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

Sialyl Lewisx-i (SLX) was found in more than 40% of patients with acute leukemia or chronic myelogenous leukemia, and in about 20% of those with myelodysplastic syndrome or malignant lymphoma. This tumor marker was absent in all patients with polycythemia vera, essential thrombocythemia, primary myelofibrosis, chronic lymphatic leukemia, multiple myeloma, and those with acute leukemia or malignant lymphoma in remission. The marker was found in 8% and of the patients with idiopathic thrombocytopenic purpura and 33% of those with autoimmune hemolytic anemia but in no patient with aplastic anemia or megaloblastic anemia. Immunostaining with SLX antibody showed that tumor cells of the patients with high levels of serum SLX were producing the SLX antigen. The detection of this marker in the serum is thought to be useful not only in the diagnosis but also in the observation of the recurrence of the diseases.
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PMID:Evaluation of serum sialyl Lewisx-i in hematologic disorders. 207 71

Twenty-six patients with the Budd-Chiari syndrome were treated surgically at the Johns Hopkins Hospital. Twenty-one of the patients were female and five were male, with a median age at diagnosis of 37 years. Nine patients had polycythemia vera, 6 were receiving estrogen therapy, 5 had a previous hepatitis A or B infection, and 4 had cirrhosis. There was one case each of hepatic malignancy, paroxysmal nocturnal hemoglobinuria, and idiopathic thrombocytopenic purpura. In five cases no etiologic factors or associated disorders were identified. Ascites was the most common presenting feature in this group of patients. Hepatic function at the time of diagnosis, as measured by standard serum chemistries, was only minimally abnormal. The diagnosis of the Budd-Chiari syndrome was confirmed in all 26 patients by hepatic vein catheterization. Inferior vena cavography was also performed and revealed caval occlusion in 4 patients, significant caval obstruction in 13 patients, and a normal vena cava in 9 patients. Interpretation of the vena cavogram was helpful in selecting the appropriate surgical procedure for each patient. Twenty-three of the twenty-six patients underwent percutaneous liver biopsy before operation, with no morbidity or mortality. Four patients had well-established cirrhosis noted on biopsy. Thirty mesenteric-systemic venous shunts were performed on the 26 patients. In 11 patients a mesocaval shunt was performed and in one instance conversion to a mesoatrial shunt was required as a second procedure. In 15 patients a mesoatrial shunt was performed as the initial procedure. Graft thrombosis occurring in 2 of these 15 patients prompted one revision in 1 patient and 2 revisions in the second patient. After mesenteric-systemic venous shunt, eight of the patients (31%) died before discharge from the hospital. The remaining 18 patients in this series were discharged from the hospital alive and well with patent shunts. Patients were followed for a median of 43 months (range, 9 months to 13 years). Five late deaths occurred between 5 and 84 months after the operation. Three- and five-year actuarial survival rates were 65% and 59%, respectively.
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PMID:Current management of the Budd-Chiari syndrome. 200 20

Out of 2,474 bone marrow biopsies we have observed 330 cases (13.3%) with presence of lymphoid nodules (LN). LN were frequent in old age (24.6% over 80 years), in females (17%) and in some diseases, such as rheumatoid arthritis and systemic lupus erythematosus (73.7% of the cases), partial aplasia (34%), hypersplenism (30.4%), hemopoietic dysplasia (25%), chronic renal failure (20.4%), polycythemia vera (20.2%), idiopathic thrombocytopenic purpura (18.8%), acute leukemia (17.7%). Nodular lymphoid hyperplasia of the bone marrow was found especially in systemic autoimmune diseases (26.3%), hypersplenism (9.8%), preleukemia (7.3%) and acute leukemia (4.2%). The presence of excessive medullary LN could indicate a bone marrow microenvironment damage, possibly of autoimmune origin.
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PMID:Lymphoid nodules and nodular lymphoid hyperplasia in bone marrow biopsies. 393 2

A case in which polycythemia vera and chronic lymphocytic leukemia (CLL) developed during the course of idiopathic thrombocytopenic purpura (ITP) is described. Observations in this case suggest that ITP was a pre-monitor of the clinical development of CLL and preceded the manifestation of polycythemia vera. The polycythemia was mild, requiring infrequent phlebotomies, and, as the CLL progressed, the requirement for phlebotomy diminished. Evidence of both CLL and myeloid proliferation, as well as severe immune thrombocytopenia, persisted throughout her course. Studies on lymphocytes revealed characteristics of those of CLL. Excellent therapeutic response of the CLL was observed with the use of vinblastine and steroids.
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PMID:Development of polycythemia vera and chronic lymphocytic leukemia during the course of refractory idiopathic thrombocytopenic purpura. 636 8

