Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A group of 52 consecutive patients with polycythemia vera was submitted to long-term therapy with radioactive phosphorus (32P). Initial phase of therapy induced complete remissions (normalization of hematogram; spleen not palpable) in 45% of the patients, and partial remissions in the remaining 55%. During maintenance therapy of the complete remission group, mean remission time was about 3.5 years. Individual remission times ranged between 1 and 6 years. In the group of patients with incomplete remission, mean remission time increased with the progression of the disease due to gradual development of "spent" -polycythemia. In patients with splenomegaly, remission time was negatively correlated to spleen size. In both groups the increment of annual accumulated dose averaged 2.4 mCi 32P. When considering polycythemia related causes of death only, mean survival time attained 12 years after initial treatment with 32P. Acute leukemia occurred in 2 patients (4%).
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PMID:32P-therapy in polycythemia vera. 80 69

Three hundred and three cases of polycythaemia vera were treated between 1949 and 1961 using radioactive phosphorus, the minimum follow-up for the patients in the group being 12 years and the maximum 24 years. Two hundred and thirty three patients died, the median duration of survival after the first treatment with phosphorus being 10 years (i.e. 12 years after the diagnosis was made). 59 patients died of the vascular complications of polycythaemia, 76 of leukaemia or myelofibrosis. The total number of deaths due to vascular complications up to the tenth year exceeded the total number of deaths due to haematological complications (leukaemia or myeloid metaplasia). At the end of the 11th year the opposite was true. From the ninth year onwards, acute leukaemia and myelofibrosis represent more than 40 p.cent of deaths of known cause and the annual probability of death from a haematological cause for the surviving patients increases regularly until the fifteenth year when it reaches approximately 5 p.cent of the patients at risk. However the median survival of patients dying from acute leukaemia or myeloid splenomegaly is slightly longer than that of patients dying from other causes, this confirming that these disorders would appear to represent the terminal phase in the course of polycythaemia vera.
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PMID:[Fate of polycythemia vera treated by radioactive phosphorus. Apropos of a series of 303 patients followed for 12 to 24 years]. 116 78

The univariate analysis of prognostic factors showed the value of clinical variables (age, symptoms, lymph node enlargement, splenomegaly, hepatomegaly, clinical stage), haematological variables (haemoglobin, platelet count, leucocyte count, lymphocyte count, bone marrow involvement and biopsy pattern, cleaved lymphocytes, prolymphocytes, LDT) and biochemical variables (BUN, creatinine, calcium, phosphorus, uric acid and albumin). The multivariate analysis chose the combination of Rai's stage, age, cervical lymph node involvement, phosphorus and BUN. Two predictive models capable of separating appropriately low, intermediate and high risk groups were developed and validated. The results were compared with others in the literature. The interest of predictive models arising from multivariate analysis is stressed.
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PMID:[Chronic lymphatic leukemia. II. Analysis of prognostic factors and development of survival predicting models. Study of 187 patients]. 177 7

Large granular lymphocyte (LGL) leukemia was induced in 40 F344 rats by inoculating them with neoplastic cells to evaluate the effect of acute leukemia on bone remodeling and calcium balance. The rats developed leukemia and splenomegaly by 9 days after inoculation. The rats had reduced body weight (day 12), food intake (days 4, 8, 12), urine production (day 12), and fecal output (day 12). Serum calcium and phosphorus and urinary excretion of calcium and phosphorus were decreased on days 8 and 12 in leukemic rats. Static bone histomorphometry of trabecular bone in lumbar vertebrae demonstrated reduced bone area, no change in the number of osteoclasts, and reduced osteoclast perimeter at day 12. Dynamic bone histomorphometry revealed reduced double labeled perimeter, mineralizing perimeter, trabecular mineral appositional rate, and bone formation rate in rats with LGL leukemia at days 9 and 12. There was no change in periosteal mineral appositional rate. Rats with leukemia and intramedullary neoplastic cells had a reduction in bone formation rate that resulted in a loss of trabecular bone.
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PMID:Effects of large granular lymphocyte leukemia on bone in F344 rats. 227 27

A 43-year-old man with hairy-cell leukemia and marked splenomegaly developed severe hypophosphatemia which improved after splenectomy. Since splenic tissue phosphorus was significantly elevated, and since serum phosphorus returned to normal levels immediately after the operation, it is postulated that excessive uptake of phosphorus by the rapidly dividing leukemic cells might have caused the transient decrease in serum phosphorus.
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PMID:[Transient hypophosphatemia associated with hairy-cell leukemia]. 231 8

