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)

Clinicopathologic features of 45 patients with fulminant hepatic failure due to massive or submassive hepatic necrosis were studied. Both percutaneous biopsies and liver explants were available in 23 patients, whole livers only in 11 cases, and biopsies only in 11 cases. An etiologic diagnosis was established in 16 cases (36%). A further 3 cases (7%) were associated with aplastic anemia. Established etiologies included drug reactions (n = 7); autoimmune hepatitis, type 2 (n = 3); halothane hepatitis (n = 1); ischemia/hypotension (n = 1); mushroom poisoning (n = 1); mitochondrial disorder (n = 1); hemophagocytic lymphohistiocytosis (n = 1); and adenoviral hepatitis (n = 1). The extent of necrosis on liver biopsy correlated poorly with that in liver explants (mean difference, 32% +/- 23.8%). Almost all cases could be classified into one of 2 broad patterns of necrosis, namely, (1) zonal coagulative necrosis or (2) panlobular (nonzonal) necrosis. These patterns differed significantly with respect to several clinical parameters including sex ratio, peripheral blood white cell count, serum aspartate transaminase and alanine transaminase, conjugated bilirubin, and alkaline phosphatase levels. Livers with panlobular necrosis showed a spectrum of histopathologic findings that included central venulitis (76%), lymphocytic infiltration of large duct/gallbladder epithelium (54%), and syncytial giant cell transformation (18%). These features were not seen in livers with zonal coagulative necrosis which frequently showed prominent steatosis (91%). Both patterns of necrosis frequently showed ductular proliferation (100%) and cholangiolitis (80%). The diagnostic yield of ancillary studies (histochemistry, immunohistochemistry, and electron microscopy) was very low (<1%). The small proportion of cases with etiologic diagnoses precluded correlation of clinical and histopathological parameters with specific etiologies. In summary, this study describes the spectrum of changes seen in massive and submassive necrosis in children and identifies clinical features that might differentiate between 2 broad patterns of necrosis.
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PMID:Clinicopathologic spectrum of massive and submassive hepatic necrosis in infants and children. 1912 48

Three patients with aplastic anemia were evaluated by the ophthalmology service within 2 months of the aplastic anemia diagnosis for bilateral visual loss. The mean age of diagnosis of aplastic anemia was 14.3 years (range: 5 to 19 years) and the mean follow-up was 25 months (range: 15 to 44 months). All 6 eyes demonstrated choroidal ischemia and vitreous hemorrhage. Pars plana vitrectomy was performed in four eyes of two patients for non-clearing vitreous hemorrhage; one patient was observed. Successful anatomic outcomes were achieved in 3 of 4 eyes that underwent vitrectomy. Initial visual acuity ranged from 20/80 to bare light perception and final visual acuity ranged from 20/20 to no light perception. All patients received immunosuppressive therapy including cyclosporine and anti-thymocyte globulin, and two underwent hematopoietic stem cell transplantation. All patients received perioperative platelet and blood transfusions. Pars plana vitrectomy resulted in functional and anatomic success in the majority of eyes in this series. Coordination of medical and surgical care with the hematology service is advisable to stabilize hematologic parameters prior to undertaking a vitreoretinal procedure.
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PMID:Posterior segment ophthalmic complications of aplastic anemia. 2080 44

In this case report of a patient with angioimmunoblastic T-cell lymphoma (AITL), we describe the occurrence of three sequential complications that have been reported uncommonly in this disease subtype. Firstly, the patient developed hypercalcemia due to elevated 1,25-didydroxyvitamin D. Although hypercalcemia in AITL is not rare (1-2% incidence), this case was unusual in that the complication developed when disease appeared stable and symptomatically, he was doing well otherwise. Hypercalcemia surprisingly resolved a few months later at a time when his disease appeared to be progressing. A year later, the patient presented with digital ischemia necessitating partial amputation of a finger. Pathological exam revealed granulomatous vasculitis of small and medium arterioles with infiltrating malignant T lymphocytes. Although skin manifestations are common in AITL, necrotizing granulomatous vasculitis with accompanying tumor cells leading to severe digital ischemia appears rare. Subsequently the patient developed profound pancytopenia with bone marrow confirming severe aplastic anemia. To our knowledge only one other case of aplastic anemia has been reported in a patient with AITL. We discuss the diagnostic and management considerations involved in this patient care and review similar reported cases.
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PMID:Sequential Complications of Hypercalcemia, Necrotizing Granulomatous Vasculitis, and Aplastic Anemia Occurring in One Patient with Angioimmunoblastic T-cell Lymphoma. 3309 93