1/21. Fibrosing cholestatic hepatitis: a report of three cases.Fibrosing cholestatic hepatitis is an aggressive and usually fatal form of viral hepatitis in immunosuppressed patients. We report three cases of fibrosing cholestatic hepatitis in various clinical situations. Case 1 was a 50-year-old man who underwent a liver transplant for hepatitis b virus (HBV)-associated liver cirrhosis. Two and a half years after the transplant, he complained of fever and jaundice, and liver enzymes were slightly elevated. serum HBsAg was positive. Case 2 was a 30-year-old man in an immunosuppressed state after chemotherapy for acute lymphoblastic leukemia. He was a HBV carrier. Liver enzymes and total bilirubin were markedly elevated. Case 3 was a 50-year-old man who underwent renal transplantation as a known HBV carrier. One year after the transplant, jaundice developed abruptly, but liver enzymes were not significantly elevated. Microscopically lobules were markedly disarrayed, showing ballooning degeneration of hepatocytes, prominent pericellular fibrosis, and marked canalicular or intracytoplasmic cholestasis. Portal inflammation was mild, but interphase activity was definite and cholangiolar proliferation was prominent. hepatocytes were diffusely positive for HBsAg and HBcAg in various patterns. patients died of liver failure within 1 to 3 months after liver biopsy in spite of anti-viral treatment.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/21. Crux medicorum ulcerated radiation-induced fibrosis - successful therapy with pentoxifylline and vitamin e.Case report of a 60 year-old patient with an ulcerated radiation-induced fibrosis after therapy for breast cancer. Treatment with oral administration of pentoxifylline 3 x 400 mg/day and vitamin e 2 x 200 mg/day was started. The ulcers were almost completely healed after 18 months. Sonographic examination showed a reduction in dermal thickness and in the laser Doppler fluxmetry, a regulation in the amplitude and increase in flux was found compared to the measurements made before the start of treatment. The therapy was very well tolerated without any side effects. The treatment of radiation-induced fibrosis with PTX and vitamin e is a practicable and cost-effective regimen, especially for inoperable patients. The efficacy of this treatment is probably due to a combination of blood flow stimulation and immune modulation which lead to a reduction in the fibrosis.- - - - - - - - - - ranking = 2809.3670244223keywords = radiation-induced (Clic here for more details about this article) |
3/21. interferon-gamma in 5 patients with cutaneous radiation syndrome after radiation therapy.BACKGROUND: Irradiation can cause acute inflammatory responses as well as chronic fibrotic alterations of the skin. Cutaneous radiation fibrosis evokes a complex of therapeutic problems. However, therapeutic options, apart from surgical approaches, are limited. patients AND methods: Five female patients suffering from severe cutaneous fibrosis were treated with interferon-gamma on a low-dose regimen, 3 x 100 microg/week subcutaneously for 6 months, then once per week for another 6 months. In 4 patients, skin thickness was measured with high-frequency (20 MHz) ultrasound in a clinically well-defined target skin lesion. In 1 patient, nuclear magnetic resonance imaging was performed to quantify the extent of cutaneous radiation fibrosis and to monitor the therapeutic outcome. RESULTS: All patients suffered from radiation-induced cutaneous fibrosis. Additionally, in 1 patient, a fistula, as assessed by lymph vessel scintigraphy, and in another patient a radiation ulcer was diagnosed. In all patients, reduction of radiation-induced fibrosis could be documented. Both fistula and radiation ulcer regressed completely under interferon-gamma therapy. CONCLUSION: Low-dose interferon-gamma therapy is a new and effective treatment modality for cutaneous radiation fibrosis caused by radiation therapy. The positive impact of interferon-gamma on our patients warrants randomized double-blind trials on therapy of radiation fibrosis.- - - - - - - - - - ranking = 936.45567480742keywords = radiation-induced (Clic here for more details about this article) |