Image localized 31P magnetic resonance spectroscopy of the spleen was performed in six normal volunteers, 13 patients with splenomegaly due to haematological malignancies, and two patients with benign causes of splenomegaly. The malignant disorders had elevated phosphomonoesters (PME) compared to controls, probably due to increased turnover of membrane phospholipids, with variable alterations in high energy phosphates. There appeared to be no relationship between grade of malignancy and relative PME peak area. An overlap in spectral characteristics between the benign and malignant cases of splenomegaly existed. Serial studies in a patient with high grade lymphoma receiving combination chemotherapy showed changes in phosphorus spectra, with an increase in the phosphodiester/beta adenosine triphosphate ratio.
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PMID:Characterization of the spleen by in vivo image guided 31P magnetic resonance spectroscopy. 264 99

Over 20 years, 58 cases of PV in young people (46 meeting the full PVSG criteria, 12 with elevated red cell volume and leucocytosis or thrombocytosis, without splenomegaly) were studied and have been followed for periods of 3-24 years. These cases represent approximately 5% of the cases of PV referred to the Department of Nuclear Medicine of St Louis Hospital during this period. They differ from older patients in the initial clinical severity, the short interval between the first symptoms and the diagnosis, frequent presentation with a life-threatening complication (two cases of hepatic vein thrombosis, six thrombotic or haemorrhagic events, six splenectomies, two abortions) and a very enlarged spleen in half the cases. However, after the initial complications, the overall survival is very long (exceeding 70%, even when including the initial complications, at 15 years). The vascular accidents occur exclusively in the phlebotomized patients, the main risk factor being the poor stability of the haematocrit. Only one acute leukaemia was observed among the 14 cases treated by radioactive phosphorus and/or alkylating chemotherapy. The most frequent late complication was evolution towards myelofibrosis. This spent phase seemed to occur earlier in patients treated by phlebotomy. On the basis of this data, we would advise the following therapeutic strategy: phlebotomies, as soon as the diagnosis is established, and a systematic long-term treatment by hydroxyurea with the hope of reducing the number of vascular complications and of delaying the evolution towards the spent phase and the myelofibrosis.
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PMID:Polycythaemia vera in young people: an analysis of 58 cases diagnosed before 40 years. 368 94

A patient had extensive foreign-body granulomatous inflammation of multiple skin sites and of the inguinal lymph nodes with splenomegaly, cutaneous anergy to common skin antigens, and peripheral blood eosinophilia. The patient had an elevated serum angiotensin-converting enzyme level. Histologically, the granulomas were of the foreign-body type with lymphocytes, histiocytes, eosinophils, and giant cells, some that contained doubly refractile crystalline material. Electron-probe x-ray microanalysis identified silicon, magnesium, iron, calcium, phosphorus, zinc, titanium, and chromium in the crystalline material. These findings suggest talc, cement, and inorganic pigment as possible sources of the crystals. This case is reported for its unusual clinical, laboratory, and morphologic features.
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PMID:Widespread foreign-body granulomas and elevated serum angiotensin-converting enzyme. 629 13

The 61 observations of primary thrombocythemia described in this report represent approximately 15% of the cases of polycythemia vera recorded by the authors over the same 18-year period. The group includes 35 females and 26 males, with a mean age of 62. The disease is usually discovered on routine blood tests (half of cases), and more rarely because of hemorrhagic or thrombotic manifestations. Splenomegaly is found in one-third of cases. Platelet count is permanently above 800 X 10(9)/l (mean : 1 500 X 10(9)/l); mild hyperleukocytosis (mean : 16 X 10(9)/l) with predominant neutrophil polynuclears is usual but myelemia is not constant (28% of cases) and always very moderate; red cell parameters are normal in three-fourths of cases, while the remaining patients have anemia, either due to iron depletion or not. Reticulinic myelofibrosis, usually minimal, is found in 40% of cases. Medullary karyotype is always normal, without chromosome Ph1. Platelet functional abnormalities are not constant and do not correlate with the magnitude of thrombocythemia. 51 patients (84%) received myelosuppressive therapy, mainly by busulfan or radioactive phosphorus. Most deaths were due to intercurrent causes and only one patient developed acute leukemia. 71% of patients are alive at five years and subsequent decrease in the actuarial survival curve is very gradual.
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PMID:[Essential thrombocythemia. Clinical, biological study and developmental study of 61 cases]. 632 10

A 7-year-old, crossbred, desexed bitch had a fluctuating illness of more than 6 months duration, with signs of inappetence, reduced exercise tolerance, restlessness, panting, black diarrhoea, and red eyes and ears. Haematological examination disclosed persistent polycythaemia, reticulocytosis, normoblastaemia , and normal total plasma protein concentration when not dehydrated. Splenomegaly was present, but there was no evidence of significant pulmonary, cardiac or renal disease on physical, radiographic or electrocardiographic examination. Further investigations revealed normal arterial Po2 and oxygen saturation, and increased total erythrocyte volume. Primary polycythaemia was diagnosed. The dog was rendered anaemic by bleeding, then treated with a single injection of radioactive phosphorus. The patient has had no recurrence of signs and remains well 50 months later.
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PMID:Primary polycythaemia in a dog. 673 71


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