Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Immunosuppression in the setting of solid organ transplantation is associated with the development of a variety of malignant tumors, most commonly squamous carcinomas and non-Hodgkin's lymphomas. Sarcomas, apart from Kaposi's sarcoma, are relatively infrequent. We recently encountered a 71-year-old man with chronic renal failure, treated by allograft kidney transplantation, who developed a high-grade epithelioid angiosarcoma at the site of a nonfunctioning arteriovenous fistula, previously constructed for hemodialysis. At diagnosis, the patient had numerous satellite nodules of angiosarcoma involving the distal skin, soft tissues, and bones. After a below-elbow amputation, there was a rapid local recurrence at the amputation stump. Currently, the patient is alive with numerous pulmonary metastases, 6 months after amputation. A literature review identified three recently reported identical cases of epithelioid angiosarcoma arising in nonfunctioning arteriovenous fistulae. All three patients had been treated by kidney transplantation for renal failure, suggesting a possible causal association between these events. We performed polymerase chain reaction for human herpes virus 8, the recently recognized herpes virus proposed as a major etiologic agent of Kaposi's sarcoma, and possibly some conventional angiosarcomas, but we failed to identify any viral DNA within the tumor.
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PMID:Epithelioid angiosarcoma arising in a surgically constructed arteriovenous fistula: a rare complication of chronic immunosuppression in the setting of renal transplantation. 973 50

Pentostatin (2prime prime or minute-deoxycoformycin, dCF) is a product of the fermentation of Streptomyces antibioticus. It is a tight-binding inhibitor of adenosine deaminase (ADA), an enzyme essential in cellular metabolism of purines. Children with congenital absence of ADA suffer from atrophy of lymphoid tissues and severe combined immune deficiency (SCID) syndrome. It was speculated that pentostatin would be lymphocytotoxic, and this proved to be the case, promoting its investigation in lymphoid neoplasms. It was anticipated that pentostatin would be most active in neoplasms with high intracellular concentrations of ADA---e.g., acute lymphocytic leukemia (ALL), particularly its T cell variety. Although pentostatin proved to be active in ALL, large doses were required and toxic effects outweighted therapeutic benefits. By contrast, pentostatin proved to be exceptionally active in hairy cell leukemia (HCL), a B cell neoplasm with low intracellular concentrations of ADA. Pentostatin has since been shown to possess activity in chronic lymphocytic leukemia, prolymphocytic leukemia, cutaneous T cell lymphomas, adult T cell lymphoma-leukemia, and low-grade non-Hodgkin's lymphomas. It potentiates the activity of vidarabine against viruses and against the cells of acute myeloid leukemia. Pentostatin is inactive in melanoma and renal carcinoma, but has not been adequately evaluated in other solid tumors. The toxic effects of pentostatin include renal failure, central nervous system (CNS) depression, immunosuppression, keratoconjunctivitis, and opportunistic infections. In the absence of pre-existing bone marrow compromise, pentostatin produces only mild myelosuppression. Aside from its use as an antineoplastic agent, pentostatin has potential applications as an immunosuppresive drug, as an antiviral agent, as an antimalarial compound, and in the protection of cells of the CNS from damage induced by ischemia and anoxia.
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PMID:Pentostatin (2prime prime or minute-Deoxycoformycin): Clinical Pharmacology, Role In Cancer Chemotherapy, and Future Prospects. 1184 52

Acute renal failure (ARF) as a consequence of non-Hodgkin lymphoma infiltration of the kidneys (LIK) is an uncommon complication of non-Hodgkin lymphoma. In literature, ARF due to LIK is reported in progressive disease. A case of non-oliguric acute renal failure secondary to relapse of large B cell non-Hodgkin lymphoma primarily localized in the mediastinum is reported. LIK of both kidneys was diagnosed by ultrasonography, computer tomography scan and 18-fluorodeoxyglucose positron emission tomography. No other causes for renal failure were found. The prognosis of renal involvement in relapsed non-Hodgkin lymphoma is poor, as is demonstrated by our case.
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PMID:Acute renal failure due to non-Hodgkin lymphoma infiltration of the kidneys detected by ultrasonography and confirmed by positron emission tomography. 1277 2