4/21. Four cases of hepatitis b virus-related fibrosing cholestatic hepatitis treated with lamivudine.Fibrosing cholestatic hepatitis (FCH) is a rare and extremely severe form of hepatitis b virus (HBV) infection. This condition was originally described in HBV-infected recipients after a liver transplantation. Recently, FCH has been reported not only in liver transplant recipients, but also in other immunosuppressed patients. It is characterized clinically by cholestatic hepatic dysfunction, and pathologically by severe periportal fibrosis, cholestasis, widespread balloon degeneration of hepatocytes, and only a mild infiltration of inflammatory cells. Without treatment, FCH is universally fatal within a few months of diagnosis. There have been only two isolated case reports of FCH with long-term patient survival, and one case report with treatment failure after lamivudine therapy. Because of the rarity of this clinical entity, the therapeutic efficacy of lamivudine in patients with FCH cannot be evaluated systematically. Here, we present four patients with HBV-related FCH treated with lamivudine. One received antineoplastic therapy for acute lymphoblastic leukemia, and the other three were renal graft recipients. Two patients who developed FCH after a renal transplantation survived with an improvement in liver function and were followed up for 20 and 30 months, respectively, and were found to be in good health. However, the other two patients died of sepsis, possibly as a consequence of the immunosuppression with hepatic failure despite lamivudine treatment. Our experience suggests that lamivudine can alter the grave natural history of FCH.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
5/21. Long-term expression of fibrogenic cytokines in radiation-induced damage to the internal anal sphincter.BACKGROUND: There is accumulating evidence, both quantitative and qualitative, that pelvic irradiation affects anorectal function. However, the molecular mechanisms responsible for radiation-induced damage to the anal sphincter remain unclear. AIM: To determine the expression of transforming growth factor-beta 1 (TGF-beta 1) and its downstream effector connective tissue growth factor (CTGF) in the anal sphincter of a patient irradiated for prostate cancer. PATIENT: A 82 year-old patient developed a rectal adenocarcinoma and underwent an abdomino-perineal resection (APR), four years after receiving pelvic irradiation for prostate carcinoma. methods: Tissue sections of the anal sphincter were processed for histology. Immunostaining for TGF-beta 1 and CTGF were performed. RESULTS: CTGF and TGF-beta 1 immunoreactivity was detected in the irradiated anal sphincter, and was absent in controls. Immunoreactivity for both cytokines predominated in the internal sphincter. CTGF and TGF-beta 1 were preferentially detected in endothelial cells, myofibroblasts and fibroblasts; in addition, there was strong immunoreactivity for TGF-beta 1, but not for CTGF in smooth muscle cells of the anal canal. CONCLUSION: Four years after pelvic irradiation, radiation-induced damage appeared to affect predominantly the smooth muscle layer of the anal canal. The molecular mechanisms responsible for radiation-induced fibrosis to these tissues involve prolonged activation of TGF-beta 1 and its downstream effector CTGF.- - - - - - - - - - ranking = 3277.594861826keywords = radiation-induced (Clic here for more details about this article) |
6/21. Marrow fibrosis associated with a philadelphia chromosome.Three patients had marked marrow fibrosis and an apparent Philadelphia (Ph) chromosome. Hematologic, cytogenetic, and molecular studies demonstrated the heterogeneity of such cases, including the first example of clinically typical myelofibrosis (MF) associated with a bcr gene rearrangement characteristic of chronic myelogenous leukemia (CML).- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
7/21. Progression of chronic myeloid leukemia to blast crisis during treatment with imatinib mesylate.CONTEXT: Previous investigators have reported discrepancies between hematologic, marrow morphologic, and cytogenetic responses to imatinib mesylate among patients with chronic myeloid leukemia (CML). In addition to disease refractoriness, rare instances of disease progression from chronic phase to blast crisis during imatinib therapy have recently been anecdotally reported. OBJECTIVES: To describe the clinicopathologic features of 3 patients with CML who rapidly progressed from chronic phase to blast crisis while taking imatinib and to perform a review of the literature. DESIGN: Morphologic, immunophenotypic, and cytogenetic analyses were performed on the 3 patients at the time of initial diagnosis, during imatinib therapy, and at blast