Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty male and 27 female patients were treated at the University College Hospital, Ibadan, over a 20 year period (January, 1971 to December, 1990) for aplastic anaemia. Age range was three months to 52 years with a median of 19 years. In 26 patients the aplasia could not be linked with a particular cause. Most patients in this category were students in secondary and tertiary institutions, office workers and casual labourers. Nineteen patients were automobile and factory workers who are exposed to lead while two were dealers in petroleum products. In two patients aplasia was linked to chloramphenicol ingestion while in three, the aplasia linked to the use of hair dye. Another three linked the aplasia to a past history of viral hepatitis. One patient had congenital aplasia of the marrow and one was a radiographer. Treatment included oxymethalone 100 mg given three times daily (tds) or intramuscular (i.m.) Durabolin 500 mg weekly. When these drugs were unavailable prednisolone 60 mg daily was administered. No patient had the benefit of bone marrow transplantation (BMT) or the superior drugs, e.g. anti-thymocyte globulin and cyclosporin A. This might have contributed to the poor prognosis as revealed by the survival pattern in which 24 patients died within six months of diagnosis while 19 survived 12 months. Seven patients died within 18 months and four others died within three years. Three patients were alive five years after presentation. Death generally resulted from complications of the aplastic anaemia with gastro-intestinal bleeding, cerebro-vascular accidents and overwhelming infections.
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PMID:Aplastic anaemia: a review of cases at the University College Hospital, Ibadan, Nigeria. 129 64

Bone marrow transplantation has become an accepted treatment for malignancy (particularly leukemia and lymphoma), aplastic anemia, and certain inborn errors of metabolism. Patients require intensive care because of chemoradiation therapy toxicity, a prolonged period of immunosuppression and thrombocytopenia, graft-versus-host disease (GVHD), and the need for parenteral nutrition. Gastrointestinal and hepatic diseases are frequent post-transplant problems. They present with intractable nausea and vomiting, intestinal bleeding, diarrhea, esophageal complaints, abdominal pain, and hepatobiliary symptoms. Our clinical approach to complex transplant patients depends on the timing of signs and symptoms after marrow grafting and on the likelihood that specific disease processes are present. Each of these major problems is covered in this review.
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PMID:A problem-oriented approach to intestinal and liver disease after marrow transplantation. 304 22

Antithyreoid drugs are known causative agents of agranulocytosis and, in rare cases, aplastic anaemia as well. This is a case report of a female patient with secondary aplastic anaemia developed two years after continual use of thiamazole. She suffered from exhaustion and massive epistaxis. Physical examination revealed pale skin and mucous membranes, skin hematomas (body and legs) and high body temperature--39 degrees C. At admission, her blood film revealed pancytopaenia with 75 g/l hemoglobin concentration, 1.0 x 10(9)/l leukocytes and severe thrombocytopaenia--7.0 x 10(9)/l. Differential count showed 91% of lymphocytes, 1% of monocytes and only 8% of neuthrophils. Bone marrow cytology and pathohistologic findings revealed severe hypocellularity, replaced with fat cells and only 10% of active hematopoietic tissue. In the acute phase of illness, in vitro growth of bone marrow progenitors was completely absent. Treatment was initiated with prednisone and danazol. During that time, she suffered from epistaxis, gastro-intestinal bleeding and herpes infection. Due to therapeutic failure cyclosporine A was added after 21 days. There upon, slow recovery ensued. After two months, she was discharged from hospital with stable blood film findings (HB 83 g/l, WBC 4.6 x 10(9)/l, and PLT 30.0 x 10(9)/l). She was forbidden thiamazole for her life time. After recovery from the acute phase of illness, in vitro haematopoietic precursor cells examination was repeated. The number of CFU-E colonies stimulated with 1 IU EPO was decreased in comparison with the control values. Upon adding 100 micro/l of thiamazole (5 mg/ml concentration e.g. 500 mg per culture), the growth of CFU-E was completely prevented, followed by marked cytotoxicity signs. The treatment including low doses (5 mg/ kg body weight daily) of cyclosporine A administration was continued on outpatient basis. After one year, blood film showed almost normal results with 120 g/l hemoglobin concentration, 4.3x10(9)/l leukocytes and mild thrombocytopaenia 72.0 x 10(9)/l. She was transfusion free.
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PMID:[Aplastic anaemia caused by thiamazole--a case report]. 1549 89

Dyskeratosis congenita (DC) is a rare inherited disease in which the telomere complex cannot be maintained. Shortened telomeres can cause a number of clinical conditions. We herein report a case of unrelated bone marrow transplantation due to aplastic anemia associated with DC. The patient died of uncontrollable refractory intestinal bleeding. Three cases of DC with life-threatening hemorrhaging after transplantation have been reported; however, the bleeding origin could not be determined. Our case is the only patient in which a gastrointestinal bleeding point, jejunal multiple angioectasia, was determined.
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PMID:Fatal Hemorrhagic Gastrointestinal Angioectasia after Bone Marrow Transplantation for Dyskeratosis Congenita. 2790 6