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)

Report of five cases of juvenile myelomonocytic leukemia; authors describe an hemopathy of the young child with important splenomegaly, hepatomegaly and adenopathies; anemia with erytroblastemia; hyperleucocytosis with myelemia and monocytosis, rich and granulous bone marrow. Also remarkable is evolution, as it fatal outcome often occurs before a two year evolution, without remission looking like acute myeloblastic leukemia, a few patients have remission of most the symptoms, ending in an apparent recovery, which may be spontaneous.
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PMID:[Myelomonocytic leukemia in childhood (author's transl)]. 106 64

Leukocyte kinetic studies using chromium-51 were performed in four patients with acute myelocytic leukemia (AML). Intravascular leukocyte survival was prolonged in comparision with granulocyte survival in normal subjects. Significant splenic pooling occurred in three patients, none of whom had splenomegaly. In one patient studied, circulating leukemic cells were shown to return to the bone marrow. The prolongation of intravascular leukocyte survival in AML in relapse, as in chronic myelocytic leukemia, probably depends on several factors including the presence of immature leukemic cells and the recycling of these cells from the spleen and bone marrow.
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PMID:Study of leukocyte kinetics in acute myelocytic leukemia utilizing chromium-51. 106 22

The reciprocal translocation (9;22)(q34;q11) is highly characteristic of chronic myeloid leukemia (CML) and the pericentric inversion inv(16)(p13q22) is almost only found in acute nonlymphocytic leukemia of the myelomonocytic subtype (ANLL M4). Only twice before have an inv(16) and a t(9;22) been found in the same cells, and both times the patients seemed to have de novo ANLL M4. We describe the case of a 21-year-old man who in July 1986 presented with a clinically and hematologically classic chronic phase CML. Treatment with busulfan led to no improvement; instead in September 1986 he developed blast crisis with ANLL M4Eo morphology. He was now cytogenetically examined and the karyotype 45,X,-Y,t(9;22)(q34;q11),inv(16)(p13q22) was found. Southern blot analysis of the bone marrow DNA sampled at this time revealed a standard rearrangement in the 3' end of the M-bcr. Intensive cytostatic treatment caused cytopenia followed by complete hematologic, clinical, and cytogenetic reversal to chronic phase CML, so that in January 1987 the bone marrow karyotype was 46,XY,t(9;22)(q34;q11). Persistent splenomegaly was treated with splenectomy, and a chloroma of the skin was removed by irradiation. In March 1987 he received an allogeneic bone marrow transplant. Since then his only medical problem has been mild graft-versus-host disease; he is well and is working full time as a blacksmith.
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PMID:Acute myelomonocytic leukemia with inv(16)(p13q22) complicating Philadelphia chromosome positive chronic myeloid leukemia. 155 89

Leukemic cell expression and serum levels of CD4, CD8, and interleukin-2 receptor (IL-2R) were determined at diagnosis for children or adolescents with acute myeloid leukemia (AML). Cellular expression of CD4 was detected in 18 of 62 cases, CD8 in none of 60 cases, and IL-2R in one of 33 cases tested. Myeloblasts of the M4 and M5 subtypes expressed CD4 significantly more frequently than other FAB subtypes (p = 0.0001). Serum levels of the three soluble factors (tested for 91 patients) were positively correlated with each other. Increased serum CD4 levels were significantly associated with cellular CD4 expression, high leukocyte count, M5 leukemia, spleen enlargement, and age less than 1 year. High serum CD8 levels correlated significantly with splenomegaly, extramedullary disease, absence of Auer rods, and high leukocyte count. Cases with high serum IL-2R levels were less likely to have Auer rods and more likely to have splenomegaly and M5 leukemia; serum levels greater than 750 U/ml were associated with a higher probability of treatment failure (p = 0.05), even after adjustment for other potential prognostic factors. Further studies of serum CD4, CD8, and IL-2R levels may help to clarify the immunoregulatory role of T-cells in patients with AML.
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PMID:Serum CD4, CD8, and interleukin-2 receptor levels in childhood acute myeloid leukemia. 190 14

A series of 27 patients with myelodysplastic syndrome (MDS) was analysed to determine the clinical and pathologic features, the natural history and the prognostic factors. The clinical features of these patients included the following: mean age 53.77 years; symptoms due to cytopenias 100 per cent; hepatomegaly 44.5 per cent; splenomegaly 11 per cent. Almost all patients with MDS presented anemia; additional cytopenias were present in many patients. The bone marrow was hypocellular in 1/3 of cases. The patients have been classified according to the French-American-British (FAB) Group criteria: there were 11 patients with refractory anemia, 11 patients with refractory anemia with sideroblasts and 5 patients with refractory anemia with excess of blasts. The mean survival was 19 months, 9 patients died: death was due to infection or hemorrhage in 7 cases and to evolution to acute myelogenous leukemia (AML) in 2 cases (10.5%). Thrombocytopenia, the Bournemouth scoring system and FAB classification were independent prognostic factors.
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PMID:[Myelodysplastic syndromes. A clinico-biological analysis of 27 cases]. 207 37

