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Bone Marrow Transplantation Feb 2013Currently at least 75% of patients with severe aplastic anaemia can be successfully transplanted using a matched unrelated donor (UD) haematopoietic SCT (HSCT). For... (Review)
Review
Unrelated donor search and unrelated donor transplantation in the adult aplastic anaemia patient aged 18-40 years without an HLA-identical sibling and failing immunosuppression.
Currently at least 75% of patients with severe aplastic anaemia can be successfully transplanted using a matched unrelated donor (UD) haematopoietic SCT (HSCT). For children, outcomes are similar to matched sibling donor (MSD) HSCT. This improvement in outcome over time is likely due to improved HLA tissue typing to identify better matched donors, improvements in the conditioning regimen, particularly fludarabine-based regimens, and improved supportive care. Graft rejection occurs in ∼15% of adults, but is less frequent in children. Chronic GVHD remains a concern but may be reduced by using Alemtuzumab instead of ATG. UD HSCT should be considered early after failure to respond to one course of immunosuppressive therapy, but for children who lack a MSD up front matched UD HSCT may be considered.
Topics: Adolescent; Adult; Anemia, Aplastic; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppression Therapy; Immunosuppressive Agents; Tissue Donors; Transplantation, Homologous; Unrelated Donors; Young Adult
PubMed: 23178545
DOI: 10.1038/bmt.2012.233 -
Clinics in Haematology Oct 1978
Review
Topics: Anemia, Aplastic; Bone Marrow; Humans; Iron Radioisotopes; Prognosis
PubMed: 363322
DOI: No ID Found -
British Journal of Haematology Jan 2018
Topics: Adult; Anemia, Aplastic; Combined Modality Therapy; Disease Management; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppressive Agents; Tissue Donors; Treatment Outcome; United Kingdom
PubMed: 29285764
DOI: 10.1111/bjh.15066 -
BMJ Case Reports Feb 2013A 13-year-old boy presented with spontaneous skin and mucosal bleeds 3 weeks after acute hepatitis of unknown aetiology. Laboratory analyses revealed pancytopenia and...
A 13-year-old boy presented with spontaneous skin and mucosal bleeds 3 weeks after acute hepatitis of unknown aetiology. Laboratory analyses revealed pancytopenia and bone marrow biopsy that confirmed the diagnosis of aplastic anaemia. Other causes of congenital and acquired aplastic anaemia were excluded. He was diagnosed with hepatitis-associated aplastic anaemia. He developed a critical clinical condition, becoming totally dependent on erythrocyte and platelet transfusions, and severe neutropenia, which led to invasive bacterial infection. He died due to sepsis with multiple organ failure 3 months after admission.
Topics: Adolescent; Anemia, Aplastic; Erythrocyte Transfusion; Fatal Outcome; Hepatitis; Humans; Male; Platelet Transfusion; Prognosis
PubMed: 23413287
DOI: 10.1136/bcr-2012-007968 -
The Indian Journal of Medical Research Mar 2013Aplastic anaemia is a rare haematological disorder characterized by pancytopenia with a hypocellular bone marrow. It may be inherited/genetic or acquired. Acquired...
BACKGROUND & OBJECTIVES
Aplastic anaemia is a rare haematological disorder characterized by pancytopenia with a hypocellular bone marrow. It may be inherited/genetic or acquired. Acquired aplastic anaemia has been linked to many drugs, chemicals and viruses. Cytogenetic abnormalities have been reported infrequently with acquired aplastic anaemia. Majority of the studies are in adult patients from the West. We report here cytogenetic studies on paediatric patients with acquired aplastic anaemia seen in a tertiary care hospital in north India.
METHODS
Patients (n=71, age 4-14 yr) were diagnosed according to the guidelines of International Agranulocytosis and Aplastic Anaemia Study. Conventional cytogenetics with Giemsa Trypsin Giemsa (GTG) banding was performed. Karyotyping was done according to the International System for Human Cytogenetics Nomenclature (ISCN).
