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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The value of routine bone marrow examination (RBME) in children during and after treatment for standard risk acute lymphoblastic leukemia (SR-ALL) was investigated. The clinical symptoms and peripheral blood findings at the time of bone marrow relapse of 28 children were reviewed and compared with those of 28 matched controls in continuous complete remission. Five (45%) children with bone marrow relapse during maintenance therapy and six (35%) after cessation of cytostatic treatment were asymptomatic at the time of relapse. Signs indicative of relapse during treatment were lymphoblast cells in the peripheral blood, thrombocytopenia, hepatomegaly, anemia, or leukopenia in decreasing order of frequency. After cessation of treatment these signs were lymphoblasts in the peripheral blood, hepatomegaly, splenomegaly, thrombocytopenia, or leukocytosis. Except for one case with thrombocytopenia, no signs suspicious for relapse were found in the control groups. When each sign was evaluated separately only the presence of lymphoblasts in peripheral blood and hepatomegaly were significant symptoms for relapse after cessation of treatment. The mean percentage of lymphoblasts in the bone marrow at the time of relapse was significantly lower for patients with an unpredicted relapse (46.8%) than patients with clinical and/or laboratory evidence of relapse (79.5%). When lymphoblasts were present in the peripheral blood the percentage of lymphoblasts in the bone marrow was always more than 40%, both during and after cessation of treatment. These data suggest a relation between clinical and laboratory symptoms and progression of the disease. It is concluded that 46% of relapses are detected by RBME in the absence of clinical or laboratory symptoms. This early detection may have a positive prognostic influence with more effective treatment for relapsed ALL.
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PMID:Value of routine bone marrow examination for detection of bone marrow relapse in children with standard risk acute lymphoblastic leukemia. 155 75

Thirty-one patients with newly diagnosed acute lymphoblastic leukemia were examined before receiving any treatment and their clinical and laboratory data were analyzed in order to determine the possible correlation between clinical presentation, morphologic sub-classes, cytochemical reactions, immunological phenotypes and cytogenetic findings. Each of the previous parameters and response to therapy were also examined for correlation. The analysis of clinical and laboratory characteristics of patients according to their immunological phenotype did not show any significant male sex bias, age distribution, hepatomegaly or splenomegaly at diagnosis. The analysis of clinical response of patients did not demonstrate any significant influence of sex, age, initial WBC count or the presence of a big tumor mass at diagnosis. There were no significant differences between our two major immunological subclasses Non-T CALLA+ ALL, and Pre-T ALL regarding proportions of patients in continuous remission, and relapse-free survival durations. The analysis of clinical and laboratory characteristics of patients on the basis of their chromosome categories did not show any significant sex bias, age distribution, initial WBC count, tumoral presentation or morphological subtypes at diagnosis, although there was an apparent male predominance in the pseudodiploid category and female predominance in the hyperdiploid category. Our results concerning the prognostic implication of CALLA were contradictory to those of several other investigators.
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PMID:The prognostic implications of an immunological classification of acute lymphoblastic leukemia. 279 26

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

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

5 cases of ALL are reported with a t(4;11) chromosomal rearrangement, and 31 cases, investigated before therapy, are reviewed. The (4;11) translocation characterizes a subentity of ALL having the following main features, as compared with ALL in general: 1) Excessively poor prognosis, with a median survival of 6 months despite high remission rates. 2) Low median age of 10 months in children. 3) CNS involvement apparently occurs more frequently. 4) Median WBC is 12 times higher in adults and children, median per cent of blasts in blood 1.5 times higher in adults and 1.8 times higher in children. 5) Splenomegaly is present more frequently. 6) Surface markers are non-B, non-T. The incidence of t(4;11) in ALL varies greatly in the series published so far, from 0.04% to 12%.
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PMID:Acute lymphocytic leukaemia with t(4;11): a clinical subentity. 386 33

