scholarly journals Treatment options and nursing care for the pediatric aplastic anemia patient requriring bone marrow or stem cell transplantation

2005 ◽  
Vol 11 (2) ◽  
pp. 98
Author(s):  
J.H. Baker ◽  
M.A. Frey ◽  
J. Kurtzberg
Keyword(s):  
Bone Marrow ◽  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Aplastic Anemia ◽  
Cell Transplantation ◽  
Nursing Care ◽  
Treatment Options ◽  
Aplastic Anemia Patient ◽  
Anemia Patient

Stem cell defect in aplastic anemia: reduced long term culture-initiating cells (LTC-IC) in CD34+ cells isolated from aplastic anemia patient bone marrow

2002 ◽  
Vol 3 (5) ◽  
pp. 230-236 ◽  
Author(s):  
Sian Rizzo ◽  
John Scopes ◽  
Modupe O Elebute ◽  
Helen A Papadaki ◽  
Edward C Gordon-Smith ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Stem Cell ◽  
Aplastic Anemia ◽  
Long Term Culture ◽  
Aplastic Anemia Patient ◽  
Cd34 Cells ◽  
Anemia Patient ◽  
Cell Defect

scholarly journals Successful Hematopoietic Stem Cell Transplantation from a Matched Related Donor with Beta-Thalassemia Minor for Severe Aplastic Anemia

Children ◽  
2020 ◽  
Vol 7 (10) ◽  
pp. 162
Author(s):  
Mi Young Jung ◽  
Young Tae Lim ◽  
Hyunji Lim ◽  
Jeong Ok Hah ◽  
Jae Min Lee
Keyword(s):  
Bone Marrow ◽  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Aplastic Anemia ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Severe Aplastic Anemia ◽  
Hematopoietic Stem ◽  
Thalassemia Minor

The first-line treatment for severe aplastic anemia (SAA) patients is hematopoietic stem cell transplantation (HSCT), with full-matched related donors considered the most suitable. We report a case of SAA in which the patient successfully underwent HSCT from a donor with β-thalassemia minor. The patient in this case underwent HSCT from a human leukocyte antigen (HLA)-matched younger brother with β-thalassemia minor. A 7-year-old girl was referred to our facility following a 6-month history of easy bruising and pallor. Laboratory examinations showed pancytopenia and hypocellular bone marrow with cellularity of <5%. She was diagnosed with acquired SAA, and HLA typing of her family members was performed. Her younger brother was an HLA-matched sibling but had β-thalassemia minor. Since his hemoglobin levels were maintained at 10–11 d/dL, he was considered a suitable HSCT donor. The conditioning regimen included fludarabine, cyclophosphamide, and anti-thymocyte globulin. The CD34+ and CD3+ cell counts were 6.6 × 106/kg and 0.48 × 108/kg, respectively. White blood cell engraftment was evident on day +11. Regimen-associated toxicities, such as anorexia and enteritis, were mild; no infections occurred, and no symptoms of acute graft-versus-host disease (GVHD) were observed. The 30-day follow-up bone marrow examination revealed normocellular marrow with 80%–90% cellularity. Acute or chronic GVHD has not been reported, and good performance status has been observed throughout the 5 years after HSCT. β-thalassemia minor patients can be considered as bone marrow donors for SAA patients.

scholarly journals Updates in acquired aplastic anemia: Can we do more for our patients?

2015 ◽  
Vol 34 (1-2) ◽  
pp. 1-31
Author(s):  
Ana-Maria Moldovianu ◽  
Anca Popp ◽  
Zsofia Varady ◽  
Alina Tanase ◽  
Alexandra Marculescu ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Aplastic Anemia ◽  
Cell Transplantation ◽  
Young Patients ◽  
Cell Transplant ◽  
Front Line ◽  
Sibling Donor ◽  
Matched Sibling

Abstract The purpose of this work is to present the results of allogeneic stem cell transplantation as therapy for patients diagnosed with acquired aplastic anemia in the Department of Bone Marrow Transplantation of Fundeni Clinical Institute and to elaborate an algorithm of treatment in aplastic anemia starting with the observations obtained from our clinical practice and following the European treatment guidelines in this group of patients. Aplastic Anemia (AA) is a rare hematological disease characterized by pancytopenia and a hypocellular bone marrow. The paradigm of bone marrow failure syndromes, aplastic anemia is a diagnosis of exclusion despite the precision of its diagnosis criteria. Although AA is not a malignant disease, but an autoimmune disorder, the grave consequences of pancytopenia and clonal transformation into acute leukemia make it a potentially fatal condition. The management of AA patients is challenging and necessitates a very well established treatment plan from the diagnosis. We present the treatment algorithm for AA patients with recommendations based on both recent guidelines in the field and on our experience treating AA patients with allogeneic stem cell transplant. Therapeutic procedure algorithm comprises different approaches for different patient populations, age categories and availability of immunosuppression therapy or different types of donors. According to the recent EBMT recommendations the treatment of choice for young patients (younger than 40 years) who have a matched sibling donor is hematopoietic stem cell transplantation (HSCT). For those patients who don’t have a matched sibling donor or are not candidates for HSCT due to older age, the immunosuppression with ATG and cyclosporine is an efficient treatment. The supportive care has an important role and the patients with aplastic anemia should be managed by a multidisciplinary team. For patients older than 40 years, the choice between immunosuppressive therapy (IST) and upfront transplant with HLA identical sibling donor remains a key question. However, the standard approaches for this category of patients is front line immunosuppression with ATG and cyclosporine and if they become refractory to at least one course of IST the allogeneic stem cell transplant using fludarabine-based conditioning is the second-line treatment option. In our institution there were eleven AA patients treated with allogeneic stem cell transplantation from 2009 till 2015. They were all young patients with age between 19 and 42 years old and all had severe acquired aplastic anemia with transfusion dependence. Six cases were transplanted from a matched sibling donor and five patients had undergone an unrelated matched donor transplant. The allogeneic HSCT procedure was done both as front line therapy in the case of three patients and as second treatment choice in the rest of eight patients. Four patients died, three of them due to transplant related toxicity and one patient experienced severe autoimmune reaction with transfusion inefficacy complicated with intracerebral haemorrhage at four months from transplant. In our opinion the most challenging aspect in treating AA patients is choosing the best treatment option taking into account the patient age and performance status, the severity of the disease and the availability of a donor for allogeneic HSCT. Although the treatment strategy must be individualized in every patient case, it is necessary to make a standardization of treatment procedures in AA and to follow the evidence based recommendations available in the management of this rare disease.

