Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Over a seven year period 52 patients having a clinical diagnosis of spontaneous peripheral arterial microembolization were identified. Sixty-one percent of patients were female, 15% were diabetic, and 73% used tobacco chronically. A striking finding was the very high incidence of associated systemic disorders such as thrombocytosis (8), polycythemia vera (3), metastatic adenocarcinoma (3), or collagen disease requiring steroid therapy (4). Forty-nine patients had significant proximal arterial lesions as the origin of their emboli. Three patients had digital ischemia as a result of increased platelet aggregation without arterial obstruction. Forty-eight patients underwent surgical therapy. Operative mortality was 4% and overall limb salvage in survivors was 96%. The clinical syndrome of arterial microembolization may result from several pathophysiologic mechanisms including cholesterol embolization from ulcerated plaques, fibrino-platelet aggregation in patients with hematologic disorders, or dislodgement of mural thrombus in those with aneurysmal disease. We observed aortoiliac disease to be more frequent than femoral-popliteal disease, and both were amenable to surgical correction. We conclude that the genesis of arterial microembolization is multifactorial and that a variety of systemic diseases may work in concert with atherosclerotic arterial disease to produce this clinical syndrome. Prompt recognition and appropriate treatment of this disorder can yield high rates of limb salvage.
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PMID:Spontaneous peripheral arterial microembolization. 139 20

Poly(A) polymerase (polynucleotide adenylyltransferase; ATP:polynucleotide adenylyltransferase, EC 2.7.7.19) was covalently linked to diazobenzyloxymethyl-filters and used to screen the sera from a number of tumor-bearing rats and human cancer patients for antibodies to poly(A) polymerase. Sera from rats that had been inoculated with any of several Morris hepatomas or a mammary adenocarcinoma contained immunoglobulins capable of complexing with poly(A) polymerase. No antibodies to the enzyme could be detected in sera from control animals or from those bearing tumors for short periods of time. Antibodies to poly(A) polymerase were also observed in sera from human patients with leukemia, polycythemia vera, and Wilms tumor. The antibodies were not evident in sera from normal volunteers or from patients with nonneoplastic diseases. These included lupus erythematosus, a disorder in which antibodies are produced against an array of nuclear proteins. Immunoglobulins from the serum of one of the human patients were capable of inhibiting poly(A) polymerase activity in vitro, whereas those prepared from the serum of a normal volunteer did not affect enzyme activity. As determined by the diazobenzyloxymethyl-filter technique, the relative concentration of antibodies in the sera of an individual with leukemia (in remission) increased severalfold during a relapse. These data suggest that the presence of antibodies to poly(A) polymerase may be characteristic of sera from cancer patients and that the relative concentration of these antibodies may be indicative of the disease state.
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PMID:Anti-poly(A) polymerase antibodies in sera of tumor-bearing rats and human cancer patients. 627 86

A 77-year-old male having multiple small nevi over the lips, face, mucous membrane of mouth, tongue, neck and thorax was admitted to our hospital because of severe anemia. He has been diagnosed as Rendu-Osler-Weber disease (Osler's disease) since his 42 years of age. Although the stools were positive for occult blood, hematologic examination disclosed no abnormalities except for severe hypochromic and microcytic anemia. Gastrointestinal examination showed multiple stigmata in the esophagus, stomach, duodenum, and several tumors in the colon. Histological findings of biopsy specimens obtained from these tumors revealed adenocarcinomas and tubular adenomas. Surgical resection was carried out. Resected specimens showed two tumors (one is in ascending colon appeared to be 2 type carcinoma, 1.9 x 2.3 cm in size, another is in caecum, 3 type, 7.0 x 5.0 cm in size) and three Yamada-II approximately III type polyps. Pathohistologically, the tumor in ascending colon showed moderately differentiated adenocarcinoma, another one in caecum showed mucinous carcinoma, and polyps showed tubular adenomas, respectively. Unfortunately, the anemia of this case was regarded as stemming from Osler's disease, so the precise gastrointestinal examination was not performed for a long time. It should be emphasized to be faithful to the fundamentals of medical diagnosis.
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PMID:[A case of Rendu-Osler-Weber disease associated with simultaneous, multiple advanced cancers in the colon]. 770 55

Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. We present the case of a 69-year-old male with a history of polycythemia vera (PV) who developed proximal muscle weakness, dysphagia, and rash. He was found to have elevated creatinine kinase and skin biopsy was consistent with DM. Due to persistent pancytopenia a bone marrow biopsy was performed and showed postpolycythemic myelofibrosis. To our knowledge, this is the first case reported of this unique association.
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PMID:Dermatomyositis Associated with Myelofibrosis following Polycythemia Vera. 2866 85

Basophils were identified in human peripheral blood by Paul Ehrlich over 140 years ago. Human basophils represent <1% of peripheral blood leukocytes. During the last decades, basophils have been described also in mice, guinea pigs, rabbits, and monkeys. There are many similarities, but also several immunological differences between human and mouse basophils. There are currently several strains of mice with profound constitutive or inducible basophil deficiency useful to prove that these cells have specific roles in vivo. However, none of these mice are solely and completely devoid of all basophils. Therefore, the relevance of these findings to humans remains to be established. It has been known for some time that basophils have the propensity to migrate into the site of inflammation. Recent observations indicate that tissue resident basophils contribute to lung development and locally promote M2 polarization of macrophages. Moreover, there is increasing evidence that lung-resident basophils exhibit a specific phenotype, different from circulating basophils. Activated human and mouse basophils synthesize restricted and distinct profiles of cytokines. Human basophils produce several canonical (e.g., VEGFs, angiopoietin 1) and non-canonical (i.e., cysteinyl leukotriene C4) angiogenic factors. Activated human and mouse basophils release extracellular DNA traps that may have multiple effects in cancer. Hyperresponsiveness of basophils has been demonstrated in patients with JAK2V617F-positive polycythemia vera. Basophils are present in the immune landscape of human lung adenocarcinoma and pancreatic cancer and can promote inflammation-driven skin tumor growth. The few studies conducted thus far using different models of basophil-deficient mice have provided informative results on the roles of these cells in tumorigenesis. Much more remains to be discovered before we unravel the hitherto mysterious roles of basophils in human and experimental cancers.
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PMID:Is There a Role for Basophils in Cancer? 3301 85