We reviewed the literature concerning the history of determination of the ploidy of human megakaryocytes and its relationship with diseases. The ploidy of rabbit megakaryocytes was analyzed by microspectrophotometry in 1964, and the analysis of the ploidy in human megakaryocytes was first performed in 1968. Presently, microphotometry and flow cytometry are the primary methods for the evaluation of the ploidy, but they have their merits and demerits. In the ploidy of human megakaryocytes, a peak has often been reported at 16N in healthy individuals, and the next peaks have been observed at 32N and 8N. The results of ploidy analyses have been reported by many investigators to be comparable between patients with idiopathic thrombocytopenic purpura and normal subjects, but various shifts of the peaks have also been documented. The ploidy is often reported to shift to a larger ploidy class in polycythemia vera and essential thrombocythemia, but it has invariably been reported to shift to a smaller class in chronic myelogenous leukemia. In reactive thrombocytosis, the ploidy pattern was reported to be the same as that in normal individuals by some investigators but to shift to a larger ploidy by others. These differences are considered to be due to heterogeneity of the subjects. In myelodysplastic syndrome, the ploidy shifts mostly to a smaller class, but it may show various patterns. We also reviewed the ploidy in other rare hematological disorders, the relationships of the ploidy with diabetes mellitus and atherosclerotic disorders, and its changes in the ontogeny. Details of the mechanism of polyploidization and its biological significance remain unknown, and further advances in the studies of these topics are anticipated.
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PMID:Human megakaryocyte ploidy. 1050 38

We analyzed the efficacy of splenic irradiation in a population of patients with hematologic diseases. The records of the Radiation Oncology Division, Naval Medical Center San Diego were retrospectively reviewed for all patients treated with splenic irradiation (SI) between January 1, 1990 and March 1, 2001. The charts of 17 patients were identified: 5 patients had chronic myelogenous leukemia, 4 had chronic lymphocytic leukemia, 4 had idiopathic myelofibrosis, 2 had polycythemia vera, and 1 patient each had idiopathic thrombocytopenic purpura and acute myelogenous leukemia. Patient ages ranged from 37 to 88 years. Sixteen of 17 suffered from symptomatic splenomegaly. Twenty-six courses of splenic irradiation were delivered to these 17 patients. Treatment courses generally consisted of two fractions of 50 cGy in the first week, two fractions of 75 cGy the second week, and two fractions of 100 cGy the third week. Blood counts were checked prior to each treatment. Seven of the 17 patients died 1 month or less after SI due to the terminal nature of their disease. Twenty-two of 25 treatment courses for splenomegaly resulted in decreased pain and symptoms. Five patients required two treatment courses for splenomegaly, and one patient required five treatment courses. Three of four patients treated for thrombocytopenia demonstrated improvement, but only one was evaluable for more than 2 weeks due to disease-related mortality. Three of five patients treated for leukocytosis had significant improvement. In general, patients suffered few significant complications from this palliative intervention. Splenic irradiation can effectively palliate symptomatic splenomegaly in patients for whom splenectomy is not an option. Retreatment is possible. Splenic irradiation is less effective in the treatment of thrombocytopenia or leukocytosis.
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PMID:Palliative irradiation of the spleen. 1271 92

In hematological disorders, thrombocytopenia is frequently observed, and it is sometimes difficult to diagnose the underlying disease. In this symposium, laboratory tests for platelet abnormality were reviewed. Tests for platelet aggregation were reported to be important for the diagnosis of platelet dysfunction. Thrombocytopenia is caused by disseminated intravascular coagulation (DIC), thrombotic microangiopathy (TMA), heparin-induced thrombocytopenia (HIT), antiphospholipid syndrome (APS), idiopathic thrombocytopenic purpura (ITP), etc. As DIC is classified according to the degree of fibrinolysis, it was stated that the measurement of hemostatic molecular markers was further required. TMA is caused by abnormality of ADAMTS13, verotoxin, DIC, etc. HIT is diagnosed by anti-PF4 antibody, but its specificity is not high. Further investigation of TMA and HIT is required. APS is one of the most important diseases which cause thrombosis or abortion, suggesting that a differential diagnosis of APS is important. It was reported that diagnostic criteria of ITP have been established using a new antibody assay for platelets, immature platelet fractions, thrombopoietin, etc. In myeloproliferative disorders such as polycythemia vera and essential thrombocythemia, the mutation of JAK2 V617F was reported to be an important risk factor for thrombosis.
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PMID:[Summary of pathophysiology and diagnosis of patients with platelet abnormality]. 1952 56