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)

A case of multiple small well-differentiated hepatomas with early peliosis hepatis, multiple pancreatic islet cell tumors, and a renal medullary interstitial cell tumor following five years of androgen and prednisone therapy for idiopathic aplastic anemia is reported in a patient who died shortly after allogeneic bone marrow transplantation. The hepatic tumors were well differentiated, and the pancreatic tumors were of mixed ribbon and islet cell pattern. Therapeutic and experimental implications and the relevant literature are briefly summarized.
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PMID:Multiple tumors after androgen therapy. 19 36

Two patients with hematologic disease, one of whom had received androgenic steroids, had liver damage associated with peliosis hepatis. In one patient with spherocytic hemolytic anemia, peliosis hepatis was an incidental postmortem finding. In the other patient, who was treated with androgenic-anabolic steroids for aplastic anemia, hepatic failure associated with peliosis hepatis developed. Splenic involvement by peliosis was present in both patients. Peliosis hepatis should be considered in the differential diagnosis of hepatic disease in patients with hematologic disorders, especially if treatment has included androgenic-anabolic steroids.
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PMID:Peliosis hepatis in hematologic disease. Report of two cases. 68 89

The clinical and pathological findings of peliosis hepatis are reported, complicating a long-term treatment with an anabolic steroid in a 19 year old male patient suffering from chronic aplastic anemia. Following a 15 months course of oxymetholone (Anapolon) a cholestatic jaundice developed without regression despite discontinuing the drug. 4 months later the patient expired as a consequence of his hematologic disease. At autopsy the liver showed a parenchymal type of peliosis hepatis with multiple blood filled cavities and focal liver cell necroses. The pathogenesis of this rare condition is discussed by means of literature demonstrating the hepatotoxic effect of C17-alkylated androgens.
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PMID:[Peliosis hepatis, complicating treatment with anabolic steroids (author's transl)]. 81 Jun 50

A retrospective study of acquired aplastic anemia in 100 Thai children treated with testosterone and prednisolone during 1969 to 1987 is reported. The age ranged from 3-14.5 years (mean 10.3 years). The male to female ratio was 2.3:1. The duration of follow up ranged from 1-17 year (mean 5 years). Prior exposure to possible etiologic agents was found in 36% : antipyretics, Ya-chood, insecticides, benzene, chloramphenicol and paint. The presenting symptoms were bleeding and anemia 72%, fever with either bleeding or anemia 28%. The common sites of bleeding were purpura, epistaxis, gum and teeth, and the gastrointestinal tract. All patients received appropriate supportive treatment and testosterone combined with prednisolone. The results of treatment were evaluated in 80 cases who were followed up until death or at least one year after the diagnosis. Two-thirds of the patients presented with laboratory findings similar to severe aplastic anemia; the fatality rate was 50% (40/80). Most of them succumbed to infection or bleeding in the first 6 months after diagnosis. The recovery rate was 50% with complete, partial and initial responses in 28%, 17% and 5% respectively. The late malignancy rate was 2%. For severe aplastic anemia or for patients who have poor prognostic factors, early approaches of bone marrow transplantation or antilymphocyte globulin administration should be considered.
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PMID:Acquired aplastic anemia in children: a review of 100 patients. 223

Immunoadsorption therapy employing protein A columns (PROSORBA columns) was used for the treatment of patients with naturally occurring or transfusion-induced immune thrombocytopenia purpura (ITP). Plasma from one unit of blood was perfused through the columns and returned to each patient. This procedure was performed once or twice weekly. In two cases of acute ITP, platelets markedly increased, and platelet-associated IgG (Pa-IgG) and circulating immune complexes (CIC) were decreased following the treatments. A transient increase in platelets was achieved in one patient with chronic ITP. Some improved response to platelet transfusions was noticed in one patient with aplastic anemia and platelet alloimmunization. Two mechanisms are suggested for the effect of protein A column therapy: one is the stimulation of the production of anti-idiotype antibody that neutralizes platelet auto-antibody and the other is activation of complement that induces solubilization and removal of CIC containing platelet autoantibodies. Immunoadsorption by protein A column is a useful therapy for some ITP cases, especially those that are acute.
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PMID:The effect of immunoadsorption therapy by a protein A column on patients with thrombocytopenia. 265 94

