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

Sixty-one patients with essential thrombocythemia (ET) were followed from 1974 through 1987 at the Medizinische Poliklinik. Fifty-one patients (84%) presented with thromboembolic complications, and eight patients (13%) with hemorrhages. In seven patients (12%), a thrombocytosis was detected accidentally. Disturbances of the microcirculation (67%), mainly of the fingers and toes (53%), were the most frequent thromboembolic symptoms. The mean age of all patients was 58 years (male patients, 61 years; female patients, 56 years). The average platelet count at diagnosis was 897,000/microliter. The average maximal platelet count was 1.231 X 10(6)/microliter (range, 500,000/microliter to 4 X 10(6)/microliter). Seventy-two percent had a moderate leukocytosis (average, 12,400/microliter), 34% a splenomegaly, 29% a hepatomegaly. Signs of hypermetabolism were infrequent, lactate dehydrogenase (LDH) and uric acid elevations, if present, were moderate. Bleeding time and viscosity were normal in most patients. Spontaneous platelet aggregation was increased in 81% of patients (n = 40). Platelet aggregation studies with the aggregation inducing substances adenosine diphosphonate (ADP), platelet activating factor (PAF), thrombin, collagen, and adrenalin showed hypoaggregation in most patients. Adrenalin-induced aggregation distinguished best between ET-patients and reactive thrombocytosis showing hypoaggregation in all ET-patients tested (n = 16) and in none of 22 controls. Bone marrow studies were performed in 57 patients. The histologic studies (done in 49 patients) were consistent with a chronic myeloproliferative disorder in all cases. In 41 cases (84%) the picture of a megakaryocytic myelosis was found, in 12 of these a granulocyte-rich form of megakaryocytic myelosis. Cytologic studies only (eight patients) did not differentiate ET well from reactive thrombocytosis. Platelet aggregation studies and bone marrow histology may be of help in the diagnosis of difficult cases of thrombocytosis. The Philadelphia status was negative in all cases studied (14 patients). Fourteen patients died. The causes of death were thromboembolic complications in probably 11 and acute leukemia in two patients. The probability of 10-year survival is 64% after a mean follow-up time of approximately 5 years. It appears that considering the average age of ET patients at diagnosis, life expectancy is close to normal.
 ...
PMID:Essential thrombocythemia. Clinical characteristics and course of 61 cases. 336 70

The use of a simple, noninvasive, isotope scanning technique for the determination of relative portal blood flow and detection of portal hypertension is described. Using this technique the presence of portal hypertension was demonstrated in seven of nine patients known to have elevated portal venous pressure. By contrast, esophageal varices were demonstrated in only five of these patients, illustrating the potential value of the method. Furthermore, this technique has been adapted to the study of portal blood flow in patients with myeloproliferative disorders with splenomegaly but without disturbances in hepatic architecture. Results demonstrate that the high relative splenic flow resulting from the presence of splenomegaly may in turn be associated with elevated relative portal blood flow and portal hypertension. The theoretic reasons for the development of flow-related portal hypertension and its relationship to splenic blood flow are discussed.
 ...
PMID:Radioisotopic flow scanning for portal blood flow and portal hypertension. 366

Ten patients with myeloproliferative disorders and massive splenomegaly were treated with splenic artery infusions of cytarabine. Nine patients demonstrated a response to treatment with reduction in spleen size. Three patients had the procedure repeated on subsequent occasions. Relief from pain was the most significant clinical benefit; lasting for up to 6 months regardless of the rate of splenic re-enlargement.
 ...
PMID:Massive splenomegaly in myeloproliferative disorders treated by splenic artery infusion of cytarabine. 372 Feb 28

Splenic pooling of red cells and an expanded plasma volume are considered to be among the major mechanisms responsible for the anaemia in hypersplenism. In those conditions in which massive splenomegaly is associated with various degrees of marrow failure, diagnosis of the cause of anaemia may be difficult. A simple technique was used to estimate the degree of hypersplenism, from red cell mass data, in 94 patients with unequivocal lymphoproliferative or myeloproliferative disorders. The splenic effect was found to correlate well with both the size of the spleen (r = 0.75-0.90) and the actual red cell mass (0.79), and was abolished by splenectomy. Clinical data is also presented on 43 of these patients who underwent splenectomy. The incidence and type of complications, survival figures, and possible criteria for patient selection are discussed.
 ...
PMID:Hypersplenism and splenectomy in lymphoproliferative and myeloproliferative disorders. 383 May 29

This study reports the case of a patient who presented with evidence for a diagnosis of chronic myelogenous leukemia, as shown by blood and bone marrow analysis, and with generalized lymphadenopathy and splenomegaly. A lymph node biopsy revealed that the majority of the cells had plasmacytoid features but were consistently negative for surface or cytoplasmic immunoglobulin products, myelomonocytic surface markers, and peroxidase. Rather, lymph node plasmacytoid cells expressed T-cell markers (T 4/Leu 3+, T 10+), transferrin receptors (T 9+), and a proportion of them was also positive for sheep erythrocyte receptors (T 11/Leu 5+). This case is strikingly similar to a case reported by Lennert's group with respect to morphology, surface phenotypic features of the malignant plasmacytoid cells, and the association between a lymphoproliferative and a myeloproliferative disorder. This association suggests that plasmacytoid T-cells might exert a regulatory role on proliferation of myeloid cells.
 ...
PMID:Plasmacytoid T-cell lymphoma associated with chronic myeloproliferative disorder. 387 6

