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 55-year-old woman was first seen in October 1986, because of splenomegaly, moderate anemia and leukocytosis. The hemoglobin was 8.8 g/dl, platelet count 24.4 X 10(4)/microliters, and the white cell count 23,800/microliters with 73% atypical lymphoid cells. The bone marrow nucleated cell count was 99,000/microliters with 36% lymphoid cells. These atypical lymphoid cells showed hairy appearance under phase-contrast microscopy, and were positive for tartrate-resistant acid phosphatase. These cells showed the surface phenotype of CD10, CD19, CD20, Leu M5, HCM, and IgG K. Biochemical data revealed marked polyclonal hypergammagloburinemia (PHG) of IgG type (IgG 8756 mg/dl). To elucidate the mechanism of the PHG, we investigated whether hairy cells produce interleukin 6 (IL-6) and express IL-6 receptor. The culture supernatant of these hairy cells increased 3H-thymidine uptake of a IL-6 dependent hybridoma clone (MH60) in a dose-dependent manner. These cells were stained with anti-IL-6 antibody using immuno-cytochemical technique. Our results suggested that these hairy cells produce and secrete IL-6. Immunocytochemical staining with anti IL-6 receptor antibody and the binding assay with 125I-labelled recombinant IL-6 revealed that these cells express little or no receptors for IL-6. It was therefore suggested that IL-6 produced by hairy cells in this case is not an autocrine growth factor for these cells but may play a role in development of PHG by stimulating normal B lymphocytes to produce an excessive amount of immunoglobulin.
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PMID:[Production and secretion of BSF2/IL6 in a case of hairy cell leukemia with polyclonal hypergammaglobulinemia]. 238 10

One hundred and eighteen patients with chronic leukaemias were seen at the Lagos University Teaching Hospital, Nigeria, between 1964 and 1982. There were 75 patients with chronic granulocytic leukaemia (CGL) and 43 patients with chronic lymphocytic leukaemia (CLL). Although most of them presented with the familiar features of chronic leukaemias, a few features were remarkably different from those reported in some of the Caucasian series. CLL is less common than CGL in contrast to their relative incidence in Caucasians. Our patients generally presented with more massive splenomegaly and more severe anaemia, which could be attributed to late presentation, endemic malaria and possibly increased hypersplenism. The peak-age incidence in our patients with CGL was found in a younger age group (20-40 yr) than in the Caucasian series. When compared with a Caucasian series, our CGL patients on presentation had a significantly higher proportion of immature cells (blasts and promyelocytes) (P less than 0.05), probably reflecting their more delayed presentation. Follow up was generally poor as a result of a high default rate. Survival duration of both leukaemias was generally lower than in Caucasian series and for CGL patients there was a significant negative correlation between survival and spleen size at presentation, while for CLL patients there was a significant association between poor survival duration and high white cell count at presentation.
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PMID:Chronic leukaemia: an African experience. 261 22

A 33-year-old female was admitted to St. Marianna University hospital in April 1983 for the purpose of examination for leukocytosis. Physical examination revealed a marked splenomegaly. The white cell count was 174 x 10(9)/l. The hemoglobin was 9.0 g/dl and the platelet was 790 x 10(9)/l. Microscopical examination of aspirated specimen of bone marrow revealed hypercellularity with granulocytic hyperplasia. The chromosomal analysis of bone marrow cells showed Philadelphia chromosomes in all metaphases analyzed. The neutrophil alkaline phosphatase activity was reduced. A diagnosis of CML was made. She was treated with busulfan in a dose of 2 mg/day until the white cell count was 14.5 x 10(9)/l. She has been followed without any therapy and clinical remission state has been continued. In April 1985, the chromosomal analysis of bone marrow cells revealed the recovery of normal karyotype hemopoiesis in 57% of metaphases analyzed. These findings of this case suggest that some of Ph1-positive cells may reduce their growth advantage over normal cells without any bone marrow hypoplasia.
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PMID:[Appearance of chromosomally normal hemopoiesis during busulfan-induced remission in a case of Ph1 positive chronic myelogenous leukemia]. 274 80