Three cases of generalized cytomegalic inclusion-body disease (salivary virus disease) in adults are reported, bringing the number of published cases up to 34. The infection is very rare in adults although well known in infants. As is often found in infants with this disease, pneumonia due to Pneumocystis carinii was also present in each case. The first patient had Wegener's granulomatosis, which presented with acute otitis media: a review of histological material obtained at mastoidectomy eight weeks before death showed that inclusion-body cytomegaly was already present then. Various antibiotics and prednisolone were given, and the lesions in the respiratory organs and the arteritis healed to a considerable extent. Renal failure, however, was progressive and led to death. The second patient had thrombotic purpura and died after a few weeks' illness, during which oxytetracycline and hydrocortisone were given. Congenital absence of the spleen was found at laparotomy, which was performed with the object of doing a splenectomy. Focal cryptococcal pneumonia was present post mortem: six years before death a solitary cryptococcal granuloma of one lung had been treated by lobectomy. The third patient had had Hodgkin's disease for 18 years. During the first 12 years the disease had the characteristics of the so-called indolent form ("Hodgkin's paragranuloma") and it then passed into the typical form. Deep x-ray therapy and cytotoxic drugs were used during the course of the disease at various times, and streptomycin and tuberculostatic drugs were given because of intercurrent tuberculous meningitis which developed three months before death. In all three cases it seems likely that the underlying disease, or the drugs used in its treatment, predisposed to cytomegalic inclusion-body disease and concomitant pneumocystis pneumonia by lowering the patients' resistance. Just as some unusual types of fungal and bacterial infections have become less rare since the introduction of certain drugs, including antibiotics and steroids, it is possible that cytomegalic inclusion-body disease and pneumocystis infection may also be met with oftener in adults, perhaps particularly as a complication of the use of these drugs in the treatment of diseases which are specially liable to interfere with the body's defences.
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PMID:Generalized cytomegalic inclusion-body disease associated with pneumocystis pneumonia in adults. A report of three cases. with Wegener's granulomatosis. thrombotic purpura, and Hodgkin's disease as predisposing conditions. 1383 12

Pentostatin (2'-deoxycoformycin, dCF) is a purine nucleoside analog and a product of the fermentation of Streptomyces antibioticus. It is a tight-binding inhibitor of adenosine deaminase (ADA), an enzyme essential in the cellular metabolism of purines. Children with congenital absence of ADA suffer from atrophy of lymphoid tissues and severe combined immune deficiency (SCID) syndrome. It was hypothesized that pentostatin would be lymphocytotoxic and this proved to be true; this finding prompted its investigation in lymphoid neoplasms. It was anticipated that pentostatin would be most active in neoplasms with high intracellular concentrations of ADA, e.g. acute lymphocytic leukemia (ALL), particularly of the T-cell variety. Although pentostatin proved to be active in ALL, large doses were required and major toxic effects outweighed therapeutic benefits. By contrast, pentostatin proved to be exceptionally active in hairy cell leukemia (HCL), a B-cell neoplasm with low intracellular concentrations of ADA. Pentostatin has since been shown to possess activity in chronic lymphocytic leukemia, prolymphocytic leukemia, cutaneous T-cell lymphomas, adult T-cell lymphoma-leukemia, and low grade non-Hodgkin's lymphomas. It potentiates the activity of vidarabine against viruses and against the cells of acute myeloid leukemia. Pentostatin is inactive in melanoma and renal carcinoma, but has not been adequately evaluated in other solid tumors. The toxic effects of pentostatin include renal failure, central nervous system (CNS) depression, immunosuppresion, keratoconjunctivitis, and opportunistic infections. In the absence of pre-existing bone marrow compromise, pentostatin produces only mild myelosuppression. Aside from its use as an antineoplastic agent, pentostatin has potential applications as an immunosuppressive drug, as an antiviral agent, as an antimalarial compound, and in the protection of cells of the CNS from damage induced by ischemia and anoxia. Clinical studies with pentostatin are ongoing, and its roles in the management of neoplastic and non-neoplastic diseases have yet to be fully defined.
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PMID:Deoxycoformycin (pentostatin): clinical pharmacology, role in the chemotherapy of cancer, and use in other diseases. 1465 Dec 24

Renal involvement with amyloidosis is common but causes patient survival to be poor, rarely reaching 5 years. In this study, we retrospectively reviewed clinical and biological characteristics as well as treatments and outcomes of patients with renal amyloidosis followed for more than 5 years. Between 1975 and 2003, 485 patients were diagnosed with renal amyloidosis including only 12 patients who were followed more than 5 years. The six men and six women of mean age 42.4 years (range 18 to 66 years) displayed renal signs of lower limb edema in all cases; hypertension in four cases, proteinuria on urinalysis in all cases with microscopic hematuria in five cases. Biological tests showed nephrotic syndrome in 11 patients, normal renal function in nine patients, and renal failure in three patients whose mean creatinine was 481.6 micromol/L (range 294 to 726). The amyloidosis was AA type in 11 cases and non-AA in one case. An etiologic survey revealed spondylarthropathy in one patient, pulmonary tuberculosis in two patients, chronic bronchitis in three patients, hepatic hydatic cyst in one patient, Mediterranean familial fever in two patients, Crohn's disease in one patient, Hodgkin's lymphoma in one patient, and multiple myeloma in one patient. Specific treatment was initiated with colchicine in seven patients. At a 110-month mean follow-up (range 53 to 153 months), remission of nephrotic syndrome was observed in four cases, progression to chronic renal failure in two patients, and to end-stage renal failure in five cases (range 53 to 196 months), with stabilization of renal function in seven patients. In conclusion, primary amyloid disease should be optimally suppressed in patients with renal involvement. The role of this treatment in remission of renal amyloidosis is not well established. This efficacy of the treatment has been demonstrated in some patients with improved survival.
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PMID:Renal amyloidosis followed more than 5 years: report of 12 cases. 1535 Apr 80