Eighty-seven adult patients who had achieved bone marrow remission of leukemia developed one or more episodes of meningeal leukemia. Multiple patient characteristics were examined for their effect on probability of achieving complete remission from meningeal disease and for their effect on duration of meningeal remission. Presence of obtundation (P less than 0.01) or other symptoms of meningeal disease (P = 0.02) were associated with a low remission induction rate. Other factors which tended (P = 0.06-0.20) to be associated with low remission induction rates included high cerebrospinal fluid (CSF) opening pressure, absence of splenomegaly at initial diagnosis, high peripheral blood leukocyte count (WBC) at the episode of marrow disease most recently preceding the meningeal disease, and use of only one as opposed to two or more intrathecal drugs as treatment. Factors associated with long duration of meningeal remissions included diagnosis (AML greater than acute undifferentiated leukemia greater than ALL, P = 0.05), absence of symptoms (P = 0.04), low CSF WBC (P = 0.01), rapid attainment of meningeal remission (P = 0.01), rapid attainment of initial bone marrow remission (P = 0.02), and long duration of initial bone marrow remission (P less than 0.01). Absence of cranial or peripheral neuropathies, low CSF protein and opening pressure, and short time interval between diagnosis of marrow and meningeal disease also tended (P = 0.06-0.20) to be associated with long meningeal remissions. Patients treated according to an intensive protocol utilizing cranial irradiation and triple drug treatment via an Ommaya reservoir had substantially longer meningeal remissions than did patients treated with less intensive therapy (P = 0.01). Relapse-free survival curves suggest that some patients are cured of their meningeal disease.
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PMID:Remission from central nervous system involvement in adults with acute leukemia. Effect of intensive therapy and prognostic factors. 389 Oct 72

One-hundred-one adult patients with ALL were analyzed to determine the prognostic implications of splenomegaly occurring at any time during the course of their illness. The clinical status of the spleen at presentation was not found to be of major prognostic significance. Complete response rates, remission durations, and survivals did not differ between patients with and without splenomegaly at presentation. An enlarged spleen accompanied relapse in four patients. In six additional patients, splenomegaly was present during complete remission, and splenectomies performed in five of these patients revealed no evidence of leukemia to account for the splenomegaly. Splenectomy does not appear to be detrimental, as all five patients are currently in complete remission from 20 to 63 mo after splenectomy. Evidence implicating the spleen as a source of an antibody directed against autologous leukemia cells in one patient is reviewed.
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PMID:The significance of splenomegaly in 101 adults with acute lymphoblastic leukemia (ALL) at presentation and during remission. 693 19

In an attempt to establish a possible correlation between clinical course and initial characteristics of the disease, 88 children with ALL (diagnosed between 1970-1978) were studied. Basis for comparison was whether relapses (medullary or extramedullary) occurred within 36 months. Twenty parameters, including history data, physical exploration, laboratory findings and early response to induction treatment, were evaluated. Statistical analysis showed a significant influence of the following factors: age less than 1 year, organomegalies (hepato or splenomegaly larger than 5 cm B.C.M.), adenomegalies (more than 3 cm in diameter), mediastinal mass, initial CNS infiltration, E-rosette forming blast-cells, acid phosphatase positivity and a good hematological response after 2nd week of treatment. Sex appeared as a prognostic factor after 36 months. Three group of patients could be distinguished according to risk factors: A) Patients without risk factors; 70.45% in continuous remission (CR) after 36 months. B) Patients with 1-2: 43.47% in CR at 36 months. C) Three or more: None attained 36 months in continuous CR. Differences are significant (p less than 0.05).
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PMID:[Evaluation of initial risk factors in acute lymphoblastic leukemias in children]. 696 35