Between 1981 and 1987, L3 ALL was diagnosed in 18 adult patients, with a median age of 26 (range 16-66) and M/F ratio of 3.5. At diagnosis, 11 patients had splenomegaly, 11 had enlarged lymph nodes, and 15 patients had central nervous system (CNS) disease, of whom 10 had mental neuropathy. Anaemia was found in 13 patients, thrombocytopenia in 17 and the median white cell count was 25.5 x 10(9)/I (range 8.6-89). Surface immunoglobulins were found on the blasts of every patient. Seventeen patients had a t(8;14) (q24;q32) translocation. One had an apparently normal karyotype, but only six mitoses could be examined. During the period of the study different treatment protocols, which comprised increasingly intensive systemic and CNS chemotherapy, were used. Six patients died less than 3 weeks after admission, two of them of acute tumour lysis syndrome and two of CNS haemorrhage. In two other patients, rapid progression of CNS leukaemia was seen in spite of the treatment. Ten patients (56%) achieved complete remission (CR). Two were allografted and two were autografted early in CR. Four patients relapsed, three of the four relapses involving the CNS. A median actuarial disease-free survival was not attained, and a plateau was achieved at 57% after 7 months, with no later relapse. Median actuarial survival of the 18 patients was only 6 months, but a plateau was obtained at 31% after 11 months. Prognosis seemed related to the intensity of chemotherapy: recent patients, treated more aggressively, achieved CR more often than earlier patients, treated with less intensive protocols, although the number of patients was too small to draw any firm conclusion. The initial white cell count was also a prognostic factor, as none of the patients with more than 30 x 10(9)/I leucocytes achieved CR. Our results suggest that the outcome of adult L3 ALL can be improved, as in children, by increased intensity of treatment, particularly with regard to CNS leukaemia therapy. Early deaths are still frequent, however, but their incidence can probably be reduced by better prevention and early management of the acute tumour lysis syndrome.
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PMID:Burkitt cell acute leukaemia (L3 ALL) in adults: a report of 18 cases. 261 Jul 42

Ten patients with follicular lymphoma presented with a high white cell count (45-220 x 10(9)/l) which resembled chronic lymphocytic leukaemia (CCL): all had pronounced splenomegaly and, except one, generalised lymphadenopathy. The blood lymphocytes were small with scanty cytoplasm, densely condensed nuclear chromatin, and deep clefts originating in sharp angles from the nuclear surface. CLL cells are larger, have more cytoplasm, a different pattern of chromatin condensation, and may have shallow nuclear indentations or foldings rather than clefts. The circulating follicular lymphoma cells had moderate to strong membrane immunoglobulins (SmIg), low mouse (M)-rosettes, strong reactivity with the monoclonal antibody FMC7, and occasional expression of the CD5-antigen; at least one third of cells in each case were positive with anti-cALLa (J5,CD10). Half the cases were referred as B-CLL but none had the typical B-CLL immunophenotype: weak SmIg, M-rosettes of greater than 50%, CD5 positive, FMC7 and J5 negative. The diagnosis of follicular lymphoma was confirmed by lymph node biopsy in seven of the 10 cases. The overall response to treatment was poor and five patients died within three years of diagnosis. This aggressive form of follicular lymphoma needs to be distinguished from B-CLL as different management is required.
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PMID:Morphology and immunology of circulating cells in leukaemic phase of follicular lymphoma. 305 87

Fifty-nine children with sickle cell anaemia (HbSS) or associated haemoglobinopathies were studied prospectively using a chromogenic Limulus amoebocyte lysate assay to detect circulating endotoxin. The 41 children with HbSS (mean age 8 years 9 months) had more serious disease than the 18 with HbSC disease (n = 14) or HbS-beta-thalassaemia (n = 4) (mean age 7 years 2 months), with a greater degree of splenomegaly, lower haemoglobin, and higher white cell counts, platelet counts and bilirubin values (P less than 0.05 for all). Twenty-nine children with HbSS had evidence of poor reticuloendothelial function, with red cell pitting of greater than or equal to 2%. Three of these 29 had low levels of endotoxin in plasma (0.12-0.24 endotoxin units (EU)/ml); two were clinically well, one had a painful crisis. Eight of 18 children with other sickle haemoglobinopathies had greater than or equal to 2% pitted red cells; none was endotoxinaemic. Therefore, in 37 patients with reticuloendothelial dysfunction, three were endotoxinaemic; all had sickle cell anaemia. Although not statistically significant, this suggests that endotoxinaemia may occur predominantly in patients with reticuloendothelial dysfunction, and is compatible with the hypothesis that systemic endotoxinaemia can derive from the intestine especially when reticuloendothelial function is depressed.
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PMID:Endotoxinaemia in sickle cell disease. 307 92

