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JPMA. the Journal of the Pakistan... Dec 2001To complete the data on the demographic features of patients diagnosed to have aplastic anemia at a single institution over a 7.5 years period.
OBJECTIVE
To complete the data on the demographic features of patients diagnosed to have aplastic anemia at a single institution over a 7.5 years period.
METHODS
Demographic information was retrieved from the patients medical records retrospectively as well as prospectively of those patients who presented with features of aplastic anaemia. Their diagnosis was confirmed by performing a complete blood count and bone marrow trephine.
RESULTS
One hundred and forty four patients were diagnosed to have aplastic anemia; there were 106 males and 38 females. Their ages ranged from 2 to 75 years, with a median of 17 years, 112 (77.7%) patients were below the age of 30 years. Severe aplastic anemia (SAA) was seen in 74 (51.4%), very severe (VSAA) in 24 (16.7%) and non-severe aplastic anemia (NSAA) in 46 (31.9%) patients. No obvious cause could be established for 74.3%. Thirteen patients admitted using drugs known to cause AA and one was a radiographer (9%). Out of 44 patients tested, 7 (15.9%) were found to have either hepatitis B virus markers or antibody to hepatitis C at the time of diagnosis of AA. However it was difficult to establish a cause and effect relationship with either drugs or viruses.
CONCLUSION
Aplastic anaemia is found to occur mostly in young males. The most common type was idiopathic severe aplastic anaemia.
Topics: Adolescent; Adult; Aged; Anemia, Aplastic; Child; Child, Preschool; Female; Humans; Male; Middle Aged; Pakistan; Prospective Studies; Retrospective Studies
PubMed: 11850982
DOI: No ID Found -
Clinics in Haematology Oct 1978The current published reports have indicated that the young patient with aplastic anaemia who has a compatible marrow donor can obtain a successful marrow graft,... (Review)
Review
The current published reports have indicated that the young patient with aplastic anaemia who has a compatible marrow donor can obtain a successful marrow graft, especially if sex matched and with a past record of little or no transfusion therapy. Despite these encouraging results, only a small number of patients will have such donors available. Immunosuppressive therapy has been considered as an alternative, but this treatment has high risk in older patients. Past studies with androgen therapy have reported a response rate at one year similar to the current recovery rates with bone marrow transplantation. However, contemporary reports have indicated a marked decrease in the recovery rate following androgens, and some of these comparisons may be related to differences in supportive transfusion therapy. In part, the decreased rates may be related to an inadequate evaluation of residual marrow function in the aplastic patient. Patients to be treated with androgens always should have a physiological evaluation of residual erythropoietic committed bone marrow cells. In the absence of such an erythropoietic nidus, one may anticipate a poor response to any steroid therapy. Supportive blood component transfusions should be provided, especially in the initial three months of androgen treatment. In past studies the majority of patients have received only oral androgens, predominantly oxymetholone. Other androgens may be more effective in a particular patient, and there is an urgent need to develop procedures that define stem cell receptors for specific testosterone preparations. Current investigations have indicated that the 5 beta steroid metabolites of testosterone are haematopoietic without the complication of virilization. It is anticipated that a variety of these metabolites can be prepared for evaluation in the patient with aplasia. While there is continuing evaluation of the immune responses and suppressive therapies the clinician should continue to treat the aplastic patient with vigorous supportive transfusion therapy and different androgens for comparative evaluation.
Topics: Acute Disease; Androgens; Anemia, Aplastic; Animals; Child; Guinea Pigs; Hematopoiesis; Humans; Leukemia; Male; Mice; Rabbits; Rats
PubMed: 363329
DOI: No ID Found -
BMJ Open Jul 2014Acquired severe aplastic anaemia is a rare and potentially fatal disease. The aim of this Cochrane review was to evaluate the effectiveness and adverse events of... (Comparative Study)
Comparative Study Review
OBJECTIVES
Acquired severe aplastic anaemia is a rare and potentially fatal disease. The aim of this Cochrane review was to evaluate the effectiveness and adverse events of first-line allogeneic haematopoietic stem cell transplantation of human leucocyte antigen (HLA)-matched sibling donors compared with first-line immunosuppressive therapy.