Primary or essential thrombocythemia is rarely observed in childhood, and familial occurrence has been reported only once. In this study, essential thrombocythemia is documented in five members of both sexes from two to 62 years of age in three successive generations. The propositus had a persistent elevation of the platelet count, splenomegaly, a normal hemoglobin level, a normal white blood cell count, and abnormal platelet aggregation. Platelet arachidonic acid metabolites assayed by high-performance liquid chromatography and serum thrombopoietin levels were normal. Megakaryocytes were increased in number and size. Both mature and early immature megakaryocytes, but no atypical megakaryocytes, were identified by surface immunofluorescence. Bone marrow cultures showed normal myeloid and erythroid colony formation, and chromosome studies revealed a normal female karyotype. These findings support the concept that familial essential thrombocythemia is a myeloproliferative disorder that is transmitted by an autosomal dominant mode of inheritance, and that untreated young women and children with essential thrombocythemia have long survival.
 ...
PMID:Familial essential thrombocythemia. 395 24

Classification of platelet disorders has been based on the platelet count. Addition of a second variable, mean platelet volume (MPV), to the routine blood count allows classification of patients into 9 categories: high, low, or normal MPV, and high, low or normal platelet count. We studied 1,244 adult inpatients. 1,134 had both platelet values normal. 11 patients had high MPV and low platelet count: all had hyperdestructive causes. 15 patients had high MPV and normal platelet count: 12 had heterozygous thalassemia, and three had iron deficiency. Seven patients had high MPV and high platelet count: causes included myeloproliferative disorders, inflammation, iron deficiency, and splenectomy, 25 patients had high platelet counts and normal MPV: the causes were inflammation, infection, sickle cell anemia, iron deficiency, or chronic myelogenous leukemia. 52 patients had an MPV that was inappropriately low for the platelet count (high, normal, or low). All had sepsis, splenomegaly, aplastic anemia, chronic renal failure, or a disease being treated with myelosuppressive drugs. High MPV thus appears correlated with myeloproliferative disease or thalassemia; and low MPV, with cytotoxic drugs or marrow hypoplasia. Addition of MPV to the platelet count allows subtler disorders to be detected (when the platelet count is normal), and allows distinction of the cause of thrombocytopenia.
 ...
PMID:Use of mean platelet volume improves detection of platelet disorders. 407 87

Osteolytic defects and osteoporosis are common in myeloma, while sclerotic lesions of bone are rare. Eighteen patients with increased bone density have been described in the literature and five patients are presented in this report. Diffuse increase in skeletal density, similar to that seen in the myelofibrosis-myelosclerosis syndrome, occurred in two patients, and progressive multiple focal areas of sclerosis with splenomegaly in a third. Two patients had solitary areas of sclerosis. Although there was increased cortical and trabecular bone, osteoblastic activity was normal on histological sections. Whether the sclerosis was due to new bone formation or interference with bone resorptive processes could not be determined. Patients with polycythemia, myelofibrosis and myelosclerosis have been found to have, or later develop, myeloma. This has led to the suggestion that myeloma be included among the myeloproliferative disorders. At present the evidence for this interrelationship is the frequency of the association of these diseases.
 ...
PMID:Sclerotic lesions of bone in myeloma. 495 77

Myeloproliferative disorders, a complex of cytologic abnormalities arising in the bone marrow, are among domestic animals most frequently recognized in cats but are relatively uncommon. A 4-year-old female Siamese, with splenomegaly and weight loss, was listless, anorectic, pale and dehydrated. A hemogram showed severe, macrocytic normochromic anemia, leukocytosis and reticulocytosis, with abnormally high numbers of nucleated RBC and undifferentiated blast cells. Bone marrow smears contained predominantly undifferentiated blast cells, RBC precursors and myeloblasts. The fluorescent antibody test for FeLV was positive. The cat died 66 days later despite a blood transfusion and chemotherapy. Necropsy confirmed a diagnosis of myeloproliferative disease, with hepatic and splenic invasion.
 ...
PMID:Myeloproliferative disease in a cat. 650 11

Two cases of acute myelofibrosis are described which exemplify diagnostic and therapeutic possibilities in this clinical and morphologic entity. Delimiting criteria against other diseases with bone marrow fibrosis are also presented. This malignant myeloproliferative disorder is characterized by acute onset, minimal or absent splenomegaly, blood cytopenias with a highly variable percentage of blast cells in circulating blood, often unimpressive tear-drop poikilocytosis, intense marrow fibrosis, and a rapid clinical course. In one case, typical proliferation of all three hematopoietic cell lines was lacking and proliferating blast cells could be classified as myeloblasts/promyelocytes histochemically. In the other case, a single course of intensive combination chemotherapy resulted in marked dissolution of the marrow fibrosis and prolonged survival in partial remission.
 ...
PMID:[Acute osteomyelofibrosis. An overview and 2 personal cases]. 657 66


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>