High-dose methotrexate is included in chemotherapy regimens used to treat a number of malignant neoplasms. Methotrexate plasma concentration is considered the best toxicity predictor. Monitoring methotrexate plasma concentrations is standard practice in the identification of at-risk patients, the titration of folinic acid doses, and the establishment of corrective measures. Methotrexate is a kidney-cleared weak acid, and renal function impairment may retard methotrexate clearance. A case of severe methotrexate-induced toxicity secondary to renal failure is reported in a patient with non-Hodgkin s lymphoma receiving methotrexate at a dose of 1 g/m2. Corrective measures included folinic acid rescue therapy, cholestyramine resin administration, hydration and urine alkalinization, urine pH monitoring, and extracorporeal clearance techniques.
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PMID:[Managing methotrexate toxicity: a case report]. 1550 95

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome characterized by headache, conscious disturbance, seizure, and cortical visual loss with neuroimaging finding of edema in the posterior regions of the brain, with a reversible course (1). The precise pathomechanism of RPLS is not understood. However, association with uncontrolled hypertension, renal failure, eclampsia, or immunosuppressive agents has been implicated (1). We describe herein a case of Hodgkin's disease (HD)-related central nervous system (CNS) angiitis with neuroimaging finding suggestive of RPLS. The pathophysiology of RPLS in cases with CNS angiitis is discussed.
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PMID:Hodgkin's disease-related central nervous system angiopathy presenting as reversible posterior leukoencephalopathy. 1557 37

We report a case of non-Hodgkin lymphoma (NHL) with acute renal failure. A 62-year-old man was admitted to our hospital on March 8, 2002 with leg edema and dyspnea on effort. About 3 weeks before admission, he was found to have slightly high serum creatinine (Cr) and high lactate dehydrogenase (LDH) levels by another home doctor. Physical examination revealed anemic conjunctivae and leg edema, but the urinary volume was preserved. Blood examination showed high BUN (64 mg/dl) and Cr levels (3.91 mg/dl). Urinary analysis showed proteinuria (1.05 g/day) and high BMG (14,434/microg/day) and NAG (4.55 U/day) levels, suggesting severe tubulointerstitial injury. On ultrasonography of the kidney, the bilateral kidneys showed marked swelling without hydronephrosis. To investigate the genesis of renal failure, we performed a renal biopsy. The specimen showed normal glomeruli, but a large number of cells infiltrated in the tubulointerstitial area with normal tubulointerstitial structure. The cells stained positively with anti-leukocyte antigen and L26 (B cell marker), and negatively with cytokeratin and UCHL-1 (T cell marker). These findings indicate that the interstitial cells were non-Hodgkin lymphoma with B cell diffuse large cells. Chemotherapy was performed with VAD (vincristine sulfate, doxorubicin hydrochloride, dexamethasone) considering his renal dysfunction. To avoid tumor lysis syndrome after chemotherapy, hemodialysis was performed on days 1-4 after the initiation of chemotherapy. After a series of chemotherapy, the urinary volume increased and serum Cr levels decreased to 2 mg/dl. After additional therapy with 4 courses of CHOP, he improved and was discharged on day 180 after admission.
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PMID:[Case of non-Hodgkin lymphoma with acute renal failure successfully treated with chemotherapy]. 1564 40

Catastrophic antiphospholipid syndrome (CAPS) is a severe and rare variant of antiphospholipid syndrome (APS) characterized by acute multiorgan failure due to small vessel thrombi in patients with positive antiphospholipid antibodies. We report a fatal case of catastrophic antiphospholipid syndrome in a young woman with a history of polymyositis and Hodgkin lymphoma. The patient was admitted to hospital because of severe foot pain following several weeks of skin ulcerations. Doppler ultrasonography showed evidence of arterial ischemia of the both lower extremities. Despite anticoagulation, immunosuppression, plasmapheresis and antibiotic therapy, she developed cutaneous gangrene, retroperitoneal hematoma, ileus, and acute respiratory and renal failure that resulted in death. Autopsy showed multifocal vascular injury and microthrombi with associated hemorrhages and infarcts in multiple organs. The patient had normal levels of functional protein C and protein S and a normal level of plasma homocysteine. Tests for common thromophilic gene mutations including prothrombin 20210, factor V Leiden 1691, and methylene tetrahydrofolate reductase 677 were negative. To our knowledge, this is the first CAPS patient with molecular studies for genetic prothrombotic mutations. Our report showed that there was no association between the development of CAPS and inherited thromophilia.
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PMID:Catastrophic antiphospholipid syndrome: a rare cause of disseminated microvascular thrombotic injury - a case report with pathological and molecular correlative studies. 1574 23


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