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 retrospective survey of 700 marrow examinations in Papua New Guinea in a 2 1/2 year period has been carried out. Over half of the total came from the Port Moresby General Hospital. Anaemia and splenomegaly were the commonest indications. A disturbing proportion of the specimens were unsatisfactory for interpretation. Hypercellular marrows with erythroid hyperplasia were very common. Iron stores were absent or nearly absent in almost half of the evaluable specimens; this proportion rose to over 95% amongst pregnant or post-partum women with anaemia. Other relatively frequent findings included: a combination of features consistent with hypersplenism; mild degrees of eosinophilia and/or plasmacytosis; and mild degrees of megaloblastic changes. Haematological malignancies were found in over 10% of evaluable specimens. Of 611 assessable marrows from Melanesians, only 11 (1.8%) were entirely normal, but another 151 (24.7%) contained only minor abnormalities.
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PMID:A review of 700 bone marrow aspirations in Papua New Guinea. 26 75

The most common haematological abnormalities in alcoholism are raised mean corpuscular volume of the erythrocytes and thrombocytopenia. The etiology is multifactorial including malnutrition with folate deficiency, a direct toxic influence of alcohol and sequestration in an enlarged spleen. Sideroblastic anaemia caused by interference of alcohol with the metabolism of pyridoxine is common and so is haemolytic anaemia caused by hypersplenism and megaloblastic anaemia. Leucopenia can be seen and is probably caused by a direct toxic effect of alcohol on the bone marrow. Other potentially toxic changes are impaired chemotaxis, motility and adherence of the granulocytes and impaired blast-transformation of the lymphocytes. In the bone marrow, vacuolized precursors of myelo- and erythropoiesis are seen. The bone marrow may be hypocellular. Other changes in the bone marrow are increased but ineffective erythropoiesis with defective iron metabolism, vacuolized pro-erythroblasts, multinucleated erythroblasts, megaloblasts and iron-containing plasma cells.
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PMID:[Hematological abnormalities in alcoholism]. 150 2

Chronic lymphocytic leukaemia was diagnosed in 1957 in a 29-year-old male patient who lived 31 years after that time. At the time of diagnosis he was in good general condition with the enlarged liver (4 cm) and spleen (5 cm). In peripheral blood the leukocyte count was 47 x 10(9)/1 with 80% lymphocytes (38 x 10(9)/1), Hb 106 g/1; the bone marrow showed hypercellularity with predominance of lymphocytes. Treatment with corticosteroids was during 1958-1966. In 1967 Chlorambucil was given for 3 months, in 1972 for one month, inducing normalization of leukocyte count, 5.7 x 10(9)/1 with 40-50% of lymphocytes (3 x 10(9)/1). In 1977 the bone marrow showed slight lymphocytosis (38%). Numerous infections were recorded. Splenomegaly disappeared in 1980. In 1982 macrocytic anaemia was observed. Diagnosis of pernicious anaemia was established based on megaloblastic bone marrow and positive Schilling test. Vitamin B12 treatment resulted in reticulocyte response (crisis) and normalization of haemoglobin level. In 1988 light anaemia and melena appeared. Gastroscopy revealed lesions in the fornix histologically identified as ventricular carcinoma. It can be presumed that the appearance of pernicious anaemia as an autoimmune disease is analogous to other autoimmune complications in chronic lymphocytic leukaemia (haemolytic anaemia, thrombocytopenia).
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PMID:[Chronic lymphocytic leukemia complicated by pernicious anemia during long-term remission]. 213 9

Liver disease, particularly alcoholic cirrhosis, is associated with a number of interesting chemical changes which result in structural and metabolic abnormalities of the erythrocyte membrane leading to microscopically observable cell shape changes and hemolytic anemia varying from very mild to potentially lethal. Increase in unesterified serum cholesterol owing to lecithin cholesterol acyl transferase (LCAT) deficiency in cirrhosis leads to expansion of the lipid bilayer and macrocytosis without megaloblastic changes in precursors. Substitutions of phosphatidyl choline (PC) moieties in the erythrocyte lipid bilayer lead to echinocytes (disaturated PC) or to stomatocytes (diunsaturated PC). In some patients, high density lipoprotein (HDL) abnormalities lead to erythrocyte surface changes causing rapid formation of echinocytes. The rapidity and reversibility of these changes suggest blockade of metabolic transport channels critical to the maintenance of erythrocyte membrane shape. Metabolic changes involving vitamin E deficiency leading to lipid peroxidation and pyruvate kinase instability leading to adenosine triphosphate (ATP) reduction have also been invoked to explain hemolysis associated with acute liver damage. The most severe hemolysis in liver disease is associated with acanthocytes (spur cells) and a marked imbalance in cholesterol-phospholipid ratio. These patients usually have hypersplenism, as well as rigid erythrocyte membrane transformations which are irreversible. Any of the other erythrocyte membrane shape changes described appear to be reversible if the liver disease abates, but they too may become irreversible if bits of projecting membrane are repeatedly removed by the macrophages of an enlarged spleen.
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PMID:Mechanisms of hemolysis in liver disease. 218 63

