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 22-year-old black male presented with erythrocytosis and proteinuria. The erythrocytosis was characterized by increased red cell mass, normal arterial oxygen saturation, and normal hemoglobin electrophoresis and oxygen affinity. There was no splenomegaly, and the white blood cell count, platelet count, serum uric acid concentration, serum B12 levels and leukocyte alkaline phosphatase activity were normal. Tumors of the liver, lung, kidney and cerebellum, which have been associated with erythrocytosis, were not found. The only associated disease was biopsy proven focal glomerulosclerosis. Renal vein thrombosis was excluded by renal venography and arteriography. This case illustrates the rarely reported association of the nephrotic syndrome and erythrocytosis. Other nephrogenic causes of erythrocytosis are mentioned, including renal cysts, tumors, renal artery stenosis and transplantation. The role of the kidney in erythropoietin production and possible mechanisms of nephrogenic erythrocytosis are discussed.
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PMID:Focal glomerulosclerosis and erythrocytosis. 50 18

Epidural myeloblastoma, which compressed the spinal cord, was the first evidence for chronic granulocytic leukemia, eosinophilic type, Ph chromosome negative. This manifestation was preceded by 3 years follow-up of a patient with persistent eosinophilia of 60% mature eosinophils. The only clues for the diagnosis of leukemia were splenomegaly and high serum vitamin B12, most of which was bound to transcobalamin I. The latter finding presents a useful diagnostic criterium in myeloproliferative disorders.
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PMID:Chronic eosinophilic leukemia complicated by epidural myeloblastoma. 80 41

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

Allogeneic immunocompetent T cells injected into chicken embryos induce a graft-versus-host reaction (GVHR) whose most prominent manifestation is splenic hyperplasia. The highly inbred CC and CB strains of chickens used here are, respectively, homozygous for the B4 or B12 MHC haplotypes. By means of a panel of immunological reagents, including alloantisera and monoclonal antibodies against public domains of the T-cell receptor, CD4, CD8, and the inducible interleukin-2-receptor light chain (CD25), it is shown that the bulk of cells in the enlarged spleen are of host origin and do not express markers typical of mature T or B lymphocytes. Among recipient splenocytes, the quantitatively most important population consists of TCR alpha beta-TCR gamma delta- CD4-CD8+CD25+ (TCR0) lymphocytes. Donor cells encountered in the spleen prevalently exhibit a TCR alpha beta+CD4+CD8-CD25+ phenotype and proliferate in vivo. The data demonstrate that nonspecific host and potentially specific donor-derived cellular elements contribute to splenomegaly.
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PMID:T-lymphocyte subsets in the embryonic spleen undergoing a graft-versus-host reaction. 182 95

A male patient, aged 78, was found with chronic neutrophilic leukemia (CNL). The patient showed sustained mature neutrophilic leukocytosis, splenomegaly, a high leukocyte alkaline phosphatase score, elevated serum vitamin B12 and uric acid, myeloid hyperplasia and absence of ph' chromosome in the bone marrow, with no evidence suggesting this condition to be a leukemoid reaction to an underlying disease. In addition to the above mentioned features, some functional characteristics of CNL cells were compared with normal cells. CNL is a very rare disease; some thought it as a variant of chronic myelogenous leukemia (CML). In this report a review of the literature is also included.
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PMID:Chronic neutrophilic leukemia--report of a case and review of the literature. 217 62

A case of blastic transformation of essential thrombocythemia (ET) is reported. A 69-year-old male was first admitted to hospital because of fever in February, 1982. He was diagnosed as having ET because of marked thrombocytosis (205.5 x 10(10)/1), absence of erythrocytosis, absence of splenomegaly, normal karyotype and no increment of blasts in the bone marrow, and normal levels of neutrophil alkaline phosphatase, vitamin B12 and folate. He was treated with busulfan, and subsequently his platelet count was well controlled for about five years. At the second admission, blasts were present in the peripheral blood, and later accounted for 49% of the total leukocyte count. Histological examination of a bone biopsy specimen showed homogeneous proliferation of blastic cells and slight reticulin fibrosis. At autopsy, the degree of bone marrow fibrosis had increased. This was considered to be a very rare case of ET with blastic transformation in the terminal phase.
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PMID:Blastic transformation of essential thrombocythemia. A case report. 258 80

