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)

Only a fraction of the patients with myeloid metaplasia have indications for splenectomy develop. Palliation for symptomatic splenomegaly is highly successful. Significant relief from hypersplenism, anemia or thrombocytopenia was achieved, however, in less than half of the patients. Since patients with severe thrombocytopenia are at continuing risk of spontaneous hemorrhage and of bleeding after minor trauma, splenectomy appears to be justified in these patients, provided that studies of peripheral blood and bone marrow have excluded those with leukemic conversion. The benefit obtained from splenectomy in patients with chronic anemia requiring frequent transfusions in less certain, particularly in men and in patients converting to myeloid metaplasia from polycythemia vera. In some instances, the continuation of transfusions may result in less morbidity and better palliation than operation. Although the course of this myeloproliferative disorder from first symptoms to death frequently extends for more than a decade, the manifestations which may provide an indication for splenectomy usually occur late in the course of the disease. The element as well as the morbidity of the operation makes a thorough evaluation of each patients essential before splenectomy is considered.
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PMID:Splenectomy for agnogenic myeloid metaplasia. 706 91

The primary polycythemias result from malignant proliferation of myeloid stem-cell. Typically, an increase of red cell mass and a decrease of erythropoietin is found. In polycythemia vera, augmentation of PCV is frequently associated with elevation of WBC and platelets, as well as splenomegaly. The treatment consists of venosection and administration of P32 or cytostatics; all of which methods exhibit a specific risk. In secondary polycythemias, augmentation of red cell mass is consecutive to increased erythropoietin production; these hypererythropoietinemias may be induced by hypoxia or, rarely, may result from an inappropriate tumoral (malignant or benign) secretion. "Spurious" polycythemias are finally defined by the more or less normal red cell mass. They are divided into three groups: micropolycythemias, relative polycythemias and "spurious" chronic polycythemias. The latter are frequent and exhibit relatively important morbidity and mortality, and therefore the recently proposed new therapeutic approaches should be considered. Tobacco addiction appears to be one of the major causes of these "spurious" polycythemias.
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PMID:[The polycythemias]. 728 Jun 28

A patient with polycythemia rubra vera and myeloid metaplasia required splenectomy because of local symptoms attributable to a massively enlarged spleen. Preoperative embolization of the splenic circulation with inert materials was performed to reduce splenic size and vascularity in order to permit safe splenectomy. This case illustrates the utility of preoperative splenic embolization when routine splenectomy is contraindicated for technical reasons. The hazards of this technique and guidelines for its clinical use are outlined.
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PMID:Splenic artery embolization prior to splenectomy in end-stage polycythemia vera. 739 59

In an autopsied female primary polycythemia was definitely diagnosed only after histological examination. A classical form of Vaquez-Osler disease was characterized by pronounced normo-, granulo- and megakaryocyte hyperplasia of the bone marrow, spleen and liver myelosis with an admixture of atypical megakaryocytes and splenomegaly. There was a complication in the form of two coronary arteries thrombosis. Lethal outcome resulted from myocardial infarction.
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PMID:[Polycythemia vera, complicated by myocardial infarction]. 767 90

The clinical course of 16 patients with polycythemia vera (PV), treated in the period 1982 to 1993, was shown. Splenomegaly occurred in three fourths of these patients (75%), and hypertension was a major symptom. Thrombosis such as myocardial infarction and cerebral infarction was noted. Eight patients was treated with myelosuppressive agents and the 8 other patients were treated with phlebotomy. A 70-year-old male who was treated with mitobronitol (DBM) developed acute myeloblastic leukemia (AML) 11 years later. He was treated with multi-combination chemotherapy (BHAC-DMP), and entered complete remission, followed by early relapse. He became refractory to chemotherapy and died of acute pneumonia 6 months later. Median survival of 16 cases of PV was more than 10 years, and long-term treatment and care are necessary.
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PMID:[A clinical study of sixteen polycythemia vera cases--acute myeloblastic leukemia in patients with polycythemia vera]. 799 24

With the very long life expectancy of Polycythaemia Vera late complications are often observed: progressive resistance to treatment, bad tolerance to maintenance by phlebotomy, progression towards myelofibrosis. Resistance to phosphorus 32 is reflected by a progressive reduction in the duration of remission and by a gradually decreasing remission rate. A complete resistance appears after a mean duration of disease of 7 years. In the maintenance treatment by hydroxyurea, there is a secondary resistance in one third of cases with a poor control of the excess platelets. Resistance to Pipobroman is less frequent (10%). The phosphorus resistance could be delayed by addition of maintenance treatment by Hydroxyurea. In the presence of resistance to 32 P, Hydroxyurea and Pipobroman often remain effective. In the case of resistance to Hydroxyurea or Pipobroman, we have several possibilities: inversion of chemotherapy, other chemotherapy as Busulfan, 32 phosphorus. Intolerance of phlebotomy as baseline treatment is almost constant. Three complications lead to discontinuation of phlebotomy: development of cardiovascular complications, raised platelet count to above 800,000 and often more than 1 million, progressive increase in the size of the spleen with appearance of signs of myeloid splenomegaly. Exclusive phlebotomies are not indicated as baseline treatment of Polycythaemia Vera. The progression towards myelofibrosis (spent phase, post polycythaemia myeloid splenomegaly) increases with the duration of the disease and the frequency of transformation differs according to the type of treatment. The time to transformation is much shorter in the patients treated by phlebotomy. The transformation towards myelofibrosis is demonstrated by bone marrow biopsy and isotope investigations (bone marrow scintigraphy and kinetic by iron 59 and chromium 51).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Clinical and laboratory assessment and therapeutic problems in longstanding polycythaemia vera. 803 42

