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)

Four cases of pure red-cell aplasia (PRCA) in association with chronic lymphocytic leukemia (CLL) are summarized. In addition, all reported cases of CLL-PRCA (27, including our 4 cases) are reviewed to consolidate their clinical features and compare them with those of 26 patients with CLL who were anemic (Stage III) and 52 who were not anemic (Stages O-II). The incidence of PRCA in our patients with CLL is about 6%. Eighty-seven percent of patients with PRCA had B-lymphocyte (B-cell) CLL and 13% had T-lymphocyte (T-cell) CLL. In 4 of 17 patients PRCA was the presenting feature, and in 13 of 17 it developed in patients previously diagnosed as having CLL. In the latter group, the average time from the diagnosis of CLL to the development of PRCA was 50.2 months (range, 1 to 96). The average age at presentation with PRCA was 61.6 years (range, 37 to 79), and the male to female ratio was 1.6 to 3:1. Eighty-one percent had splenomegaly and 65% had lymphadenopathy. The average hematocrit was 17 +/- 3.6% with zero reticulocyte count, and the lymphocyte count was 134 X 10(3)/microliter. Normoblasts were absent in the marrow and lymphocytes constituted 50 to 100% of the marrow cells. In patients with B-cell CLL and those with T-cell CLL, the T cells were found to inhibit the growth of the marrow erythroid progenitors in in vitro cultures. These abnormal T cells (Tr cells) possessed IgG Fc receptors on their surface, which may be responsible for induction of PRCA in these patients. Continued administration of cyclophosphamide and prednisone in moderate doses appears to induce remission from PRCA, but not until the leukemic mass and abnormal Tr cells are markedly reduced. Daily or alternate-day cyclophosphamide administration in small to moderate doses seems to be essential to maintain remission.
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PMID:Pure red-cell aplasia in patients with chronic lymphocytic leukemia. 309 91

Ferrite, a new magnetic resonance (MR) contrast agent, was evaluated in the detection and diagnosis of splenic lymphoma. Before administration of ferrite, normal rat spleens and spleens with diffuse lymphoma showed similar in vitro relaxation times and in vivo MR imaging signal-to-noise ratios (S/N). After the administration of ferrite (50 mumol Fe/kg), the T2 time of lymphomatous spleen was 27.0 msec +/- 2.9 (mean +/- standard deviation), which was significantly greater than that of normal spleen (10.7 msec +/- 1.5, P less than .005). The S/N of ferrite-enhanced in vivo MR images of lymphomatous spleen was 12.4 +/- 0.9, which was significantly greater than normal (5.7 +/- 0.2, P less than .005). Similar experiments with animal models of micronodular lymphoma also demonstrated that ferrite-enhanced MR imaging can distinguish micronodular lymphoma from normal spleen. Benign splenomegaly, studied with an animal model of erythroid hyperplasia, showed ferrite-enhanced MR tissue characteristics that were indistinguishable from those of normal spleen.
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PMID:Splenic lymphoma: ferrite-enhanced MR imaging in rats. 333 17

The lactate dehydrogenase mouse mutant Ldh-1c/Ldh-1c is afflicted with a severe hemolytic anemia associated with extreme reticulocytosis (95%) and splenomegaly. Ninety-one percent of the total body colony-forming units--erythroid (CFU-E) have been quantified in the seven- to ten-times enlarged spleens of the mutant mice. Moreover, the splenic fraction of morphologically recognizable erythroid precursors was 134 times normal. From these data it was apparent that the spleen crucially contributes to the maintenance of steady state erythropoiesis in the mutants. On the other hand, an enhanced sequestration of red blood cells in the enlarged spleen may augment the anemia. Splenectomy experiments were performed with LDH mutant and wild type mice in order to investigate the role of the spleen in this particular hemolytic disease. Following splenectomy, the peripheral blood values and the frequency of femoral stem and progenitor cells were determined, and histological investigations were carried out. The life span of the splenectomized mutants was not shortened, in spite of a very low red blood cell count (25% of the untreated mutant value). Compared to the splenic loss only a moderate increase in bone marrow erythropoiesis was observed, such as a 250% increase of CFU-E. It is concluded that the reduction in red blood cell survival due to splenic sequestration in the mutants is of such a magnitude that it counterbalances a significant portion of splenic erythropoiesis.
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PMID:The role of the spleen in a lactate dehydrogenase mutant mouse (Ldh-1c/Ldh-1c) with hemolytic anemia. 336 65

A preleukemic state in mice inoculated with Moloney murine leukemia virus (Mo-MuLV) was characterized. Six to 10 weeks after neonatal inoculation, animals developed mild splenomegaly and generalized hematopoietic hyperplasia. The hyperplasia was evident from myeloid and erythroid progenitor assays. A nonleukemogenic variant, Mo+PyF101 Mo-MuLV, did not induce the hyperplasia; this suggests that the hyperplasia is a necessary event in Mo-MuLV leukemogenesis. Another variant, MF-MuLV, which contains the long terminal repeat of Friend MuLV and causes erythroid leukemia instead of T-cell lymphoma, also induced the preleukemic hyperplasia. A model for Mo-MuLV leukemogenesis is presented in which two infection events are necessary: the first leads to generalized hematopoietic hyperplasia, and the second results in site-specific insertion and long terminal repeat activation of cellular protooncogenes.
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PMID:Characterization of a preleukemic state induced by Moloney murine leukemia virus: evidence for two infection events during leukemogenesis. 347 32

