Cases reported "Disease Progression"

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11/16. Fas ligand-mediated lethal hepatitis after rapid lysis of a localized natural killer cell lymphoma.

    Natural killer (NK) cell malignancies have been associated with neutropenia and disturbances of liver function tests, thought to be related to high levels of soluble Fas ligand (FasL) in the circulation. We report a case of fulminant hepatitis occurring 3 weeks after the initiation of salvage therapy by arginine butyrate and ganciclovir for refractory Epstein-Barr virus-positive NK cell lymphoma. Pathologic examination revealed disappearance of the NK tumor and massive liver injury caused by apoptosis of virtually all hepatocytes. immunohistochemistry revealed an intense staining for FasL. To our knowledge, this is the first description of the occurrence of FasL-mediated lethal hepatitis after lysis of a NK cell lymphoma.
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keywords = apoptosis
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12/16. Novel KIND1 gene mutation in Kindler syndrome with severe gastrointestinal tract involvement.

    BACKGROUND: Kindler syndrome (online Mendelian Inheritance in Man No. 173650) is an autosomal recessive genodermatosis characterized by acral trauma-induced blistering that improves with age and by progressive poikiloderma in later life. Other clinical features include photosensitivity, webbing of the fingers and toes, nail dystrophy, periodontal disease, and mucosal alterations. Aside from esophageal or anal stenosis, gastrointestinal tract involvement seems to be rare in Kindler syndrome. Recently, mutations in the KIND1 gene that encodes for the membrane-associated protein kindlin-1 have been identified. Kindlin-1 links the actin cytoskeleton to the extracellular matrix and is supposed to have cell-signaling functions owing to different functional domains. In particular, a domain with high homology to 4.1/ezrin/radixin/moesin (FERM) proteins is closely related to the sequences of talin that mediate integrin binding and therefore may play a role in integrin-dependent processes such as cell growth, differentiation, and apoptosis. observation: Complete loss of this multifunctional protein in our patient with Kindler syndrome resulted in severe gastrointestinal tract involvement with hemorrhagic colitis. Mucosa of the descending and sigmoid colon and the rectum showed erosions and ulcers with pseudomembranous alterations of an overall highly vulnerable mucosa. mutation analysis revealed a homozygous status for the novel mutation 20/21delTT in exon 2 of the KIND1 gene resulting in a preterminal stop codon creating a nonfunctional peptide 17 amino acids in length. CONCLUSION: Because of our experience with this and another patient, we propose that gastrointestinal tract involvement should be looked at more frequently in Kindler syndrome.
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keywords = apoptosis
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13/16. Histopathologic abnormalities of the lymphoreticular tissues in organic cation transporter 2 deficiency: evidence for impaired B cell maturation.

    Immunohistology of lymphoreticular tissues of a fatal case of organic cation transporter 2 deficiency revealed inhibited proliferation with increased apoptosis in the germinal centers, resulting in "burned out" follicles. This is indicative of impaired antigen driven B cell affinity maturation. Defective humoral immune response might explain the recurrent infections in untreated organic cation transporter 2 deficiency.
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keywords = apoptosis
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14/16. apoptosis as a possible cause of gradual development of complete heart block and fatal arrhythmias associated with absence of the AV node, sinus node, and internodal pathways.

    BACKGROUND: Gradually progressive development of complete heart block in young people often is associated with cardiac arrhythmia and sudden death, but the pathogenesis remains unexplained. methods AND RESULTS: A young woman with complete heart block died suddenly. Her mother had serological but no clinical evidence of antiphospholipid syndrome. Five brothers of another family had arrhythmia and heart block. Three died suddenly; the other two have automatic defibrillators and are alive. The hearts from the young woman and two of the three brothers who died were available for our histological examination of their cardiac conduction systems. In two of the three hearts, the AV node was absent; in the third heart, only fragments of the AV node remained. In all three hearts, the sinus node was nearly destroyed by a noninflammatory degeneration with no abnormal fibrosis or infiltrate. In each heart, the interatrial and internodal pathways were similarly involved, and in the young woman, there were no myocardial cells in which these pathways normally exist. CONCLUSIONS: In these three subjects with progressive development of complete heart block and various arrhythmias, all of whom died suddenly, the histological abnormalities of their cardiac conduction systems are best interpreted as resulting from apoptosis. Programmed cell death is a logical explanation for the pathogenesis of this puzzling clinical picture.
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keywords = apoptosis
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15/16. Functional adrenocortical adenoma in a case with B cell chronic lymphoproliferative disorder.

    We describe a B cell chronic lymphocytic leukemia/lymphoma (B-CLL) patient with an adrenocortical adenoma. He was treated initially with oral cyclophosphamide and prednisolone for 2 months, followed by low-dose prednisolone for an additional 2 years, which resulted in the prompt disappearance of CLL cells. After a period of 2 years without therapy, a functional adrenocortical adenoma was found. After adrenalectomy, the CLL cells rapidly increased. Progression of the disease and clinical-hematological relapse was well controlled by low doses of steroids alone, and the patient's condition stabilized. Considering the recent data that corticosteroids can induce apoptosis of CLL cells, the above clinical course suggests that augmented glucocorticoid production from the tumor may have suppressed disease progression.
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keywords = apoptosis
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16/16. Prolonged treatment with biologic agents for malignant glioma: a case study with high dose tamoxifen.

    Traditional study design for treatment of malignant gliomas does not allow tumor progression to be greater than 25-50 percent without terminating treatment. This design may prevent recognition of patients who benefit from the treatment either by slowed growth or delayed response. A delayed response or slowed growth may be characteristic of biologic agents being evaluated in the treatment of malignant glioma. Because of the low toxicity of certain biologic drugs, continued treatment through tumor growth can be ethically considered in study design. The effect of biologic agents on a neoplasm may include cellular differentiation, retardation of growth, cytostasis, cytocidal effects, or apoptosis. Such effects may clinically translate into a complete response, partial response, stable disease or retardation of growth with or without an eventual reduction of tumor. We present a patient with a recurrent malignant glioma who was continued on high dose tamoxifen despite radiologic documented doubling of the tumor size and who eventually showed a delayed response to this agent nine months after initiation of treatment. Strong consideration should be given to the prolonged treatment of non-toxic biologic agents in a controlled clinical trial, where agents have shown some benefit in phase one studies.
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keywords = apoptosis
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