CGL is a highly specific disease that is defined by strict hematologic parameters that include a pathognomonic differential leukocyte count. Usually CGL is accompanied by the presence, in bone marrow cells, of the Ph chromosome, the first chromosomal anomaly to be regularly associated with a human neoplastic disease. CGL is predominantly a disease of the productive middle years of life, which maximizes its adverse impact on family life and family economics. The disease is of worldwide distribution and there is a slight male preponderance. The disease is characterized by an initial chronic phase when it behaves as a differentiated neoplasm and responds very well to simple, nonintensive therapy. After a variable interval, CGL undergoes metamorphosis to a refractory phase that responds poorly or sometimes not at all to therapy, even when this is intensive. At the stage of metamorphosis a great variety of clinical and hematologic pictures occur, and CGL may mimic a myeloproliferative disease, a myelodysplasia, a subacute leukemia, AML, or ALL. The old concept of an abrupt, explosive transition from the chronic phase to a so-called blastic crisis is incorrect: this rarely occurs and in most patients who are carefully followed, CGL is observed to undergo two or more stepwise evolutions, eg, from chronic phase to an accelerated myeloproliferative phase to a phase that resembles AML. Many patients with CGL conform to an established pattern of clinical features. There is a history of insidious symptoms of anemia and of splenomegaly. The physical signs are those of pallor and marked splenomegaly, while the hematologic findings are of moderate anemia, moderate thrombocytosis, and a marked granulocytic leukocytosis with a specific differential count. The radiologic findings are typically normal. Diagnostic difficulty seldom arises with this classic presentation. The patient who is detected at an early stage of CGL may lack the history, physical signs, and fully developed hematologic picture of CGL. Before the availability of cytogenetic studies, the diagnosis could only be established with confidence by observing the patient until the typical features of the disease emerged. Also considered are the less frequent but important atypical presentations of CGL. The symptoms and complaints, findings on examination, complications and hematologic findings may depart from the typical case in a bewildering variety of ways, so that the diagnosis may be difficult, indeed, CGL is generally not the initial diagnosis that is made. When the patient with CGL has received treatment, it is usual for he or she to become asymptomatic, with no abnormal physical signs.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Clinical manifestations of chronic granulocytic leukemia. 763 35

Deletions of the long arm of chromosome 6 have been described in acute and chronic lymphocytic leukemia (ALL and CLL) and prolymphocytic leukemia (PLL), and have been associated with t(14;18)(q32;q21) in non-Hodgkin's lymphoma (NHL). Of 55 cases of small lymphocytic (sm lym) NHL, deletions of 6(q21q23) were the most common recurring cytogenetic abnormality. Among 14 sm lym NHL with del(6)(q21q23), this abnormality occurred as a solitary change in 3 cases. Each of these 3 cases, and 5 additional cases with del(6q) and other abnormalities, showed atypical larger forms with the morphologic appearance of prolymphocytes or paraimmunoblasts in the peripheral blood. In comparison, of the 11 cases without del(6q) and circulating abnormal cells, prolymphocytoid forms were observed in 4 cases (P < .001). Of the 31 sm lym without del(6q), trisomies of chromosomes 3, 12, or 18, or t(11;14)(q13;q32) occurred in greater than 10% of cases. Proliferation centers or infiltration by larger forms were observed in similar proportions of tissue sections derived from sm lym NHL with or without del(6q). The presence of the larger forms in the peripheral blood did not have an adverse prognostic impact on the survival of the del(6q) cohort, who experienced a median survival in excess of 6 years. All 14 cases of del(6q) sm lym NHL were characterized by a mature B-cell phenotype. Expression of CD11c, a feature of a CLL/PLL variant previously described, was not detected in 9 cases analyzed. In 5 cases of del(6q) sm lym NHL, no circulating abnormal lymphocytes were noted. Twelve cases presented with, or developed, clinical splenomegaly. These results suggest that deletion of a gene or genes at 6q21-23 is associated with the pathogenesis of a subset of B-cell sm lym NHL that may display larger prolymphocytoid cells in the peripheral blood, but that follows a clinical course typical of other well-differentiated lymphocytic neoplasms.
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PMID:Clinical and morphologic features of B-cell small lymphocytic lymphoma with del(6)(q21q23). 816 42


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