crisis. RESULTS: The 3 patients were men, aged 39, 42, and 43 years. Two had been treated with hydroxyurea for 16 and 21 months before imatinib therapy, while 1 was started on a regimen of imatinib following diagnosis. Despite a hematologic response in all 3 patients, none of them achieved cytogenetic remission, and all progressed to blast crisis at 7 to 10 months of imatinib therapy. blood findings during blast transformation were heterogeneous, including normal blood morphologic findings in 1 patient, leukocytosis with circulating blasts and basophilia in 1, and marked pancytopenia in 1. All 3 marrow specimens demonstrated moderate to marked diffuse reticulin fibrosis with more than 20% blasts. Clonal cytogenetic evolution was evident in 2 of the 3 patients and included an extra philadelphia chromosome in both. All 3 patients underwent allogeneic bone marrow transplantation. One was alive with no evidence of disease at 14 month follow-up, while 2 had residual disease after bone marrow transplantation and died of complications at 4 and 5 months after transplantation. CONCLUSIONS: blood data did not always reflect marrow status. Therefore, bone marrow follow-up is critical for monitoring of response. Our findings suggest that significant progression of marrow reticulin fibrosis during imatinib therapy can be an indicator for a return or progression of CML and, in some patients with CML, imatinib may promote cytogenetic clonal evolution, resulting in a poor response to treatment.- - - - - - - - - - ranking = 5keywords = leukemia (Clic here for more details about this article) |
8/21. Treatment of radiation-induced fibrosis of the face with manual compression therapy.radiation-induced fibrosis (RIF) is an uncommon complication of radiation therapy. RIF most often occurs in the extremities; it is rare in the head and neck. Only a few medical treatments for RIF are available, and they have been mediocre at best. We describe a case of RIF of the face that was treated successfully with a nonmedical modality: manual compression therapy.- - - - - - - - - - ranking = 1872.9113496148keywords = radiation-induced (Clic here for more details about this article) |
9/21. Myelodysplastic/myeloproliferative disease with erythropoietic hyperplasia (erythroid preleukemia) and the unique translocation (8;9)(p23;p24): first description of a case.We report on a patient fulfilling the diagnostic criteria of unclassifiable myelodysplastic/myeloproliferative diseases with prominent erythropoietic hyperplasia/dysplasia (erythroid preleukemia) and the unique translocation (8;9)(p23;p24). The patient presented with B-symptoms, erythroblastemia, thrombopenia, marked eosinophilia, presence of myeloid precursors in the peripheral blood, and decreased erythropoietin level. Nodular peritrabecular polymorphous blasts, dysplastic megakaryocytes, and a diffuse argyrophilic fibrosis were detected in the trephine bone marrow biopsy. Immunohistochemically, the blasts stained positively for glycophorin C and hemoglobin a; the proliferation fraction was nearly 90% in the Ki-67 stain. Expression of the phosphorylated janus kinase 2 was detected in almost all megakaryocytes and in isolated erythroblast islets, suggesting a probable activation of janus kinase 2, the jak-2 gene being mapped on 9p24. Ten months after initial diagnosis, the disease progressed to frank acute erythroid leukemia. We report for the first time a myelodysplastic/myeloproliferative disease (erythroid preleukemia) accompanied by the specific chromosomal aberration t(8;9)(p23;p24), distinct histopathology, and clinical and laboratory symptoms, and progress to acute erythroid leukemia.- - - - - - - - - - ranking = 8keywords = leukemia (Clic here for more details about this article) |
10/21. Recurrent syncope following radiation therapy.This case report describes a 51-year-old man who presented with syncope. His electrocardiogram showed an abnormally long pause and the electrophysiology study revealed a prolonged H-V interval. This was attributed to the radiation therapy he received to the chest. He was successfully treated with implantation of a permanent pacemaker. This case highlights this rare complication of radiation-induced conduction system disease and management of this potentially life-threatening condition.- - - - - - - - - - ranking = 468.22783740371keywords = radiation-induced (Clic here for more details about this article) |
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