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1/25. The role of clostridium septicum in paraneoplastic sepsis.

    CONTEXT: clostridium septicum infections are rare but often associated with serious if not fatal outcomes. clostridium septicum infection does not appear to be associated with a single specific defect in cellular or humoral immunity. It has been associated with multiple medical problems, including but not limited to leukemia, malignancy of the bowel, other solid tumors, cyclic neutropenia with enterocolitis, diabetes mellitus, and severe arteriosclerosis. Most cases of C septicum are associated with malignancy, and mortality approaches 100% if care is not rendered within 12 to 24 hours. OBJECTIVES: To evaluate outcomes of patients with C septicum bacteremia, whether treated medically or surgically or both, and to note associated conditions. DESIGN: Retrospective evaluation of patients found to have C septicum bacteremia in the past 6 years. SETTING: Two teaching hospitals, Brooke Army Medical Center (250 beds) and Wilford Hall Medical Center (292 beds), were the source of our patients. PATIENTS: All patients found to have C septicum bacteremia during hospitalization or postmortem examination were included in the study. There were no exclusion criteria. MAIN OUTCOME MEASURE: mortality associated with C septicum infection. RESULTS: In our case series, mortality was 33%, which is slightly lower than reported in prior studies (43%-70%). CONCLUSION: Presumptive identification based on Gram stain, awareness of C septicum infection as a paraneoplastic syndrome, and prompt, clear communication between laboratory personnel and clinicians are necessary for early diagnosis of C septicum infection. Early institution of antibiotic therapy improves prognosis.
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ranking = 1
keywords = paraneoplastic, paraneoplastic syndrome
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2/25. adult Henoch-Schonlein purpura associated with malignancy.

    OBJECTIVE: Malignancies are among the well-established causes of vasculitis. We studied the association between adult Henoch-Schonlein purpura (HSP) and malignant neoplasms. methods: We retrospectively reviewed 14 cases of adult HSP diagnosed during a 6-year period and found a malignant neoplasm in four. Fifteen reports of adult HSP with malignant disease were identified in the literature. These 19 cases were compared with 158 adults who had HSP but no malignancy and who where reported in the literature. RESULTS: Most (63%) of the malignant neoplasms associated with adult HSP were solid tumors: lung, n = 6; prostate, n = 2; breast, n = 1; renal, n = 1; stomach, n = 1; and small bowel, n = 1. Hematologic malignancies (37%) included non-Hodgkin lymphoma, n = 2; hodgkin disease, n = 2; IgA multiple myeloma, n = 1; myeloproliferative disease, n = 1; and myelodysplastic syndrome, n = 1. Patients with malignancy were older (median age, 59 years), were more likely to be male (M/F = 8.5), had joint involvement more frequently (95%), and had a lower frequency of prior acute infection than those without malignancy. A typical paraneoplastic course was documented in only two cases. CONCLUSIONS: Epidemiological studies are needed to determine the association between adult HSP and malignancy. In practice, it may be wise to suspect a malignancy in men older than 40 years of age who develop HSP in the absence of a precipitating factor. Pathogenic hypotheses involve tumor antigens or abnormal IgA production leading to immune complex formation.
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ranking = 0.17058256913584
keywords = paraneoplastic
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3/25. Interferon-induced retinopathy in asymptomatic cancer patients.

    OBJECTIVES: Interferon-induced ocular complications, including retinal ischemia and ischemic optic neuropathy, can be associated with significant visual loss. We report three cases of asymptomatic ischemic retinopathy in cancer patients receiving interferon. DESIGN: Retrospective, interventional, noncomparative small case series. methods: Retrospective review of the medical records and fundus photographs. RESULTS: Interferon-induced ischemic retinopathy can occur in asymptomatic cancer patients. The retinal changes are usually reversible with discontinuation of interferon therapy. CONCLUSIONS: These three cases underscore the importance of dilated funduscopic examination at baseline and during follow-up, at least every 3 months, for all cancer patients receiving interferon to identify retinal toxicity at its earliest stages. A prospective study evaluating the incidence and severity of interferon retinopathy in cancer patients would be prudent.
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ranking = 0.00011330863291339
keywords = ocular
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4/25. Temporal concurrence of vasculitis and cancer: a report of 12 cases.

