evidence of myeloid leukemia. He underwent umbilical cord blood transplantation, and he is currently living without a sign of recurrence at 10 months after surgery. Conclusions We experienced a very rare case of primary spleen MS that was discovered without a hematologic malignancy. Two cases of surgically resected primary splenic MS have been reported, including the present case.

2017 ◽  
Vol 6 (1) ◽  
pp. 31-37
Author(s):  
Nihar Ranjan Sarker ◽  
Ashis Kumar Ghosh ◽  
Santosh Kumar Saha ◽  
Abdullah Shahriar

The discussion of disease management focuses on the use of transfusion therapy and the newly developed oral iron chelators, deferiprone and deferasirox, especially combination of the chelator drugs. It has been also discussed on splenectomy and pediatrician management of endocrinopathies and cardiac complications. In addition, the use of hematopoietic stem cell transplantation has produced cure rates as high as 97%, and the use of cord blood transplantation as well. Major advances have being made in the discovery of critical modifier genes, such as Myb and especially BCL11A (B cell lymphoma 11A), a master regulator of HbF (fetal hemoglobin) and hemoglobin switching. Finally, the year 2010 has brought in the first successful experiment of gene therapy in a ß-thalassemia patient, opening up the perspective of a generalized cure for all ß- Thalassaemia patients.J Shaheed Suhrawardy Med Coll, June 2014, Vol.6(1); 31-37


Author(s):  
Liangliang Ren ◽  
Ling Li ◽  
Lei Zhang ◽  
Xin Li ◽  
xiaorui Fu ◽  
...  

Umbilical cord blood allogeneic hematopoietic stem cell transplantation(UCBT) has been gradually applied in the treatment of patients with blood system diseases. This paper reports a case of a child patient with highly invasive T-cell lymphoma who underwent UCBT after chemotherapy and developed minimal change glomerulopathy after transplantation.


Blood ◽  
2011 ◽  
Vol 117 (17) ◽  
pp. 4460-4466 ◽  
Author(s):  
Sandra N. Catlin ◽  
Lambert Busque ◽  
Rosemary E. Gale ◽  
Peter Guttorp ◽  
Janis L. Abkowitz

Abstract Hematopoietic stem cells (HSCs) replicate (self-renew) to create 2 daughter cells with capabilities equivalent to their parent, as well as differentiate, and thus can both maintain and restore blood cell production. Cell labeling with division-sensitive markers and competitive transplantation studies have been used to estimate the replication rate of murine HSCs in vivo. However, these methods are not feasible in humans and surrogate assays are required. In this report, we analyze the changing ratio with age of maternal/paternal X-chromosome phenotypes in blood cells from females and infer that human HSCs replicate on average once every 40 weeks (range, 25-50 weeks). We then confirm this estimate with 2 independent approaches, use the estimate to simulate human hematopoiesis, and show that the simulations accurately reproduce marrow transplantation data. Our simulations also provide evidence that the number of human HSCs increases from birth until adolescence and then plateaus, and that the ratio of contributing to quiescent HSCs in humans significantly differs from mouse. In addition, they suggest that human marrow failure, such as the marrow failure that occurs after umbilical cord blood transplantation and with aplastic anemia, results from insufficient numbers of early progenitor cells, and not the absence of HSCs.


Blood ◽  
2010 ◽  
Vol 116 (4) ◽  
pp. 649-652 ◽  
Author(s):  
Shinsuke Takagi ◽  
Yasunori Ota ◽  
Naoyuki Uchida ◽  
Koichi Takahashi ◽  
Kazuya Ishiwata ◽  
...  

Abstract Although allogeneic hematopoietic stem cell transplantation has recently been applied to patients with myelofibrosis with reproducible engraftment and resolution of marrow fibrosis, no data describe the outcomes of umbilical cord blood transplantation. We describe 14 patients with primary (n = 1) and secondary myelofibrosis (n = 13) who underwent reduced-intensity umbilical cord blood transplantation. Conditioning regimens included fludarabine and graft-versus-host disease prophylaxis composed cyclosporine/tacrolimus alone (n = 6) or a combination of tacrolimus and mycophenolate mofetil (n = 8). Thirteen patients achieved neutrophil engraftment at a median of 23 days. The cumulative incidence of neutrophil and platelet engraftment was 92.9% at day 60 and 42.9% at day 100, respectively. Posttransplantation chimerism analysis showed full donor type in all patients at a median of 14 days. The use of umbilical cord blood could be feasible even for patients with severe marrow fibrosis, from the viewpoint of donor cell engraftment.


Author(s):  
Juan Montoro ◽  
Jaime Sanz

Umbilical cord-blood (UCB) is a well-recognized alternative source of stem cells for unrelated donor hematopoietic stem cell transplantation (HSCT). As compared with other stem cell sources from adult donors, it has the advantages of immediate availability of cells, absence of risk to the donor and reduced risk of graft-versus-host disease despite donor-recipient HLA disparity. However, the use of UCB is limited by the delayed post-transplant hematologic recovery due, at least in part, to the reduced number of hematopoietic cells in the graft and the delayed or incomplete immune reconstitution. As a result, severe infectious complications continue to be a leading cause of morbidity and mortality following UCB transplantation (UCBT). We will address the complex differences in the immune properties of UCB and review the incidence, characteristics, risk factors, and severity of bacterial, fungal and viral infectious complications in patients undergoing UCBT.


2019 ◽  
Vol 5 (8) ◽  
pp. FSO412 ◽  
Author(s):  
Akane Takamatsu ◽  
Yasuaki Tagashira ◽  
Shinya Hasegawa ◽  
Hitoshi Honda

Human adenoviruses cause a wide spectrum of illnesses, including invasive infections, in immunocompromised hosts. We report a case of disseminated adenovirus infection following unrelated cord–blood transplantation in a 46-year-old male with a lymphoma. A review of the literature on disseminated adenovirus infections in adult patients with hematopoietic stem cell transplantation has also been included. Despite antiviral therapy, the mortality rate in hematopoietic stem cell transplantation recipients with a disseminated adenovirus infection is as high as 72%, and estimating the risk of human adenovirus infection in a timely manner is crucial to improving outcomes.


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