A case of purpura fulminans produced by Aeromonas hydrophila in a twelve year-old Thai boy was reported. The patient also had aplastic anemia which facilitated the severity of disease. He died despite appropriate antimicrobial therapy.
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PMID:Purpura fulminans produced by Aeromonas hydrophila: a case report. 383 94

166 aplastic anaemia patients involved in a cooperative study including androgens were followed up for more than 2 years from the beginning of treatment. The mortality rate from aplasia was low (20% of all cases) after 2 years and nil beyond 5 years. The patients who died were either those who failed to improve but survived 2 years or those who late untreated relapse. Five deaths were due to acute leukaemia, but in 3 of these the diagnosis of aplastic anaemia was retrospectively doubtful. Long-term adverse reactions were rare: 2 cases each of benign hepatoma and peliosis; jaundice was frequent, but no cirrhosis was observed. Survivors who had been treated before puberty reached normal weight and height. Six normal pregnancies occurred. Paroxysmal nocturnal haemoglobinuria was noted in 8 patients with uncontrolled disease. After androgen therapy was discontinued 50% of the patients relapsed with equally or less severe symptoms. Relapses were more frequent when androgens were abruptly withdrawn or in cases with incomplete initial improvement. Most relapses responded to androgen treatment. More than 25% of the patients are still androgen-dependent. With or without maintenance androgen therapy, long-term improvement is incomplete in most cases, which suggests residual bone marrow deficiency, as seen in rodents with experimentally induced aplastic anaemia.
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PMID:[Long-term survival of patients with bone marrow aplasia treated with androgen therapy. 166 cases]. 622 31

The livers were collected from 47 autopsy cases of aplastic anemia aiming to study the correlation between the use of androgenic anabolic steroid and the formation of peliosis hepatis. Association of peliosis hepatis was found in 7 cases out of 19 cases with anabolic steroid therapy, while peliotic cavities were found only in one case out of the remaining 28 cases without anabolic steroid therapy. Out of 7 cases specified above, peliotic cavities in 5 cases were phlebectatic; phlebectatic and parenchymal types of peliotic cavities coexisted in one case, and one case was parenchymatous. Besides these cavity formations, remarkable dilatation of sinusoids often distinct at the periphery of the hepatic lobule was found in the 7 cases.
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PMID:High incidence of peliosis hepatis in autopsy cases of aplastic anemia with special reference to anabolic steroid therapy. 650 88

Morphologic and chromium release studies demonstrated degeneration in monolayer cultures of endothelial cells exposed to the anabolic steroidal hormone oxymetholone. The concentrations approximated those occurring transiently in the hepatic sinusoidal blood after oral administration of the drug during treatment for aplastic anemia. It is suggested that damage to sinusoidal endothelium, with impairment of its ability to maintain sinusoidal collagenous reticulin, is a mechanism by which oxymetholone can cause peliosis hepatis in these patients.
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PMID:Cytotoxicity of oxymetholone to endothelial cells in vitro. 689 31

Two cases of peliosis hepatis, treated with a large dose of predonine for aplastic anemia and multiple myeloma, respectively, were reported. Case 1 showed no abnormal liver function, but, in Case 2 hepatomegaly, retention of ICG, and elevation of LDH level despite the normal serum transaminase were noted. In both cases, peliotic lesions were incidentally observed at autopsy. In Case 1, the lesion was localized in the right lobe only, but, in Case 2 the lesion was present throughout the entire lobes of the liver involving also a part of the spleen and bone marrow of the sternum. Innumerable blood-filled cavities were surrounded by incomplete reticulin or injured hepatocytes. In smaller lesions observed in Case 1, focal necrosis, hemorrhage, inflammation, and thrombi were prominent features. On the other hand, in extensive lesions, as in Case 2, a marked liver cell dissociation with sequent irregular reticulin arrangement was present. Though the etiology and peliosis hepatis is still uncertain in these cases, it was assumed that a large dose of predonine might have some correlation to the occurrence.
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PMID:Peliosis hepatis. Report of two autopsy cases with a review of literature. 713 99


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