We have analysed 139 consecutive cases (71 males and 68 females) of nutritional megaloblastic anaemia over a period of four and a half years. The majority of these patients belonged to the low socio-economic class and many of them were strict vegetarians. Sixty one percent were in the second and third decades of life. At the time of presentation, 46% had mild hepatomegaly, 42% fever, 34% mild splenomegaly and 20% bleeding manifestations. Of 102 cases in whom the biochemical parameters were available, vitamin B12 deficiency was detected in 76%, folate deficiency in 6.8%, combined B12 and folate deficiency in 8.8%; the remaining 7.8% had normal vitamin levels at presentation. All 139 patients had severe anaemia, 80.5% had thrombocytopenia and 43.8% had neutropenia as well as thrombocytopenia. It appears that during progression (in terms of duration) of megaloblastosis, anaemia is followed by thrombocytopenia and then neutropenia. Infection and bleeding in these patients may be aggravated by impaired functions of neutrophils and platelets, respectively.
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PMID:Pancytopenia in nutritional megaloblastic anaemia. A study from north-west India. 263 48

Forty patients with hypereosinophilic syndrome (HES), defined according to the criteria established by Chusid et al., were studied retrospectively in order to determine the prognostic factors in this heterogeneous disease. A myeloproliferative syndrome (MPS) was diagnosed 17 times when splenomegaly and/or a greater than 5 times the normal vitamin B12, level existed. Cutaneous-visceral involvement was almost always present (95%), but could appear after several years of evolution. Twenty-three patients had cardiac involvement, determined by clinical, radiological, electrocardiographic and echocardiographic examinations; 12 of these had endomyocardiac fibrosis. The overall survival rate was 80% at 5 years and 42% at 10 and 15 years. Among the factors evaluated that are capable of influencing this survival, 5 were found to impair the prognosis; presence of an MPS; non-response of the hypereosinophilia to corticoids; existence of a cardiopathy; being male; and an elevated maximum eosinophilia; the last two factors were significantly associated with the cardiopathy. Because hematological or cardiac anomalies are not always present at the time of the first examination, HES patients require very close surveillance.
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PMID:[Prognostic factors of hypereosinophilic syndrome. Study of 40 cases]. 267 14

Twenty-seven consecutive patients with previously untreated, or minimally treated benign phase Philadelphia-chromosome-positive, chronic myelogenous leukaemia (CML) were treated with partially purified human leucocyte (alpha) interferon; 24 of the 27 patients responded to therapy achieving either haematological remission (20 patients) or partial haematological remission (four patients). In the responding patients the peripheral white blood cells declined from a median of 89.6 X 10 X 10(9)/l to 4.5 X 10 X 10(9)/l. The serum lactate dehydrogenase declined from a mean of 8.36 Katal/l (492 mu/ml) to 2.8 Katal/l (165 mu/ml), and the vitamin B12 levels declined from 1492 pg/ml to 838 pg/ml. Fifteen patients had splenomegaly. The spleen size normalized in four and decreased by a median of 30% in 10 additional patients. The bone marrow cellularity fell from a median of 100% to a median of 62%. In seven of the 24 responding patients, followed for greater than or equal to 6 months, the percentage of Ph1-positive cells in the bone marrow declined to a median of 70% (range 5-75%). Alpha interferon was found to be an effective therapeutic agent for controlling the myeloid proliferation in CML, and in partially restoring the nonclonal haematopoietic cells in some of the patients.
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PMID:Chronic myelogenous leukaemia: haematological remissions with alpha interferon. 346 63

The prognosis of the hypereosinophilic syndrome (HS) depends mainly on the development of endomyocardial fibrosis (EMF). This complication may be overlooked at an early stage, although its presence is an indication for steroid or antimitotic therapy of the HS. Even at an advanced EMF and associated intracardiac thrombi may not be visualised by angiography. This study was undertaken to assess the diagnostic value of 2D echocardiography in 12 patients. The patients were all men (12 of them) aged 22 to 64 years with unexplained eosinophilia 1 500/mm3 for over 6 months, and visceral lesions. The patients were divided into 3 clinical groups. Group A comprised 4 "allergic" patients with chronic asthma and a significant elevation of IgE; Group B comprised 5 "myeloproliferative" patients with splenomegaly and/or hepatomegaly and a significant elevation of serum B12 levels. The 3 remaining patients who could not be allocated to either Group A or B formed the third group (Group C). 2D echocardiography was carried out on average 30 months after diagnosis of the HS and six planes of examination were used systematically (two parasternal, two apical, one extreme apical and one subcostal). Right and left ventriculography was performed in 6 patients (less than one month before or after 2D-echo). Anatomical studies were obtained in 4 cases (2 operations, 3 autopsies). Echocardiographic signs of EMF were observed in 8 cases. Four patients had a restrictive cardiopathy associated to a large LV thrombus in 2 cases.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cardiac manifestations of the hypereosinophilic syndrome. The value of 2-dimensional echography (12 cases)]. 643 27


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