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The Lancet. Haematology Sep 2022
Topics: Anemia, Aplastic; Bone Marrow Transplantation; Humans
PubMed: 35907409
DOI: 10.1016/S2352-3026(22)00242-3 -
British Journal of Haematology Jul 2002The clinical outcome of childhood aplastic anaemia (AA) with aberrant cytogenetic clones at diagnosis was surveyed. Among 198 children with newly diagnosed AA registered... (Review)
Review
The clinical outcome of childhood aplastic anaemia (AA) with aberrant cytogenetic clones at diagnosis was surveyed. Among 198 children with newly diagnosed AA registered with the AA Committee of the Japanese Society of Paediatric Hematology between 1994 and 1998, cytogenetic studies of bone marrow (BM) cells were completed in 159 patients. Apart from one Robertsonian translocation, seven patients (4.4%) showed clonal chromosomal abnormalities in hypoplastic BM without myelodysplastic features. The patients included six girls and one boy with a median age of 11 years (range 5-14 years). Six patients had del(6), del(5), del(13), del(20), or -7, and one showed add(9). Four patients responded to the first immunosuppressive therapy (IST: cyclosporin A plus anti-thymocyte globulin) and one obtained a spontaneous remission. Cytogenetic abnormalities remained in two patients with an IST response. On the other hand, two patients showed no IST response. One did not respond to repeat IST and died of acute graft-versus-host disease after an unrelated-BM transplant. Another obtained a complete response after a successful BM transplant. No haematological findings at diagnosis predicted the treatment response. No significant morphological changes developed during the course of the illness. A literature review revealed that half of 24 AA patients with chromosomal abnormalities responded to the first IST, and that +6 was the sole predictable marker for IST unresponsiveness. These results suggest that IST can be applied as the initial therapy for AA with cytogenetic abnormalities in the absence of completely matched donors.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Anemia, Aplastic; Bone Marrow Transplantation; Child; Child, Preschool; Chromosome Aberrations; Female; Humans; Immunosuppressive Agents; Japan; Male; Middle Aged; Transplantation, Autologous; Treatment Outcome
PubMed: 12100167
DOI: 10.1046/j.1365-2141.2002.03582.x -
Bone Marrow Transplantation Jun 2010Graft rejection, with persistent pancytopenia, is well documented after allogeneic BMT (hematopoietic SCT (HSCT)) for severe aplastic anemia (SAA) and the prognosis is...
Graft rejection, with persistent pancytopenia, is well documented after allogeneic BMT (hematopoietic SCT (HSCT)) for severe aplastic anemia (SAA) and the prognosis is poor. The recovery of host-hematopoiesis, autologous recovery (AR), after allogeneic HSCT is a rare event and the incidence and long-term survival are unknown. We report a retrospective analysis of consecutive patients in the Aplastic Anaemia Working Party of the European Group for Blood and Marrow Transplantation (EBMT-WPSAA) registry between 1973 and 2005. A total of 45 cases of AR, of 1205 patients transplanted for SAA in 57 centers are reported. We describe characteristics and long-term outcome of patients with AR, compared with SAA patients from participating transplant centers without AR (n=1024) and patients with graft rejection (n=136) without autologous recovery. The estimated cumulative incidence of AR was 4.2% (3.1-5.6) (confidence interval (CI) 95%) with an OS of 84% (95% CI 83-107%). The OS of the control group was 74% (81-90) at 10 years of follow up, whereas the patients with graft failure had an OS of 16% (CI 12-28%). This retrospective analysis establishes the incidence and long-term survival of patients experiencing AR after allogeneic HSCT for SAA.
Topics: Adolescent; Adult; Anemia, Aplastic; Child; Child, Preschool; Female; Graft Rejection; Graft vs Host Disease; Hematopoiesis; Hematopoietic Stem Cell Transplantation; Humans; Infant; Male; Pancytopenia; Registries; Retrospective Studies; Survival Rate; Survivors; Young Adult
PubMed: 19915627
DOI: 10.1038/bmt.2009.296 -
Postepy Higieny I Medycyny... 1992The new aspects in the pathogenesis of aplastic anaemia and others acquired states of bone marrow aplasia as well as congenital defects of the marrow stem cells have... (Review)
Review
The new aspects in the pathogenesis of aplastic anaemia and others acquired states of bone marrow aplasia as well as congenital defects of the marrow stem cells have been reviewed. Role of immune mechanisms, viruses, chemical and irradiation exposure in the etiology and pathophysiology of bone marrow failure is presented. The new achievements in the treatment of aplastic anaemias including bone marrow transplantation, antilymphocyte globulin, cyclosporin A, high doses of 6-methyl prednisolone and recombinant haemopoietic growth factors are also discussed.
