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)

Ten clinical observations concerning six families with familial myeloproliferative disorders are reported. Family no. 1 : two brothers, RES with myelosclerosis and ROS with chronic myeloid leukemia. Family no. 2 : PG atypical myeloproliferative syndrome and his brother polycythemia vera. Family no. 3 : DF myelosclerosis and her son (DR) polycythemia vera. Family no. 4 : DM, polycythemia vera, the mother and a sister with splenomegaly. The brother died with myelofibrosis. Family no. 5 : GA and ML, cousins with polycythemia vera. Family no. 6 : MB and ZG, a brother and sister with polycythemia vera. No consanguinity and no toxic, infections or malignant etiology were found in these families. The literature reviewed emphasises the rarity of the familial incidence of myeloproliferative disorders.
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PMID:[Familial myeloproliferative syndromes. Study of 6 families and review of literature]. 35 25

Polycythemia developed in progeny from mothers who were exposed during pregnancy to a combination of methylmercury chloride plus ethylurea and sodium nitrite. The polycythemia occurred as early as one month of age and as many as 24% of the offspring developed the polycythemic condition. Many features of this condition are similar to those of polycythemia vera in man, such as elevated hematocrits and white and red blood cell counts, splenomegaly, and hyperplasia of bone marrow accompanied by megakaryocytosis.
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PMID:Polycythemia produced in rats by environmental contaminants. 47 69

We have identified partial trisomy 1q in 2 patients with different hematologic disorders. The first patient was a 55-year-old female with myelosclerosis and myeloid metaplasia diagnosed at age 38 years presenting with anemia, fatigue, bruising, fever, and splenomegaly. At age 56, she had 50--95% myeloblast cells and 95--100 nucleated RBC precursors per 100 WBC. Chromosome analysis of unstimulated leukocytes with Q, G, and C banding showed 46,XX,-6,+t(1;6) (q25;p22) in all metaphase cells. In vitro incorporation of Fe55 was demonstrated 90% of metaphases by autoradiography. The second patient, a 49-year-old male, was diagnosed as having polycythemia vera at age 30 during a regular checkup. He since developed hepatosplenomegaly. Chromosome analysis from a direct bone marrow preparation at age 44 and 45 showed grossly normal karyotypes. At age 49, his marrow by Q and G banding showed almost 100% of cells with 46,XY,-13,+t(1;13) (q12;p12). Eleven cases of trisomy of 1q have been reported in various hematologic disorders. It is apparent that partial trisomy 1q represents another nonrandom chromosomal abnormality, in addition to the most common nonrandom chromosomal aberrations, such as the Philadelphia chromosome, trisomy 8, trisomy 9, and monosomy 7 in hematologic disorders.
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PMID:Partial trisomy of the long arm of chromosome 1 in myelofibrosis and polycythemia vera. 60 27

In order to study the effect of oxymetholone therapy in advanced myelofibrosis, 11 patients (4 females, 7 males) were given, 3--5 mg per kg body weight, long-term oxymetholone treatment in a prospective multicenter study. Five cases had previously had a diagnosis of polycythemia vera. All patients had anemia initially, 4 leukocytopenia and 10 thrombocytopenia in addition. Hepato-splenomegaly was present in all cases but in varying degree. Five patients required regular blood transfusions before treatment. In 9 of the 15 courses given, there was normalization of the peripheral blood or substantial improvement (better than 3 g hemoglobin/dl or 50 X 10(9) platelets/1) after androgens. Significant effects were noted both on hemoglobin values and platelet counts. The need for blood transfusions ceased completely in all 5 cases. When oxymetholone treatment was reduced or interrupted 4 patients relapsed; 2 of them responded to a renewed course. The red cell counts returned to previous polycythemic values in one patient and another died from acute leukemia. The results of this study suggest that androgens might be of value in advanced cases of myelofibrosis with transfusion-requiring anemia or severe thrombocytopenia.
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PMID:Oxymetholone treatment in myelofibrosis. 66 66

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

Forty-three operative procedures were performed on a population of 250 patients with myeloproliferative disorders, including polycythemia vera, myeloid metaplasia (MM) and chronic myelogenous leukemia (CML). The overall operative mortality was approximately 7% and the incidence of excessive bleeding which could be related to coagulopathy was 5%. Twenty-one patients with MM or CML underwent splenectomy for palliation of symptoms related to the enlarged spleen or hematologic problems. Eighty-four percent of the latter group were improved. Adverse hematologic effects which could be attributed to splenectomy in these patients were confined to two patients who developed marked thrombocytosis. Among the 23 patients with MM, 9 had portal hypertension. Three underwent portacaval shunt and one a splenorenal shunt for bleeding varices. One of the patients died of hepatic necrosis. Estimated hepatic blood flow determinations (EHBF) in 4 patients with portal hypertension demonstrated a marked absolute increase and an increase in the ratio of EHBF/Cardiac Index. Absence of any evidence of intrahepatic or extrahepatic obstruction in these patients and the demonstration that splenectomy relieved portal hypertension defined at surgery in 4 patients, suggests that augmented adhepatic flow contributes to portal hypertension in some cases. The review leads to the conclusions that: 1) Operative procedures in prepared patients with myeloproliferative disorders are not associated with prohibitive mortality and morbidity rates. 2) Splenectomy is indicated for patients with increasing transfusion requirements and symptomatic splenomegaly or hypersplenism and should be performed early in the course of disease. 3) When associated portal hypertension and bleeding varices are present, hemodynamic studies should be carried out to define if splenectomy alone, or a portal systemic decompressive procedure is indicated.
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PMID:Myeloproliferative disorders. 105 48