SETTING
Specialised stem cell transplantations units in primary care hospitals.
PARTICIPANTS
We included 302 participants with newly diagnosed acquired severe aplastic anaemia. The age ranged from early childhood to young adulthood. We excluded studies on participants with secondary aplastic anaemia.
INTERVENTIONS
We included allogeneic haematopoietic stem cell transplantation as the test intervention harvested from any source of matched sibling donor and serving as a first-line therapy. We included immunosuppressive therapy as comparator with either antithymocyte/antilymphocyte globulin or ciclosporin or a combination of the two. PRIMARY AND SECONDARY OUTCOME MEASURES PLANNED AND FINALLY MEASURED: The primary outcome was overall mortality. Secondary outcomes were treatment-related mortality, graft failure, graft-versus-host disease, no response to immunosuppressive therapy, relapse after initial successful treatment, secondary clonal disease or malignancies, health-related quality of life and performance scores.
RESULTS
We identified three prospective non-randomised controlled trials with a study design that was consistent with the principle of 'Mendelian randomisation' in allocating patients to treatment groups. All studies had a high risk of bias due to the study design and were conducted more than 15 years. The pooled HR for overall mortality for the donor group versus the no donor group was 0.95 (95% CI 0.43 to 2.12, p=0.90).
CONCLUSIONS
There are insufficient and biased data that do not allow any firm conclusions to be made about the comparative effectiveness of first-line allogeneic haematopoietic stem cell transplantation of HLA-matched sibling donors and first-line immunosuppressive therapy of patients with acquired severe aplastic anaemia.
Topics: Adolescent; Anemia, Aplastic; Child; Child, Preschool; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppressive Agents; Infant; Severity of Illness Index; Siblings; Tissue Donors; Treatment Outcome; Young Adult
PubMed: 25031191
DOI: 10.1136/bmjopen-2014-005039 -
British Journal of Haematology Jun 2021
Topics: Anemia, Aplastic; Fetal Blood; Humans; Tissue Donors; Transplantation Conditioning
PubMed: 33993468
DOI: 10.1111/bjh.17401 -
Nursing Mirror and Midwives Journal Sep 1966
Topics: Anemia, Aplastic; Nursing
PubMed: 5179744
DOI: No ID Found -
Nursing Times
Topics: Anemia, Aplastic; Anti-Bacterial Agents; Blood Transfusion; Bone Marrow Examination; Causality; Dyspnea; Fatigue; Growth Substances; Headache; Hemorrhage; Humans; Immunosuppressive Agents; Infections
PubMed: 16827042
DOI: No ID Found -
Casopis Lekaru Ceskych Jul 1998Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal disorder of haematopoiesis. Clinically it is characterized by intravascular haemolysis, venous... (Review)
Review
Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal disorder of haematopoiesis. Clinically it is characterized by intravascular haemolysis, venous thrombosis and often by bone marrow hypoplasia. Haemolysis and thrombosis develop as a consequence of deficiency of several proteins on the cell membrane of the affected clone of blood elements. This is caused by somatic mutations in the PIG-A gene, which encodes an enzyme involved in the biosynthesis of glycosylphosphatidylinositol (GPI) anchor. Spectrum of mutations in the PIG-A gene is different to that observed in other genes. The mutations are mainly small deletions and insertions causing frameshift; large deletions are rare. Recently, however, a 88 base pairs direct tandem repeat insertion has been reported in a patient with PNH developed on the background of aplastic anaemia (AA). The peculiar pattern of the PIG-A gene mutations and the finding that more than one mutated clone is commonly present in patients with PNH might suggest that some form of hypermutability, caused by decreased DNA stability, deficient repair or increased generation of mutagens, might underline PNH. As most mutations cause cell death, it would explain the hypoplastic nature of the disorder and its association with AA. Other models of pathogenesis of PNH are also discussed.