This study of acute leukaemia in adult at the two main hospitals in Dakar- the General Hospital from January 1980 to June 1986 and the A. Le Dantec Hospital from January 1978 to June 1986 - shows the progression of this ailment, which remains rare (0.85% of admissions - 5 cases per year). Certain clinical peculiarities are stressed (high frequency of infectious symptoms, considerable splenomegaly , adenopathy often present in acute myeloid leukaemia. More severe biological signs are evident than in series in the West, and (L.A.M.3) forms dominate. Grave developments are often the result of a shortage of diagnostic and therapeutic equipment. The authors stress the need to create a haematological clinic.
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PMID:[Acute leukemia in Dakar. Retrospective study apropos of 36 cases]. 249 86

A patient is reported who presented simultaneously with two distinct forms of leukemia: chronic lymphocytic leukemia (CLL) and acute myeloid leukemia (AML). The diagnosis of CLL was supported by the presence of lymphocytosis, lymphadenopathy, and splenomegaly with mature B-cell markers on the population of lymphoid cells in the bone marrow. AML was documented by the presence of circulating blast cells and 50% blasts with myeloid markers and Auer rods in the bone marrow. A complete remission from both forms of leukemia was obtained after treatment for the AML. The patient also experienced the temporal occurrence of venous thrombosis for the first time at the age of 69 years, shortly before the diagnosis of leukemia. Protein C deficiency was documented and may have been hereditary as suggested by the strong family history of thrombosis or alternatively may have been the consequence of one of the leukemias.
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PMID:Concomitant chronic lymphocytic leukemia, acute myeloid leukemia, and thrombosis with protein C deficiency. Case report and review of the literature. 264 7

We determined in 35 patients with severe thrombocytopenia (AML n = 10; ALL n = 4; CML = 1; idiopathic myelofibrosis n = 1, aplastic anemia n = 1; undergoing bone-marrow transplantation n = 17) factors influencing the corrected count increment (CCI) after platelet transfusions. Out of 195 transfusions 86 (44%) failed to increased platelet counts (CCI less than 5 X 10(9) platelets/l). A significant percentage of transfusion failures occurred in patients with splenomegaly and/or fever (54% vs. 29%; p less than 0.002). Antibodies directed against donor platelets were found only twice. No correlation between reactivities demonstrable by the lymphocytotoxic test (n = 144) or the radioimmune antiglobulin test (n = 67) and the CCI was obvious. HLA antigen identity was also not predictive. Thus, transfusion failures in patients with low alloimmunization will not be predicted by in vitro antibody screenings. The patients' clinical condition has the most important influence on posttransfusion increment.
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PMID:[Thrombocyte transfusion: increase in platelets in relation to clinical and immunologic prerequisites]. 329 59

Neurological involvement in acute myeloid leukaemia has become more common in recent years. The increase seems to be related to the longer survival rates made possible by more intensive treatment protocols. The predictive elements appear to be the tumour mass, cytomorphological variety M5, splenomegaly and serum LDH. Prophylaxis with craniospinal radiotherapy or spinal chemotherapy does not modify the course of the leukaemia or diminish the frequency of neuromeningeal complications. Six cases of AML involving the central nervous system were examined, two at onset and 4 at first relapse. Neuromeningeal complications are to be feared since they are extremely difficult to eradicate completely and the prognosis is extremely unfavourable especially if a bone marrow relapse occurs simultaneously.
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PMID:[Neurological complications of acute myeloid leukemia in adults]. 345 91

To identify adults with acute nonlymphocytic leukemia at risk for the development of central nervous system involvement, we performed periodic cerebrospinal fluid examinations on patients in remission. Among 58 consecutive patients monitored during first remission, central nervous system leukemia developed in nine (16 percent). Four patients, including one who was symptomatic, had central nervous system leukemia detected simultaneously with marrow relapse. Five additional patients were asymptomatic and continue to have bone marrow remission. Following central nervous system and systemic treatment, two of these five patients have never had relapse, and three had relapse in the bone marrow five, 10, and 21 months later. Factors at diagnosis associated with the subsequent development of central nervous system leukemia were elevated leukocyte count, serum lysozyme and lactate dehydrogenase, extramedullary infiltration including splenomegaly, and monocytic (FAB M4 or M5a) morphology. In six of 17 patients (35 percent) with monocytic morphology, central nervous system leukemia developed compared with only three of 41 patients (7 percent) with other subtypes (p = 0.02). Discriminant analysis identified leukocyte count, splenomegaly, and M4 or M5a morphology as the most important risk factors and led to a mathematical formula that correctly identified 90 percent of the patients. Although the risk of central nervous system leukemia in adults with acute nonlymphocytic leukemia is too low to justify routine prophylaxis, those patients recognized to be at a greater risk should receive prophylaxis or be monitored closely with periodic lumbar punctures.
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PMID:Central nervous system involvement in acute nonlymphocytic leukemia. A prospective study of adults in remission. 366 83


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