Topics: Adult; Agranulocytosis; Anemia, Aplastic; Antilymphocyte Serum; Bone Marrow; Bone Marrow Transplantation; Child; Cyclosporine; Fanconi Anemia; Granulocyte-Macrophage Colony-Stimulating Factor; Hematopoietic Stem Cells; Humans; Methylprednisolone; Thrombocytopenia
PubMed: 1641375
DOI: No ID Found -
JPMA. the Journal of the Pakistan... Jan 2023We aim to document the frequency of HAAA cases among AA patients presenting at a tertiary care hospital, and to determine the most common agents (viral/drug induced) and...
OBJECTIVE
We aim to document the frequency of HAAA cases among AA patients presenting at a tertiary care hospital, and to determine the most common agents (viral/drug induced) and Clinico-haematological features among HAAA patients at a tertiary care hospital.
METHODS
This study was a retrospective review, conducted at a tertiary care hospital in Karachi, Pakistan.
RESULTS
A total of 21 patients were included in the study. Hepatitis among the HAAA patients was viral in 17 cases, while 4 were idiopathic. All the patients acquired aplastic anaemia within 3-12 months of the Hepatitis episode and most presented with bleeding, bruises and petechiae.
CONCLUSIONS
This study indicates and proves that presence and prevalence of this disease in the Pakistani population is quite significant. Unlike the rest of the world, HAAA in Pakistan is not entirely of unknown aetiology, most of the cases can be associated with one of the Hepatitis viruses.
Topics: Humans; Anemia, Aplastic; Pakistan; Tertiary Care Centers; Hepatitis; Hepatitis A; Prevalence
PubMed: 36842054
DOI: 10.47391/JPMA.5115 -
British Journal of Haematology Feb 2024The great pathologist Paul Ehrlich in Berlin is commonly credited with describing the first clear case of aplastic anaemia in 1888: a 21-year-old woman who presented... (Review)
Review
The great pathologist Paul Ehrlich in Berlin is commonly credited with describing the first clear case of aplastic anaemia in 1888: a 21-year-old woman who presented with haemorrhage and signs and symptoms of severe anaemia, quickly succumbing to her illness. Ehrlich's description of this patient's background and clinical course allowed individual identification. Re-analysis of this case suggests an inherited bone marrow failure syndrome as a possible additional diagnosis.
Topics: Female; Humans; Young Adult; Anemia, Aplastic
PubMed: 38044033
DOI: 10.1111/bjh.19241 -
British Journal of Haematology Aug 1973
Topics: Androgens; Anemia, Aplastic; Humans; Time Factors
PubMed: 4726910
DOI: No ID Found -
British Medical Journal May 1953
Topics: Anemia; Anemia, Aplastic; Humans; Toluene
PubMed: 13042166
DOI: 10.1136/bmj.1.4820.1143 -
Blood Reviews May 2005Outcome of patients with aplastic anaemia (AA), whether treated with allogeneic BMT or immunosuppressive therapy has steadily increased over the last three decades.... (Review)
Review
Outcome of patients with aplastic anaemia (AA), whether treated with allogeneic BMT or immunosuppressive therapy has steadily increased over the last three decades. However, there is a difference in quality of outcome between these two therapeutic modalities. There is no plateau for survival after ATG as patients are at later risk of transformation to myelodysplasia (MDS) or acute myeloid leukaemia (AML), paroxysmal nocturnal haemoglobinuria and relapse of their aplasia. In contrast, AA patients are not at risk of these later complications if they have undergone successful bone marrow transplantation. Long term survival after HLA identical sibling BMT is 80-90%, but GVHD and graft rejection remain to be addressed. The results of unrelated donor BMT for AA have shown considerable improvement over the last five years. Difficulties remain for those patients who fail immunosuppressive therapy and in whom BMT is not possible, since alternative immunosuppressive agents have so far proven to be somewhat disappointing.
Topics: Anemia, Aplastic; Combined Modality Therapy; Cyclosporine; Humans; Immunosuppressive Agents
PubMed: 15748962
DOI: 10.1016/j.blre.2004.06.002 -
Acta Medica Scandinavica Oct 1974
Review
Topics: Anemia, Aplastic; Autoantibodies; B-Lymphocytes; Blood Platelets; Cell Division; Chloramphenicol; Chlorpromazine; Erythropoiesis; Granulocytes; Hematopoiesis; Hematopoietic Stem Cells; Hepatitis A; Humans; Immunity; Radiation Effects; T-Lymphocytes; Thymoma
PubMed: 4611144
DOI: 10.1111/j.0954-6820.1974.tb01006.x