RESULTS
Of the 71 patients, 42 had successful karyotyping where median age was 9 yr; of these 42, 27 (64.3%) patients had severe, nine (21.4%) had very severe and six (14.3%) had non severe aplastic anaemia. Five patients had karyotypic abnormalities with trisomy 12 (1), trisomy 8 (1) and monosomy 7 (1). Two patients had non numerical abnormalities with del 7 q - and t (5:12) in one each. Twenty nine patients had uninformative results. There was no difference in the clinical and haematological profile of patients with normal versus abnormal cytogenetics although the number of patients was small in the two groups.
INTERPRETATION & CONCLUSIONS
Five (11.9%) patients with acquired aplastic anaemia had chromosomal abnormalities. Trisomy was found to be the commonest abnormality. Cytogenetic abnormalities may be significant in acquired aplastic anaemia although further studies on a large sample are required to confirm the findings.
Topics: Adolescent; Anemia, Aplastic; Child; Child, Preschool; Chromosome Aberrations; Cytogenetics; Female; Humans; India; Karyotyping; Male; Pancytopenia
PubMed: 23640556
DOI: No ID Found -
JPMA. the Journal of the Pakistan... Jan 2021Hepatitis-associated aplastic anaemia (HAAA) is an uncommon variant of aplastic anaemia which may present as acute or chronic, mild and transient or fulminant disease....
Hepatitis-associated aplastic anaemia (HAAA) is an uncommon variant of aplastic anaemia which may present as acute or chronic, mild and transient or fulminant disease. The development of aplastic anaemia is usually fatal if not treated in time, with mortality rate being as high as 85%. A high index of clinical suspicion is required for the diagnosis and exclusion of acquired forms of aplastic anaemia. Here we present a case of a 28-year-old male who presented with sero-negative hepatitis and rapidly progressive bone marrow failure who was given a trial of Granulocyte Colony Stimulating Factor followed by a successful allogenic bone marrow transplant.
Topics: Adult; Anemia, Aplastic; Hepatitis; Hepatitis A; Humans; Male; Pakistan; Tertiary Healthcare
PubMed: 35157686
DOI: 10.47391/JPMA.1340 -
Lancet (London, England) Feb 1988
Topics: Anemia, Aplastic; Erythrocyte Count; Humans; Reticulocytes
PubMed: 2893118
DOI: 10.1016/s0140-6736(88)90388-1 -
Lancet (London, England) Jun 1998
Review
Topics: Anemia, Aplastic; Antilymphocyte Serum; Bone Marrow Transplantation; Cyclosporins; Humans; Immunosuppressive Agents; Patient Selection
PubMed: 9652663
DOI: 10.1016/s0140-6736(05)78801-2 -
BMJ Case Reports Mar 2022Hepatitis-associated aplastic anaemia (HAAA) is a rare condition characterised by onset of acute hepatitis which is followed by development of severe pancytopenia due to...
Hepatitis-associated aplastic anaemia (HAAA) is a rare condition characterised by onset of acute hepatitis which is followed by development of severe pancytopenia due to bone marrow failure within 6 months. This syndrome can be precipitated by acute viral infections, but the aetiology remains unknown in the majority. Drug-induced HAAA is extremely rare and has been reported with nutritional and dietary supplements in current literature. We report the first cases of ayurvedic herbal and homeopathic remedies-associated HAAA in two patients which proved fatal in both. Evaluation of patients with acute hepatitis and severe pancytopenia must include a detailed evaluation for complementary and alternative medicine use.
Topics: Anemia, Aplastic; Chemical and Drug Induced Liver Injury; Gymnema sylvestre; Hepatitis; Humans; Materia Medica
PubMed: 35318201
DOI: 10.1136/bcr-2021-247867 -
British Journal of Haematology Nov 2017
Topics: Anemia, Aplastic; Animals; Biomarkers; Bone Marrow; Cell Culture Techniques; Cell Differentiation; Hematopoietic Stem Cells; Humans; Immunohistochemistry; Mesenchymal Stem Cells; Mice; Phenotype; Stem Cell Niche
PubMed: 27480905
DOI: 10.1111/bjh.14234