A male, 66, developed Acute Erythremic Myelosis in the course of Polycythemia Vera. The time of onset of Polycythemia Vera could not be determined, his first symptoms being vascular complications. He received treatment with Phlebotomies and Myleran. Five years later he became ill with malaise, fever, splenomegaly; in the peripheral blood profound pancytopoenia with immature, bizzare erythro-and megaloblasts have been found. Bone marrow was full of atypical megaloblasts, some of them having two or more nuclei. The number of mitoses was increased. Chromosomal abnormalities consisted of ane-uploid cells, chromatid breakes and translocations (G to A-1). The therapy with B12, Cytosin-Arabinoside, Oncovin and Blood transfusions was unsuccessful. He died 21 days after being admitted to the Hospital.
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PMID:[Acute erythremic myelosis as a terminal phase of polycythemia rubra vera]. 106 69

Measurement of blood volume is an important clinical tool in establishing a diagnosis as in polycythemia vera, in assessing the true significance of a low blood count in a patient with splenomegaly, and in evaluating a bleeding patient. In theory, blood volume measurements should be of great value in treating acute blood loss, but this is often too time-consuming to be practicable. The main value of blood volume studies so far has been in clinical research where it is essential for correct interpretation of the peripheral blood measurements. Estimates using the hematocrit (packed cell volume) could be misleading since this measurement does not take into account total blood volume and changes in the plasma volume. A variety of isotopes that tag the red blood cell have been used to measure total blood volume and red cell mass. Most commonly used at present is autologous 51-Cr labeled red cells, thus eliminating the risk of hepatitis in transfusion to the subjects. Radio labeled albumin and other plasma proteins were used to measure plasma volume. A large molecular protein is ideal to eliminate overestimation from extravascular diffusion of labeled material for accurate determination of plasma volume. Physiologic changes in the newborn, in pregnant women, during exercise, bed rest, and altitude in the nonpathologic state are discussed. Studies in the normal subject's adaptation in different physiologic conditions have revealed a variety of factors that regulate blood volume.
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PMID:Physiological changes in blood volume. 109 3

A 57-year-old man, diagnosed as Polycythemia vera (PV), had been treated with administrations of Busulfan since 1984. Three years later, the number of neutrophils in peripheral blood increased to 50,000/microliters with progression of splenomegaly, and the case was diagnosed as Chronic neutrophilic leukemia (CNL) based on the criteria by Miura et al, in November, 1989. In spite of 6MP and Busulfan therapy, marked neutrophilia and splenomegaly progressed, and the patient died due to liver dysfunction in June of 1991. To clarify the pathophysiology of PV and CNL, we studied the in vitro growth kinetics of hematopoietic progenitor cells in bone marrow of this unique case and made a comparison with those of 4 cases of PV and 4 normal volunteers employing methylcellulose culture. As in other cases of PV, erythroid colonies were formed in culture of bone marrow from this patient without addition of erythropoietin. Furthermore, spontaneous colonies derived from CFU-GM and CFU-Mix increased remarkably in this case only. The results suggest that the hematopoietic abnormalities in this case involve the multipotent stem cells as well as erythroid and granuloid-macrophage progenitors.
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PMID:[Polycythemia vera terminating in chronic neutrophilic leukemia: studies on in vitro growth of hematopoietic progenitor cells]. 147

Polycythemia vera (PV) is one of the myeloproliferative diseases, and, as such, is an example of clonal hematopoiesis. The progeny of a single, abnormal, hematopoietic stem cell gain a growth advantage over their normal counterparts resulting in overproduction of red cells generally accompanied by overproduction of granulocytes and platelets as well. There are a variety of nonspecific symptoms at onset related to the increased red cell mass and hematocrit accompanied by the more specific manifestations of pruritus, erythromelalgia, and hepatic, portal, and mesenteric vein thrombosis. Splenomegaly and hypertension are common. The laboratory hallmark is an increased red cell mass. There is also often an increase in white cell count, platelet count, and leukocyte alkaline phosphatase along with other findings reflecting the increased rate of turnover of hematopoietic cells. The bone marrow biopsy generally displays hypercellularity involving all three cell lines and absent iron stores. The diagnosis of PV depends on excluding spurious polycythemia in which there is a high hematocrit but a normal red cell mass and secondary polycythemia in which there is an increased red cell mass in response to tissue hypoxia or the inappropriate production of erythropoietin, generally by a tumor. In addition, one should try to establish the diagnosis in a positive fashion by a combination of studies of the blood and bone marrow. Phlebotomy and occasionally plateletpheresis should be used as acute therapy. Chronic therapy is guided by the knowledge that patients treated with phlebotomy alone have an increased rate of thrombotic complications particularly in older patients and those with previous thrombotic disease. Myelosuppressive therapy can reduce the incidence of these complications, but is commonly associated with an increased incidence of second malignancies, particularly acute leukemia. At present, hydroxyurea is the myelosuppressive agent of choice. Antiplatelet agents have a limited role except in the palliation of the syndrome of erythromelalgia. Median survival is approximately 10 years. As implied above, the causes of morbidity and mortality vary with the mode of chronic therapy which has been employed, leukemia being more common after myelosuppressive therapy and thrombotic complications being more common after therapy with phlebotomy alone. Ten percent to 50% of patients move into a spent phase followed by postpolycythemic myeloid metaplasia, irrespective of previous therapy employed. Eventually, the major problems may be cytopenias and massive splenomegaly.
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PMID:Polycythemia vera. 158 7


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