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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three cases of childhood acute lymphatic leukaemia masquerading as juvenile chronic arthritis are presented. All had symptoms and signs for at least 4 months before leukaemia was diagnosed and in two the full blood count was normal at presentation. The importance of a high index of suspicion is emphasized, particularly if the white cell count is low.
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PMID:Arthritic presentation of childhood leukaemia. 192 27

Attention has been focused on two problems of acute leukaemia: (1) the origin of normal-appearing haemopoietic cells during relapse, and (2) the inverse relationship between leukaemic blast cell proliferation and useful haemopoietic cell production. The available evidence suggests that the normal-appearing cells during relapse may not all be remnants of normal haemopoiesis but may at least in part be derived from leukaemic cells. Although a differentiation defect is a major characteristic of acute leukaemia, it seems as if this defect is not absolute: some cells may succeed in differentiating more or less normally in spite of their descent from a leukaemic stem cell. Acute leukaemia is usually considered to be a primary white cell disorder which indirectly affects the other haemopoietic cell lines. It appears more likely, however, that acute leukaemia, at least the myeloid type, is a disorder of a stem cell common to granulocytopoiesis, erythropoiesis, and probably thrombocytopoiesis. Most descendants from the diseased stem cell fail to differentiate and remain at the blast cell level where they proliferate for some time; however, at a certain point proliferation ceases and the cells ultimately die. Another fraction of the progeny of the leukaemic stem cells may differentiate to some extent and may give rise to functionally useful cells. This is analogous to chronic myeloid leukaemia. The mechanism by which useful haemopoiesis apparently is suppressed in the presence of leukaemic blast cells has remained enigmatic so far. Previously suggested explanations which all assume some kind of cell-cell interaction by which normal haemopoietic cells succumb have neither been proved nor disproved. In this chapter, a new hypothesis is presented. It is assumed that some normal haemopoietic stem cells enter a dormant state at various distances in lineage from the fertilized ovum ('sleepers'). Another fraction of haemopoietic stem cells ('feeders') are actively proliferating and serve to feed the differentiating haemopoietic cell lines and to maintain the 'feeder' pool. When the 'feeder' pool is exhausted, a 'sleeper' cell is activated and sets up a new 'feeder' clone. Otherwise, 'sleepers' are protected against acting as 'feeders' in order to keep 'sleeper' divisions at a minimum and thus preserve their genetic information as intact as possible. It is suggested that the leukaemic event initially takes place in one or a few 'sleepers'. If the leukaemic 'sleeper' never succeeds in setting up a 'feeder' clone, clinical leukaemia will not develop. Clinical leukaemia will result if a leukaemic 'sleeper' establishes a leukaemic 'feeder' pool.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Acute leukaemia: development, remission/relapse pattern, relationship between normal and leukaemic haemopoiesis, and the 'sleeper-to-feeder' stem cell hypothesis. 195 81

The immunophenotype of leukemicblasts from 111 patients with T-ALL or T-NHL were further examined by using a panel of standardized McAbs of CD nomenclature to human leukocyte differentiation antigens. Four major subsets of T-ALL were defined: pre T-ALL, immature T-ALL (I), common T-ALL (II) and mature T-ALL (III), with the percentages 20.7%, 20.7%, 20.7% and 37.0% respectively. In addition there was a case with M-T acute hybrid leukemia. Some of the clinical features of the patients with T-ALL and T-NHL were compared. It was found that male predominance, older age, higher leukocyte count, lower platelet level, relative higher hemoglobin level and increased incidence of extramedullary involvement, including hepatomegaly, splenomegaly and lymphadenopathy were alike for all subsets of T-ALL cases. However, the average white cell level and incidence of lymphadenopathy in the pre T-ALL subset significantly differed from those in other subsets. The correlation of immunophenotype with morphologic characterization was also discussed in this paper.
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PMID:[Correlation of immunophenotype with clinical features in T-cell acute lymphoblastic leukemia]. 203 95

During the 1970s, despite apparently similar treatment, the prognosis for children with lymphoblastic leukaemia (ALL) improved more in some countries, notably the United States and West Germany, than in others. To find out why, the first phase of the United Kingdom (UK) Medical Research Council (MRC) childhood ALL trial, UKALL VIII, was designed to see whether similar results to the United States Children's Cancer Study Group (CCSG) could be obtained in the U.K. using an identical protocol (CCG 162). Protocol 162 was one of a series of regimens devised by the American Children's Cancer Study Group in the 1970s and was used specifically for their average risk patients (all children with ALL with an initial white cell count up to 50 x 10(9)/l except those aged 3-6 years with white cell counts under 10 x 10(9)/l). One arm (1A) of their study was adopted by the MRC for all children in the U.K. aged 0-14 years with confirmed ALL. Eight hundred and twenty-nine consecutive patients were entered between 1980 and 1984. The first 199 patients formed a single arm study as per the original protocol 162 (arm 1A), but the subsequent 630 children were randomized to receive or not two doses of daunorubicin on the first 2 d of induction. This randomization was an attempt to answer the important question as to whether event-free survival was influenced by the use of four rather than three induction agents. A second randomization between 2 and 3 years continuing therapy was also introduced at this stage as it had been by the CCSG in their protocol. With a minimum follow up period of more than 5 years, disease-free survival for the whole group is 55%, a considerable improvement on all previous UKALL trials. Results for patients directly comparable with those in CCSG 162 ('average risk' patients) and their American counterparts were similar. Daunorubicin was associated with more early deaths but improved disease-free survival for those achieving remission. More children relapsed who stopped treatment after 2 years than those who continued for 3, but this was balanced by increased treatment mortality in the third year. The fact that for UKALL VIII the results were similar to those of the CCSG suggests that previous MRC protocols were not sufficiently sustained and intensive, particularly during the maintenance phase of treatment.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Results of Medical Research Council Childhood Leukaemia Trial UKALL VIII (report to the Medical Research Council on behalf of the Working Party on Leukaemia in Childhood). 206 56