Familial chronic myeloproliferative syndrome (CMS) was observed in five members from two different generations of the same kindred. Diagnosis included agnogenic myeloid metaplasia (case 1), polycythemia vera (case 2), and essential thrombocythemia (cases 3-5). Cases 1-3 were siblings, case 5 was the daughter of case 1, and case 4 was the cousin of cases 1, 3. Age at diagnosis ranged from 28 to 75 years, cases 1 and 3 were male, and the others were female. The diagnosis was made after an episode of cerebral thrombosis in one patient, during a study for headache and dizziness in another, and fortuitously in the three remainders. All patients had splenomegaly and varying degrees of thrombocytosis. The cytogenetic exam was normal in all four cases. A woman patient was treated with interferon during a pregnancy. Fetal growth was retarded, and the newborn showed bone and genital malformations. No environmental leukemogen factor was found. This familial case strengthens Dameshek's theory of a common pathogenesis of CMS and suggests a genetic and hereditary etiology.
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PMID:Familial myeloproliferative syndrome. 819 53

We studied the effects of recombinant alpha 2-b interferon (alpha 2-b IFN) in a dose of 3 x 10(6)U intramuscularly three times a week for 1 year in 13 patients affected by polycythemia vera (PV) previously treated with phlebotomy only. Response to treatment was evaluated by reduction of the number of phlebotomies required to retain normal hematocrit value. Ten out of 13 patients (77%) responded to treatment; in 4 of them the exigency of phlebotomy was completely eliminated. In all responders a concomitant decrease of platelet count and splenomegaly was obtained. Treatment was well tolerated and side effects were easily controlled. We conclude that alpha-IFN may represent an attractive therapeutic option in the management of the proliferative stages of PV.
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PMID:Recombinant interferon alpha-2b in the treatment of polycythemia vera. 821 63

The present report is based on an analysis of the evolution of 720 cases of Polycythaemia vera treated with pipobroman and 624 cases treated with hydroxyurea. General modes of treatment are identical for the two drugs, consisting of initial therapy at relatively high dose aimed at obtaining complete remission and maintenance therapy essential to conserve the improved clinical status. Both types of treatment must be adapted to suit the patient. Complete remission is achieved in 95 to 100% of cases with pipobroman and in 80 to 90% of cases with hydroxyurea. Incidents which may occur during initial therapy include cytopenia, more frequent and severe under treatment with hydroxyurea, rare transitory digestive troubles and cutaneous and mucous eruptions. Subject to control of the blood cell count every three to four months, maintenance therapy may be continued for many years and while the time lapse is as yet insufficient for hydroxyurea, resistance to pipobroman does not appear to develop even after more than 20 years of treatment. Although neither of these two drugs entirely avoids the occurrence of acute leukaemia which appears in 5 to 8% of subjects irrespective of the duration of therapy, on the contrary to observations in patients treated by bleeding alone, myeloid splenomegaly with myelofibrosis is rare and develops in no more than 2% of cases. The frequency of visceral cancers is not increased by either drug. Provided Polycythaemia vera is maintained in complete remission, thrombotic accidents occur no more often than in a normal population of the same age bracket.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Indications, procedure and results for the treatment of polycythaemia vera by bleeding, pipobroman and hydroxyurea. 829 23

Diagnosis of chronic myeloproliferative disorders (CMPD) can encounter difficulties due to overlaps and possible transitions between the different entities and their similarity to reactive myeloproliferations. In this study DNA analysis has been applied to improve differentiation of CMPD. All subtypes of CMPD analyzed, including chronic myeloid leukemia (CML), agnogenic myeloid metaplasia (AMM), polycythemia vera (PV), and essential thrombocythemia (ET), had in common that granulocytes and bone marrow cells were clonal in origin as shown by X-chromosome-linked DNA polymorphism in conjunction with methylation patterns. Reactive myeloproliferations, by contrast, revealed a polyclonal inactivation pattern. Clonality could not distinguish CMPD from cases of myelodysplastic syndrome (MDS) since the latter also exhibited clonal hematopoiesis. AMM belongs to the group of myeloproliferative syndromes. Up to now the cellular phase at onset of the disease (megakaryocytic myelosis) has not been analyzed for clonality of the hematopoietic cells. Granulocytes as well as bone marrow cells from the cellular phase and advanced stages of the disease revealed a monoclonal inactivation pattern of X-chromosomal genes. These results show that the cellular phase already represents a monoclonal, and hence probably a neoplastic, proliferation of a pluripotent stem cell. The monoclonality of granulocytes could also be demonstrated in patients with splenomegaly and strongly argues against a compensatory proliferation of regular hematopoiesis in this organ. Because of their clonal origin, peripheral granulocytes were used in all cases (n = 244) for the detection of bcr-gene rearrangement. Despite possible morphological overlaps between different types of CMPD, bcr-gene rearrangement proved to be specific for CML and could be applied to differentiate CML from other CMPD in cases of uncertain morphological diagnosis. It is concluded that CMPD represent clonal hemopoietic disorders that probably have specific underlying genetic defects. Thus, DNA analysis can substantially aid in the differential diagnosis of CMPD.
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PMID:[Histopathology and molecular pathology of chronic myeloproliferative disorders]. 837 86


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