    OBJECTIVE: Vasculitis has been associated with solid organ and hematologic cancer. The rarity of these associations, and in many reports the lack of temporal relationships, has led to skepticism about vasculitis being a paraneoplastic syndrome. The objective of the present study was to review cases of concurrent vasculitis and cancer at the Cleveland Clinic Foundation over an 18.5-year period and explore evidence that would support the notion of vasculitis being a type of paraneoplastic disease. methods: Retrospective review of the records of all patients diagnosed with vasculitis and cancer within 12 months of each other was performed using an ICD-9 diagnostic data base at the Cleveland Clinic Foundation. Patients with known chronic autoimmune disease or serologic evidence of hepatitis b or C infection were excluded. A standardized data collection instrument was used to document information about presentation, treatment, and course of illness. RESULTS: During the 18.5 years of our study, more than 15 million inpatients and outpatients were seen at the Cleveland Clinic. Of these, 2,800 patients had vasculitis independent of cancer, more than 69,000 patients had cancer, and 69 patients had been identified who had both malignancies and systemic vasculitis. Only 12 patients were identified in whom both vasculitis and cancer occurred within the same 12 months. Mean age was 65 years (range 45-79). There was no gender preference (M = F). In 8 of the 12 cases, diagnoses were made within 3 months of each other. In 6 of the patients, the diagnoses of both processes were made within 1 month. Ten of the 12 patients had vasculitis 1 to 3 months prior to or concurrent with the diagnosis of cancer. Six of the 12 patients had solid organ tumors, 4 had lymphoma, 1 had leukemia, and 1 had multiple myeloma. The most common vasculitis was cutaneous leukocytoclastic vasculitis (LCV), which occurred in 7 cases. Four cases of LCV were associated with solid organ tumors. Other vasculitides included giant cell arteritis (n = 2), polyarteritis nodosa (n = 2), and Wegener's granulomatosis (n = 1). The response of the vasculitis to glucocorticoid and cytotoxic therapy varied. Complete remission of vasculitis occurred in 4 of the patients, partial improvement occurred in 4 patients, and no improvement was noted in 4 patients. Complete remission occurred in 3 of the 4 patients in whom vasculitis and cancer were treated concurrently. Eight of 10 patients in whom followup was greater than 2 months demonstrated concordance of disease activity and treatment response for both cancer and vasculitis. CONCLUSION: The close temporal relationship of cancer and vasculitis in our patients adds to circumstantial evidence of vasculitis at times being a paraneoplastic condition. Failure of a vasculitis to respond to conventional therapy should raise questions about underlying malignancy. Effective treatment of the cancer enhances the likelihood of improvement in vasculitis.
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ranking = 0.65883486172831
keywords = paraneoplastic, paraneoplastic syndrome
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5/25. stevens-johnson syndrome and toxic epidermal necrolysis: oncologic considerations.

    stevens-johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe skin toxicities that may occur in patients with cancer. They are caused by infection or a drug reaction and can result in sepsis, severe ocular complications, and even death. skin lesions usually are preceded by prodromal flulike symptoms. A rash with subsequent blistering and denudation follows. Diagnosis is made by skin biopsy, and classification is based on body surface area and visceral organ involvement. Because many of the drugs associated with SJS and TEN are used to treat cancer, early recognition and intervention are critical to achieving a favorable outcome. Interventions include stopping the suspected offending agent. The use of steroids is controversial. Healthcare professionals always should consider an early transfer to a burn unit for patients with a comorbid condition such as cancer because this action is associated with improved outcomes. Implications for oncology nurses and a case study are presented in this article.
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ranking = 0.00011330863291339
keywords = ocular
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6/25. Early-onset drusen in a girl with bloom syndrome: probable clinical importance of an ocular manifestation.

    Ophthalmic examination of a girl admitted with the complaint of growth failure revealed retinal hard drusen. It was surprising to observe drusen in a child because they represent an age-related degenerative change in normal individuals. After further evaluation, she was diagnosed to have bloom syndrome, a premature aging syndrome. To the authors' knowledge, this is the first case of bloom syndrome associated with drusen. It is probable that not only aging but also other fundamental cell processes, especially uncontrolled cell proliferation, might be similarly affected and might follow a more rapid course in this inherited condition presenting with drusen. The authors suggest paying extra attention to drusen during the ophthalmic assessment in the diagnosis of all bloom syndrome patients; it may be prudent to watch more carefully for the development of cancer in patients with drusen than those without drusen.
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ranking = 0.00045323453165354
keywords = ocular
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7/25. Amphiphysin autoimmunity: paraneoplastic accompaniments.

