Acquired Aplastic Anemia

Teo, Juliana; Dror, Yigal

doi:10.1002/9780470987001.ch4

Cited by 2 publications

(5 citation statements)

References 162 publications

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“…Severe aplastic anemia (SAA) was previously considered a fatal hematologic disease; however, advances in supportive care and therapeutic options improved survival rate, quality of life, and long‐term outcomes in this group of patient. In cases where a human leukocyte antigen (HLA)‐identical related donor is available, hematopoietic stem cell transplantation (HSCT) is widely accepted as the treatment of choice for SAA 1. The current cure rate with such treatment is 90% 2.…”

Section: Introductionmentioning

confidence: 99%

Outcome of children with aplastic anemia treated with immunosuppressive therapy

Pongtanakul

Das

Charpentier

et al. 2007

Pediatric Blood & Cancer

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Background Immunosuppressive therapy (IST) is the alternative treatment in children with aplastic anemia (AA) who do not have an HLA‐matched sibling. The aim of this study is to evaluate the outcome of children with AA treated with IST. Methods We retrospectively reviewed the hospital records of children with AA from 1984 to 2004, treated at our institution with antithymocyte globulin (ATG), cyclosporine (CS), and short course of prednisone. Result Forty‐two patients were treated with IST (24 boys, 18 girls); of whom 26% received G‐CSF. The median age at diagnosis was 8.5 years. Sixty‐nine, 19, and 12% were diagnosed with severe, very severe, and moderate AA, respectively. Twenty‐one percent had hepatitis‐associated AA. Median follow‐up time was 53.3 months. Sixty‐two percent had complete response; 19% had partial response. Two patients relapsed and received a second course of ATG; both had a partial response. The actuarial 5 years survival rate was 67.5%. Two patients developed myelodysplastic syndrome (MDS); both received long‐term G‐CSF and had partial response after two courses of IST. Fifteen percent of survivors had significant hypertension which persisted after CS was discontinued. Conclusions This study shows promising response in children with AA treated with IST; however, the outcome was inferior to our institutional results with hematopoietic stem cell transplantation from a sibling donor. Hypertension and MDS are late complications. Longer follow‐up, larger cohorts, and prospective studies are warranted to evaluate late complications and risk factors. Pediatr Blood Cancer 2008;50:52–57. © 2007 Wiley‐Liss, Inc.

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Section: Introductionmentioning

confidence: 99%

Outcome of children with aplastic anemia treated with immunosuppressive therapy

Pongtanakul

Das

Charpentier

et al. 2007

Pediatric Blood & Cancer

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“…This was in accordance with some literatures that showed the incidence of SAA with nearly similar affectsin boys and girls. [1,2,3] Melayu race had the highest ranks of SAA patients (36.1%), because it was the majority race in Malaysia. But surprisingly there were considerable amountsof the dusun/peribumi race (33.3%), which was a race in Sabah, this was in accordance with the origin of SAA patients, in which many of them came from Sabah and Sarawak region (52.8%).…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3][4][5][6] On review that conductedto SAA patients who had undergo allogenic hematopoietic stemcell transplantation in Bone Marrow Transplantation (BMT) ward in Institut Pediatrik Hospital Kuala Lumpur (IPHKL) during the last 10 years, there were 36 patients with SAA with age range between 1 year 8 months to 15 years 7 months, and the mean age was 9.4 years. From 37 patients, 20 (55.6%) were boys and 16 (44.4%) were girls.…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…[1,2,3] The clinical presentation is typically due to symptoms associated with thrombocytopenia, anemia and neutropenia. [1,2,3,4] Aplastic anemia is classified based on the marrow cellularity and the degree of pancytopenia. Diagnostic criteria for severe aplastic anemia include a bone marrow biopsy showing an overall cellularity of less than 25% of the age appropriate for normal cellularity or less than 50% normal cellularity in which fewer than 30% of the cells are hematopoietic.…”

Section: Introductionmentioning

confidence: 99%

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Characteristics and Outcomes of Severe Aplastic Anemia Patients Who Were Treated with Allogenic Hematopoietic Stemcell Transplantation at Institute Pediatric Hospital Kuala Lumpur during 2003-2012

Suryawan¹,

Reniarti²,

Ibrahim³

et al. 2017

AJCMR

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Aplastic anemia is characterized by the development of peripheral blood pancytopenia associated with hypocellularity of the bone marrow. Treatment strategies for aplastic anemia include immunosupressive therapy and hematopoietic stemcell transplantation. The choice of therapy depends on the severity of the disease, patient's age and availability of a human leukocyte antigen (HLA) matched sibling donor. To describe the clinical characteristics and outcomes of all patients diagnosed with severe aplastic anemia who were treated with allogenic hematopoietic stemcell transplantation at IPHKL. All patients with SAA who had underwent matched sibling hematopoietic stemcell transplantation at IPHKL from January 2002-December 2013 were identified. Retrospective data was collected with regards to patient's characteristics, transfusion history, conditioning regime, time of engraftment, length of stay, complication and outcomes of patients. In this study there were 36 patients with SAA who underwent allogenic hematopoietic stemcell transplantation. Of the 36 patients, 20 (55.6%) were boys and 16 (44.4%) were girls. The most regimes used for conditioning was combination of fludarabine, cyclophosphamide and ATG horse in 19 patients (52.8%). Mostly the granulocyte engraftment at 10 days after transplantation, while platelet engraftment occurred at 12 days after transplantation. Complications that occurred after transplant were febrile neutropenia in 32 patients (88.9%), mucositis in 25 patients (69.4%), fungal infection in 4 patients (11.1%) and only 2 patients had GvHD. No patients died after underwent allogenic hematopoietic stemcell transplantation. Thirty four patients (94.4%) were cured after a single hematopoietic stemcell transplantation, and only 2 patients (5.6%) had late graft rejection, but all were cured after having the second transplantation The outcomes of patients with severe aplastic anemia who had underwent allogenic hematopoietic stemcell transplantation in bone marrow transplantation ward IPHKL were good. All patients were cured from the disease.

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Acquired Aplastic Anemia

Cited by 2 publications

References 162 publications

Outcome of children with aplastic anemia treated with immunosuppressive therapy

Outcome of children with aplastic anemia treated with immunosuppressive therapy

Characteristics and Outcomes of Severe Aplastic Anemia Patients Who Were Treated with Allogenic Hematopoietic Stemcell Transplantation at Institute Pediatric Hospital Kuala Lumpur during 2003-2012

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