A case of congenital dyserythropoietic anaemia presented with recurrent jaundice and painful splenomegaly. There was binuclearity, internuclear chromatin bridges, megaloblastic changes in erythrocyte precursors and positive acidified serum test with normal serum.
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PMID:Congenital dyserythropoietic anaemia. 239 25

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

Transient granulocytopenia and lymphopenia may occur in acute alcoholics without splenomegaly, cirrhosis, infection, and megaloblastic anemia due to folate deficiency. The bone marrow in granulocytopenic patients is frequently hypocellular with few mature granulocytes, and the functional marrow granulocyte reserve is reduced. These findings suggest a depressed granulopoietic activity in these patients. The mechanism by which alcohol suppresses granulopoiesis remains unclear. Direct toxicity of alcohol on granulopoietic stem cells and increased individual susceptibility to the toxic effect of alcohol may be important factors. Alcohol also causes functional impairment of granulocytes (adherence, motility, and chemotaxis), macrophages (motility and phagocytosis), and lymphocytes (blastogenic transformation and development of delayed dermal hypersensitivity reaction), probably by perturbation of the cell membrane resulting in an increased intracellular cyclic AMP level.
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PMID:Effects of alcohol on granulocytes and lymphocytes. 737 52

Increased numbers of bone marrow mast cells were found in 45 (2.2%) of 2,000 bone marrow specimens obtained from patients who had hematologic disorders. Mast cells were most frequently seen in the marrows of patients who had preleukemic syndromes, lymphoproliferative disorders, and acute leukemia. The 16 patients who had preleukemic syndromes included those with refractory sideroblastic and megaloblastic anemia (with or without an excess of blasts), idiopathic pancytopenia or pure erythrocytic aplasia, paroxysmal nocturnal hemoglobinuria, idiopathic refractory neutropenia, agranulocytosis or thrombocytopenia, and persistent eosinophilia. Five of the seven patients who had acute leukemia had nonlymphoblastic leukemia; two had blastic crisis of chronic granulocytic leukemia. Of the 13 patients who had lymphoproliferative disorders, eight had chronic lymphocytic leukemia, three had macroglobulinemia, and two had non-Hodgkin's lymphoma. Three patients who had chronic renal failure associated with severe anemia and two who had chronic liver disease, splenomegaly, or hypersplenism were also encountered. In this study there appeared to be a consistent relationship between the presence of increased numbers of mast cells and the lymphocyte and plasma cell counts in the bone marrow. The significance of the presence of secondary mastocytosis in premalignant lesions, neoplasia, and, in particular, lympho- and myeloproliferative disorders, is still unclear.
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PMID:Increased bone marrow mast cells in preleukemic syndromes, acute leukemia, and lymphoproliferative disorders. 745 27

Imerslund-Najman-Grasbeck disease is a rare inherited megaloblastic anaemia secondary to a selective malabsorption of vitamin B12 by ileal enterocytes. The authors report on a 4 year-old tunisian girl who presented as visceral infantile leishmaniasis because of huge splenomegaly and major anaemia. The diagnosis of Imerslund disease was performed on the basis of the association of typical megaloblastic cells in the marrow, permanent proteinuria and favourable outcome under parenteral B12 administration. In addition, ther were no folate deficiency, no anti-intrinsic factor antibodies and no intrinsic factor deficiency. The outcome of the disease is always favourable if parenteral administration of vitamin B12 maintained.
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PMID:[Imerslund-Najman-Grasbeck anemia. Apropos of a case]. 801 68

A 19 years old male admitted to our hospital with fever, abdominal pain in May 1991. Physical examination revealed anemia, jaundice and marked splenomegaly. Severe pancytopenia with macrocytic hyperchronic anemia was noted along with elevated LDH and reduced serum folate. Blood smear showed nucleated RBCs, but only few microspherocytes. Bone marrow showed erythroid hyperplasia with remarkable megaloblastic changes. Megaloblasts were negative for PAS stain. Chromosome analysis revealed normal karyotype. Erythroleukemia was suspected initially, but his general condition as well as hematological data improved following 10 units of RBC transfusion. Following brief folic acid supplements, numerous microspherocytes became evident, typical osmotic fragility test revealed a pattern for hereditary spherocytosis. These observations led us to the diagnosis of hereditary spherocytosis complicated by megaloblastic anemia due to folate deficiency. As he developed folate deficiency again 10 months later, splenectomy were performed. The anemia improved after splenectomy.
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PMID:[Megaloblastic anemia due to folate deficiency associated with hereditary spherocytosis]. 802 87


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