This retrospective review of 136 children with idiopathic thrombocytopenia assesses the prognostic significance of various presenting parameters and the effect of therapy. Initial haemoglobin, white cell count, actual platelet count, splenomegaly, hepatomegaly and lymphadenopathy had no significant effect upon the final outcome. Cases of acute I.T.P. were associated with a higher incidence of preceding infection, a shorter history of bleeding and a preponderance of males. The presence of greater than 20 per cent lymphocytes in the bone marrow was associated with a longer time to achieve a normal platelet count (p = 0.05). Steroid therapy shortened the time for acute cases of I.T.P. to obtain a normal platelet count (p = 0.05), but had no effect on long-term prognosis. Chronicity occurred in 25 children (18%) and nine of these had spontaneous remissions. Immunosuppressive therapy was ineffective, but nine out of 12 children were cured by splenectomy. Mortality was 0.7 per cent (one death) and general morbidity was low.
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PMID:Idiopathic thrombocytopenia in childhood. Edinburgh experience 1962-82. 379 80

Fifteen of 73 newly diagnosed patients with acute myeloid leukemia (AML), admitted to Mount Sinai Hospital between July 1977 and October 1979, presented with leukocyte counts greater than 100,000/microliter. Eleven of these 15 patients with hyperleukocytosis had myelomonocytic (AMML-M4) or monocytic (AMOL-M5) leukemia compared to 15 of 58 patients with lower white cell counts (p < 0.001). Identification of type of leukemia, using the FAB classification, was based on morphology and special stains, including myeloperoxidase, Sudan black B, periodic acid-Schiff and nonspecific esterase with and without inhibition by fluoride. The proportion of patients with splenomegaly is higher in those with hyperleukocytosis (73 percent) than in those with lower white blood cell counts (p < 0.001) regardless of cell type. Leukemic infiltration of the skin, gums and central nervous system was seen exclusively in patients with AMML and AMOL. The serum lysozyme levels were significantly higher for all patients with AMML and AMOL regardless of the white blood cell count. The mean serum lysozyme for M-4, M-5 patients was 59.7 microgram/ml compared to 18.9 microgram/ml in patients with other cell types (p < 0.0001). Patients with a white blood cell count less than or equal to 100,000/microliter had a complete remission rate of 69 percent compared to 47 percent for patients with higher white blood cell counts.
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PMID:Association of monocytic leukemia in patients with extreme leukocytosis. 693 15

Fifty cases of visceral leishmaniasis were admitted in Children's Hospital, Islamabad. Common clinical features were fever (100%), splenomegaly (100%), hepatomegaly (100%), anaemia (96%), abdominal distension (40%), bronchopneumonia (26%) and bleeding diathesis (22%). Hb was below 7.0 G/dl in 80%, white cell count below 4 x 10(9)/cmm in 88% and platelet count below 100 x 10(9)/c4mm in 86%. All the patients showed leishmania donovani bodies in the marrow smears. Fourteen patients were treated with aminosidine (15 mg/kg), intramuscularly daily for 4 weeks. All responded dramatically and none of them went into relapse in a year's follow-up. No side-effects were observed. Aminosidine can therefore, be recommended as a treatment of choice for visceral leishmaniasis in children.
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PMID:A new breakthrough in treatment of visceral leishmaniasis in children. 747 90

Although the expression of myeloid-associated antigen CD13 has been reported in aggressive B-cell chronic lymphocytic leukemia, its expression in other mature B-cell neoplasms appears to be rare. We report a 74-year-old female with B-cell prolymphocytic leukemia (B-PLL) expressing CD13 antigen. On admission, splenomegaly was noted. Hematological examination revealed a platelet count of 90 x 10(9)/l and a white cell count of 68 x 10(9)/l with 73% PLL cells. The hemoglobin concentration was 10.6 g/dl. A bone marrow aspirate showed a normocellular marrow with 64% PLL cells. Surface marker analysis of the PLL cells was positive for CD11b, CD13, CD19, CD20, CD24, HLA-DR, FMC7, mu and lambda. Simultaneous expression of CD13 and CD19 antigen was confirmed by dual color flow cytometry. Southern blot analysis of DNA from circulating mononuclear cells gave a rearranged band for the immunoglobulin gene (JH) but not for TCR-beta. Cytogenetic analysis of marrow cells showed an abnormal karyotype involving numbers 1, 7, 10, 12, 14, 15 chromosomes.
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PMID:B-cell prolymphocytic leukemia expressing CD13 antigen. 752 29


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