We report three cases of polycythaemia with no evidence of clinical splenomegaly and normal splenic red cell pool on isotope spleen scan. In each case, however, a diagnosis of primary proliferative polycythaemia (PPP) was suggested by in-vitro erythropoietin-independent growth of peripheral blood erythroid colonies. In one of these cases two possible causes of secondary polycythaemia were also identified. The use of investigations such as isotope spleen scanning and erythroid cell culture in helping to establish a diagnosis of PPP is discussed.
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PMID:Primary proliferative polycythaemia without splenomegaly: a diagnostic problem. 362 56

The effects of an autologous transplanted mammary tumor (RIII-T3) on hemopoiesis in RIII mice are described. Tumor-bearing animals died 30 to 40 days after inoculation and displayed splenomegaly, extreme neutrophilia, and moderately increased monocyte levels in the spleen, peripheral blood, and bone marrow. The precursors of neutrophils and monocytes, granulocyte/macrophage colony-forming cells (GM-CFC) were elevated in the spleen, bone marrow, and peripheral blood. RIII-T3-conditioned medium stimulated bone marrow GM-CFC and caused the myelomonocytic cell line, WEHI-3B, to differentiate in vitro. The conditioned medium did not stimulate erythroid, megakaryocyte, or eosinophil colony formation. When conditioned medium was fractionated, two peaks of activity corresponding to GM-CSF and G-CSF were observed, suggesting that the extreme neutrophilia observed in tumor-bearing animals may result from chronic exposure of the hemopoietic system to these hemopoietic hormones.
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PMID:Effects of a murine mammary tumor on in vivo and in vitro hemopoiesis. 387 4

The presence of teardrop-shaped red cells in peripheral blood has traditionally been felt to reflect altered marrow architecture, namely myelofibrosis. We evaluated two patients with splenomegaly, moderately severe hemolytic anemia due to warm-reactive IgG anti-red cell autoantibody, and bone marrow erythroid hyperplasia without myelofibrosis. A striking predominance of teardrop-shaped red cells was noted upon examination of their blood films. Removal of a spleen containing extramedullary hematopoiesis in one and resolution of splenomegaly in the other were accompanied by disappearance of these cells. Our observations support a role for the spleen and for extramedullary hematopoiesis in the pathogenesis of this distinctive red cell morphologic abnormality.
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PMID:Teardrop-shaped red cells in autoimmune hemolytic anemia. 395 60

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.
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PMID:Familial essential thrombocythemia. 395 24

Cyclohexanone oxime (CHO) was given po to male and female Fischer 344 rats at dose levels of 10, 25, 75, 150, and 300 mg/kg, five times a week for a period of 2 weeks. Control animals received distilled water. All animals given intermediate dose levels (10, 25, 75, and 150 mg/kg) and one half of the animals which were dosed at the high dose (300 mg/kg) as well as one half of the controls were terminated 14 days after administration of the first dose. The remaining rats received no treatment for an additional 14 days and were sacrificed on Day 28 of the study (recovery phase). Dose-related decreases in erythrocyte number, hemoglobin, and hematocrit, with an accompanying increase in reticulocytes and circulating nucleated erythrocytes, were observed in both sexes at Day 14. Methemoglobin levels, determined only at the high dose, were elevated in both sexes at this time. Splenomegaly and hepatomegaly were observed in both sexes at 14 and 28 days. Histopathological examination of the spleen and bone marrow revealed dose-related erythroid hyperplasia at 14 days which subsided by Day 28. The above effects were more pronounced in males. Erythrocyte numbers were only slightly depressed and reticulocytes mildly elevated in males at Day 28. Hematological values were not statistically different from controls in females at this time. These results suggest that CHO induces oxidative damage to the erythrocyte, resulting in a hemolytic anemia accompanied by increased erythropoiesis. The toxic effects appear reversible upon cessation of exposure.
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PMID:Toxicity of cyclohexanone oxime. I. Hematotoxicity following subacute exposure in rats. 398 89

Hemopoietic changes in male C57BL/6Cum BR mice engrafted with Lewis lung carcinoma (3LL) were evaluated between day 7, when palpable tumors were present, to day 30 postengraftment. All experimental animals demonstrated decreasing hematocrits (down 40% by day 30) with concurrent leukocytosis which by day 30 postengraftment had reached levels 13.4 times normal. The myelocytic/erythrocytic ratio for normal animals was 1:3 (bone marrow: spleen). The ratio for engrafted animals ranged between 10:1 and 40:1. This apparent shift in production priorities is even more significant in light of the fact that femoral bone marrow cellularity had decreased by 33% on day 17. Splenomegaly, evident by day 7, was seven times control by day 17. Clonogenic analysis of erythroprogenitor cell concentrations revealed an inverse relationship between bone marrow and spleen. 27 days after engraftment, splenic populations demonstrated significant increases in colony forming unit-erythroid (115-fold), burst forming unit-erythroid (7.4-fold), whereas bone marrow concentrations had decreased (6-fold). This report suggests that initiation of 3LL tumor in mice results in a change in the degree of hematopoietic priorities and participation of erythroid organs.
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PMID:Modulations in mouse hemopoiesis after engraftment with Lewis lung (3LL) carcinoma cells. 404 4


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