Topics: Anemia, Aplastic; Hemoglobinuria, Paroxysmal; Humans; Mutation
PubMed: 9748736
DOI: No ID Found -
BMJ Case Reports Dec 2017Prognosis of hepatitis-associated aplastic anaemia (HAAA) was improved with haematopoietic stem cell transplantation (HSCT) and immunosuppression, but the long-term...
Prognosis of hepatitis-associated aplastic anaemia (HAAA) was improved with haematopoietic stem cell transplantation (HSCT) and immunosuppression, but the long-term outcome remains undefined. Case 1: a girl aged 3 years with acute liver failure (ALF) submitted to orthotopic liver transplantation (OLT) subsequently developed aplastic anaemia and HSCT from a compatible sibling was performed. Post-HSCT, the patient developed post-transplant lymphoproliferative disorder and rituximab was administered with good response. Fifteen years later, both grafts show good outcome. Case 2: a girl aged 10 years submitted to OLT due to ALF, developed pancytopenia 2 months later. Due to the absence of a human leucocyte antigen compatible donor, she was treated with ciclosporin and antithymocyte globulin with very good long-term outcome. These clinical cases suggest that, for patients with HAAA that underwent OLT, aggressive therapy with HSCT or immunosuppression may provide a benign long-term outcome.
Topics: Acyclovir; Anemia, Aplastic; Antiviral Agents; Child; Child, Preschool; Diagnosis, Differential; Female; Follow-Up Studies; Hematopoietic Stem Cell Transplantation; Hepatitis; Humans; Liver Failure, Acute; Liver Transplantation; Postoperative Complications
PubMed: 29237659
DOI: 10.1136/bcr-2017-221434 -
Acta Reumatologica Portuguesa 2009Eosinophilic fasciitis is a rare rheumatic condition characterized by inflammatory thickening of the skin and fascia, peripheral eosinophilia, elevated erythrocyte...
Eosinophilic fasciitis is a rare rheumatic condition characterized by inflammatory thickening of the skin and fascia, peripheral eosinophilia, elevated erythrocyte sedimentation rate and hypergammaglobulinemia. Internal organ involvement is uncommon. It is often difficult to diagnose eosinophilic fasciitis and its course may be variable. Glucocorticoids are most commonly used in the treatment but in many cases they are ineffective, requiring combined immunosuppressive treatment. Several cases of eosinophilic fasciitis and serious haematological disorders such as immune thrombocytopenia, Hodgkin's disease and aplastic anaemia have been described. The authors report an atypical severe case of eosinophilic fasciitis complicated by aplastic anaemia non responsive to treatment.
Topics: Anemia, Aplastic; Eosinophilia; Fasciitis; Humans; Male; Middle Aged
PubMed: 19365308
DOI: No ID Found -
British Journal of Haematology Feb 2019Aplastic anaemia (AA) is infrequently observed in pregnancy. We describe 19 pregnancies in 9 women at a tertiary care centre over a period of 30 years. Spontaneous...
Aplastic anaemia (AA) is infrequently observed in pregnancy. We describe 19 pregnancies in 9 women at a tertiary care centre over a period of 30 years. Spontaneous resolution of AA did not occur postpartum in the five pregnancies where AA was first diagnosed in pregnancy. In the remaining pregnancies, although haematological indices declined and transfusion support was needed in 35% of pregnancies, relapses were not observed. There were no deaths, but complications occurred in 79% of pregnancies. Preterm delivery and postpartum haemorrhage were observed in 21% and 26% of pregnancies, respectively.
Topics: Adult; Anemia, Aplastic; Blood Transfusion; Female; Humans; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Premature Birth; Retrospective Studies
PubMed: 30460693
DOI: 10.1111/bjh.15654