Twenty-nine patients with leukemia were observed for the development of and recovery from oral herpes simplex virus (HSV) lesions. In patients with seropositive test results, lymphocyte and monocyte counts may provide a guide to predict the onset of HSV infections and to indicate when to institute acyclovir prophylaxis. When HSV developed, acyclovir was effective in preventing progression of the lesions, which did not resolve until white cell counts had recovered.
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PMID:Clinical study of herpes simplex virus infection in leukemia. 216 54

Seventy-two consecutive and previously untreated adults with acute non-lymphoblastic leukaemia (ANLL), having a median age of 36 years (range 12 to 71), were prospectively randomised to receive conventional doses of cytosine arabinoside and doxorubicin combined with either etoposide (CTR III) or 6-thioguanine (DAT). Morbidity was comparable between the two regimens and complete remission (CR) rates of 52% and 62% respectively (p greater than 0.50) were not influenced by age above or below 50 years, initial white cell count, French-American-British classification, or race. However, growth pattern in the GM: CFUc assay was found to identify a subgroup of patients who had a significantly higher CR rate. Similarly, the secretion of tissue plasminogen activator by leukaemic blasts in vitro uniformly predicted for primary drug resistance, whereas a CR rate of 68% was associated with production of the urokinase type or a mixture of both enzymes. Remission duration and survival did not differ between these two forms of chemotherapy, nor were they influenced by immunotherapy with C. parvum or the duration of maintenance therapy, whereas age below 50 and the species of plasminogen activator secreted were significant prognostic factors. It is concluded that etoposide can be substituted for 6-thioguanine in these cytosine arabinoside and doxorubicin-containing regimens and that for both combinations the most sensitive prognostic factor for CR and survival is the species of plasminogen activator secreted in vitro by the leukaemic blasts.
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PMID:Chemotherapy of adult acute nonlymphoblastic leukaemia. 220 94

Seven cases of infant acute lymphoblastic leukaemia with t(11; 19) (q23; p13) are described. They are characterized by a high white cell count, organomegaly, early central nervous system (CNS) disease, and a poor prognosis. Blasts are usually of an immature early B-cell lineage although monocytoid features are present in some cases. The characteristics of infant acute leukaemia with t(11; 19) are very similar to those found with t(4; 11), and the presence of t(11; 19) may indicate the same poor prognosis.
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PMID:Infant acute lymphoblastic leukaemia with t(11; 19). 233 35

We have found a single 4p+ chromosomal abnormality, 46,XX, -4, +der(4)t(3;4)(q13.3;p16), in a patient with an unusual B cell leukemia of mature phenotype characterized by a high white cell count, tartrate-resistant acid phosphatase-positive malignant cells, splenic white pulp proliferation, and a serum IgM monoclonal gammopathy. The malignant cells were characterized by surface expression of CD19 (B4), CD20 (B1), IgM, IgD, kappa, and HLA-DR. They were weakly positive for CD21 (B2) and negative for CD25 (interleukin-2 receptor). The malignant cells also showed clonal rearrangement of the immunoglobulin heavy chain and kappa light chain genes. A cell line, designated HCLW-3B, was derived from unstimulated peripheral blood obtained during the leukemic phase and was found to contain the same 4p+ chromosomal abnormality as well as genomic sequences of the Epstein-Barr virus nuclear antigen. A somatic cell hybrid constructed from HCLW-3B containing the derivative chromosome 4 was used to confirm that chromosome 3q was the source of the translocated material. The availability of a cell line which is clonally derived from the patient's circulating leukemia cells should permit further characterization of this translocation at the molecular level.
Leukemia 1989 Sep
PMID:Association of a mature B cell leukemia with a 4p+ chromosomal abnormality: derivation and characterization of a cell line. 254 46

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

Children from the UKALL V trial were studied to assess the clinical importance of myelosuppression during uninterrupted 'maintenance' treatment of 'standard risk' lymphoblastic leukaemia. Those receiving daily 6-mercaptopurine and weekly methotrexate who were in first remission 20 months from diagnosis were divided into two groups on the basis of whether or not they had ever had an absolute neutrophil count of less than 0.5 x 10(9)/l recorded during maintenance treatment up to that time. Of 105 evaluable children, 45 (43%) became neutropenic at least once, and 60 (57%) did not. Seven (16%) of the neutropenic group subsequently relapsed compared with 27 (45%) of the remainder. This difference was still significant if the analysis was stratified by total treatment time (two or three years), age, sex, or diagnostic white cell count. Seven (16%) neutropenic children died in remission, compared with one (2%) of the non-neutropenic children. Therapeutic myelosuppression during standard maintenance treatment of 'standard risk' lymphoblastic leukaemia is associated with increased toxicity but a reduced risk of relapse. The unexplained improvement in long term survival in the United Kingdom in recent years may in large part be due to this.
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PMID:Prognostic importance of myelosuppression during maintenance treatment of lymphoblastic leukaemia. Leukaemia in Childhood Working Party of the Medical Research Council. 268 39


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