    Amphiphysin-IgG was identified in 71 patients among 120,000 evaluated serologically for paraneoplastic autoantibodies. Clinical information was available for 63 patients. Cancer was detected in 50 (mostly limited), proven histologically in 46, and was imaged intrathoracically in 4 patients (lung, small-cell [27] and non-small cell [1]), breast [16] and melanoma [2]). Neurological accompaniments included (decreasing frequency): neuropathy, encephalopathy, myelopathy, stiff-man phenomena, and cerebellar syndrome. In a case examined neuropathologically, parenchymal T-lymphocyte infiltration (predominantly CD8( )) was prominent in lower brainstem, spinal cord, and dorsal root ganglion. Coexisting paraneoplastic autoantibodies, identified in 74% of patients, predicted a common neoplasm and indicated other neuronal autoantigen targets that plausibly explained several neurological manifestations; for example, P/Q-type Ca(2 )-channel antibody with Lambert-Eaton syndrome (n = 5), anti-neuronal nuclear antibody type 1 with sensory neuronopathy (n = 7), K( )-channel antibody with limbic encephalitis (n = 1) or neuromyotonia (n = 1), and collapsin response-mediator protein-5-IgG with optic neuritis (n = 3). Patients with isolated amphiphysin-IgG (n = 19) were more likely to be women (with breast cancer, p < 0.05) and to have myelopathy or stiff-man phenomena (p < 0.01). overall, a minority of women (39%) and men (12%) had stiff-man phenomena. Only 10% of women (some with lung carcinoma) and 4% of men fulfilled diagnostic criteria for stiff-man syndrome.
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ranking = 1.0234954148151
keywords = paraneoplastic
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8/25. Pseudoachalasia: a case series and analysis of the literature.

    OBJECTIVE: Pseudoachalasia frequently cannot be distinguished from idiopathic achalasia by manometry, radiologic examination or endoscopy. Mechanisms proposed to explain the clinical features of pseudoachalasia include a circumferential mechanical obstruction of the distal esophagus or a malignant infiltration of inhibitory neurons within the myenteric plexus. MATERIAL AND methods: Between January 1980 and December 2002, the clinical features of 5 patients with pseudoachalasia and 174 patients with primary achalasia, diagnosed in a single center, were compared. A literature analysis of the etiology of pseudoachalasia for the time period 1968 to December 2002 was performed. The search concentrated on the databases and online catalogues pubmed, Web of science, Cochrane Library and Current Contents Connect. RESULTS: In our case series, patients with pseudoachalasia reported a shorter duration of symptoms and tended to be older than patients with primary achalasia. Conventional manometry, endoscopy and radiologic examination of the esophagus proved to be of little value in distinguishing between the diseases. In the majority of cases only surgical exploration revealed the underlying cause. A coincidence of primary achalasia and disorders of the gastroesophageal junction was excluded by showing return of peristalsis following treatment. The analysis of the literature showed a total of 264 cases of pseudoachalasia in 122 publications. Most cases of were due to malignant disease (53.9% primary and 14.9% secondary malignancy), followed by benign lesions (12.6%) and sequelae of surgical procedures at the distal esophagus or proximal stomach (11.9%). In rare instances, the disease was an expression of a paraneoplastic process due to distant neuronal involvement rather than to local invasion with destruction of the myenteric plexus (2.6%). CONCLUSIONS: The diagnosis of pseudoachalasia is difficult to establish by conventional diagnostic measures. The main distinguishing feature of secondary versus primary achalasia is the complete reversal of pathologic motor phenomena following successful therapy of the underlying disorder.
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ranking = 0.17058256913584
keywords = paraneoplastic
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9/25. Tumor-induced osteomalacia.

    Tumor-induced osteomalacia (TIO) is a rare paraneoplastic form of renal phosphate wasting that results in severe hypophosphatemia, a defect in vitamin d metabolism, and osteomalacia. This debilitating disorder is illustrated by the clinical presentation of a 55-year-old woman with progressive fatigue, weakness, and muscle and bone pain with fractures. After a protracted clinical course and extensive laboratory evaluation, tumor-induced osteomalacia was identified as the basis of her clinical presentation. In this article, the distinctive clinical characteristics of this syndrome, the advances in diagnosis of TIO, and new insights into the pathophysiology of this disorder are discussed.
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ranking = 0.17058256913584
keywords = paraneoplastic
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10/25. Vasculitis as a paraneoplastic syndrome. Report of 11 cases and review of the literature.

    In a study of 222 patients with vasculitis, we identified 11 who had an associated neoplasia. Seven had hematological neoplasia and 4 had solid malignant tumors. In 4 patients vasculitis gave the first evidence of the neoplasia or of its recurrence. Nine of our patients had cutaneous vasculitis. The other 2 had vasculitis involving the intestine and resulted in acute abdomens. These 2 patients needed prednisone treatment for the vasculitis. Neoplasia should be considered in patients with vasculitis without an apparent cause.
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ranking = 1.2706788938265
keywords = paraneoplastic, paraneoplastic syndrome
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