scholarly journals Brazilian Consensus Meeting on Pediatric Hematopoietic Stem Cell Transplantation for Acquired Aplastic Anemia and Inherited Bone Marrow Failure Syndromes

JBMTCT ◽  
2021 ◽  
Vol 2 (4) ◽  
pp. 151
Author(s):  
Luiz Guilherme Darrigo Junior ◽  
Gisele Loth ◽  
Phillip Scheinberg ◽  
Elias Hallack Atta ◽  
Carmem Bonfim
Keyword(s):  
Bone Marrow ◽  
Stem Cell ◽  
Hematopoietic Stem Cell Transplantation ◽  
Stem Cell Transplantation ◽  
Aplastic Anemia ◽  
Cell Transplantation ◽  
Hematopoietic Stem Cell ◽  
Bone Marrow Failure ◽  
Hematopoietic Stem ◽  
Bone Marrow Failure Syndromes

THE BRAZILIAN SOCIETY FOR BLOOD AND MARROW TRANSPLANTATION (SBTMO) PRESENTS THE BRAZILIAN GUIDELINES ON HEMATOPOIETIC STEM CELL TRANSPLANTATION FOR ACQUIRED APLASTIC ANEMIA AND INHERITED BONE MARROW FAILURE SYNDROMES

Donor-Type Myelodysplastic Syndrome with t(2;3) and Monosomy 7 after Allogeneic Peripheral Blood Stem Cell Transplantation and Liver Transplantation in a Patient with Severe-Type Aplastic Anemia.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5357-5357
Author(s):  
Satoshi Hashino ◽  
Fumie Fujisawa ◽  
Takeshi Kondo ◽  
Masahiro Imamura ◽  
Kazuya Sato ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Liver Transplantation ◽  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Aplastic Anemia ◽  
Cell Transplantation ◽  
Drug Induced ◽  
Monosomy 7 ◽  
Allogeneic Pbsct ◽  
Donor Type

Abstract Secondary clonal hematological disease in donor cells has rarely been reported as a complication of allogeneic stem cell transplantation in hematological disease. We report a case of myelodysplastic syndrome that showed cytogenetic abnormalities of t(2;3) and monosomy 7, which developed two years after peripheral blood stem cell transplantation for aplastic anemia and one year after liver transplantation for drug-induced hepatic failure. A 23-year-old female was diagnosed as having a severe-type aplastic anemia in April, 2000. Treatment with immunosuppressive drugs was not successful. Nineteen months later, she received allogeneic PBSCT from an HLA-matched elder on November 12, 2001. Her clinical course after PBSCT was marked by serious drug-induced hepatitis. Ten months after allogeneic PBSCT, she suddenly developed hepatic failure. On September 29, 2002, she was emergently transplanted with living donor-derived liver from the sibling who was the donor for allogeneic PBSCT. One month after liver transplantation (LT), pancytopenia had gradually progressed. At that time, chromosomal analysis showed 46, XX (20/20), and chimerism analysis of PB showed 100% donor-type. Late graft rejection of bone marrow was not suspected due to there being no relative lymphocytosis and the persistence of 100% complete donor-type chimerism. Thereafter, pancytopenia worsened, even with the stable complete donor-type chimerism, and G-CSF and EPO were administered. Bone marrow aspirate at seven months after the start of cytokine therapy showed trilineage dysplasia, suggesting secondary myelodysplasia. Cytogenetic analysis at that time showed t(2;3) in 17/20 cells. Eight months later, at 31 months post allogeneic PBSCT and 21 months post-LT, her bone marrow showed chromosomal abnormality with t(2;3) and additional monosomy 7 in 14/20 cells. There was no evidence of graft rejection because of sustained 100% donor-type chimerism. There was no possibility of second SCT because of hepatic dysfunction and hyperbilirubinemia even after LT as well as repeated intra-abdominal bacterial infection through a gastrostomy tube for nutrition. Bone marrow aspirations performed two and six months later showed an increased proportion of t(2;3) and monosomy 7 to 100%. She suddenly died of intra-abdominal bleeding due to profound thrombocytopenia 42 months after allogeneic PBSCT and 32 months after LT. The donor is currently alive and well with completely normal hematological data. This secondary malignancy of donor origin is most frequently seen in patients with leukemia. We suspect that the chromosomal abnormalities are related to hepatitis-associated aplastic anemia, administration of granulocyte colony-stimulating factor and erythropoietin for post-transplant pancytopenia, and repeated infections after liver transplantation.

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