Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A two-year-old girl presenting with de novo acute myelomonocytic leukemia with eosinophilia (French-American-British [FAB] classification, M4Eo) and inv(16)(p13q22), t(1;16)(q32;q22) involving the same chromosome 16 is described. This is the second report of a variant translocation of an inverted chromosome 16 with chromosome 1 at 1q32. However, the segment 1q32----1qter has been exchanged for 16q22----qter and not 16p13----pter, as reported in the previous case. The additional break at 1q32 and the juxtaposition of 1q32----qter onto chromosome 16 could be relevant to the pathogenesis of the disease.
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PMID:Acute myelomonocytic leukemia with bone marrow eosinophilia and inv(16)(p13q22),t(1;16)(q32;q22). 199 8

A 78-year-old woman with acute myelogenic leukaemia (AML M5 (FAB)) was treated with standard induction chemotherapy followed by recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) (250 micrograms/m2/day) in an effort to accelerate neutrophil recovery. After 10 days of rhGM-CSF therapy, increasing numbers of promonocytes and monocytes were detected in the peripheral blood, with a maximum total white blood count of 14,900/microliters of which 39% were promonocytes, 39% monocytes, and only 3% neutrophils. The bone marrow during GM-CSF therapy was hypercellular and contained 95% monocytic forms. After discontinuation of rhGM-CSF, this monocyte lineage stimulation was completely reversible. Without further chemotherapy the patient entered a complete remission after 9 months and is now relapse free after 24 months. Since the stimulation was restricted to the previously leukaemic lineage of this patient, the profound monocytosis observed in this case suggests the possibility that GM-CSF may exert reversible effects on the proliferation of clonogenic cells in acute monocytic leukaemia.
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PMID:Reversible leukaemic regrowth under GM-CSF treatment after chemotherapy for AML. 199 44

Serum interleukin-2 (IL-2), soluble IL-2 receptors (sIL-2R) and tumor necrosis factor-alfa (TNF-alpha) levels were determined in 66 previously untreated consecutive patients with acute myeloid leukemia (AML) and in 22 normal volunteers. The following mean (+/- SE) values were observed in patients and controls, respectively: 35 +/- 14.7 (range 0.5-500) and 0.7 +/- 0.02 (0.5-0.8 U/ml for IL-2 (p = 0.001); 1622 +/- 289 (110-10,600) and 422 +/- 30 (207-666) U/ml for sIL-2R (p = 0.0001); 1247 +/- 196 (218-4672) and 152 +/- 11 (75-308) pg/ml for TNF-alpha (p = 0.0001). With respect to the FAB classification system, we found a significantly different distribution of serum IL-2 mean values in distinct subcategories, i.e. 3.4 +/- 1.9 U/ml in M1-M2-M3 and 42.4 +/- 20.4 U/ml in M4-M5 subgroups, respectively (p = 0.01), whereas sIL-2R and TNF-alpha levels were 1144 +/- 322 U/ml and 1120 +/- 317 pg/ml in M1-M2-M3 patients and 1945 +/- 317 U/ml and 1270 +/- 259 pg/ml in the M4-M5 group. A significantly positive correlation between TNF-alpha and sIL-2R (r = 0.53; p = 0.002) was also detected in the M4-M5 group. Sixty-three out of 66 patients received an intensive chemotherapy program. Univariate analysis showed that age and sIL-2R greater than 2000 U/ml significantly affected both complete remission rate and overall survival, whereas by multivariate analysis, age was the only independent variable significantly influencing survival. These data confirm recent in vitro evidence suggesting the role of IL-2, sIL-2R, and TNF-alpha in the control of normal hematopoiesis and leukemogenesis. Since the availability of recombinant cytokines for clinical use in AML, it is crucial to understand their spectrum of interaction in order to select the appropriate combination for in vivo administration.
Leukemia 1991 Jan
PMID:Serum interleukin-2 (IL-2), soluble IL-2 receptors and tumor necrosis factor-alfa levels are significantly increased in acute myeloid leukemia patients. 199 55

We report a case of microgranular acute promyelocytic leukemia (APL, M3 V) presenting with the typical features of disseminated intravascular coagulopathy and cells with irregular, folded, or bilobed nuclei with occasional intracytoplasmic multiple Auer rods in the peripheral blood. A cytogenetic study of the bone marrow and blood showed translocation between chromosomes 15 and 17, characteristic of APL. The flow cytometric study also confirmed this diagnosis. The unusual feature in this case is the existence of 80% hand-mirror cells, which also contain multiple Auer rods, in the bone marrow. The hand-mirror variant of acute leukemia has been frequently encountered in acute lymphoblastic leukemia, but documented in only six cases of acute myelogenous leukemia, including the FAB groups of M1, M2, M4, and M5. This patient is, to our knowledge, the first reported case of a hand-mirror variant in microgranular APL. The mechanism of hand-mirror cell formation and its prognostic implication are discussed.
Leukemia 1991 Mar
PMID:Hand-mirror variant of microgranular acute promyelocytic leukemia. 201 82

It has been suggested that circulating immune complexes (CIC) favor tumor progression by suppressing the host's immune response to malignant cells via blocking factors to cell-mediated cytotoxicity. We prospectively measured CIC by the C1q binding assay in 100 untreated patients with acute myeloid leukemia (AML) de novo. The median CIC level was 135, the range 0-1000, and the mean +/- standard error (SE) 175 +/- 18 micrograms/ml. Sixty-eight patients, termed abnormal, had C1q binding levels greater than 2SE above the mean of the normal population (61 +/- 15 micrograms/ml). There were no significant differences between the 32 patients with normal CIC and the 68 with abnormally elevated CIC in any pretreatment characteristic: gender, age, white blood cell count (WBC), platelets, leukemia cell mass, LDH, immunoglobulins, or fibrinogen. Abnormal CIC levels did not correlate with FAB morphology, the presence of a clonal chromosomal abnormality (76% of all patients), or with specific cytogenetic subgroups, although nine of 11 patients with acute promyelocytic leukemia and t(15;17) had abnormal CIC. There were no significant differences in complete remission (CR) rates after the first chemotherapy course (45 vs 40% for normal vs abnormal CIC) or after all courses of treatment (55 vs 65%). Survival from diagnosis was not significantly different for the normal and abnormal groups (9.3 vs 5.8 months, p = 0.24), but survival after achieving a CR was markedly longer for those with normal pretreatment CIC (33.8 vs 11.7 months, p = 0.0068). Pretreatment CIC strongly correlated with remission duration for the 59 patients who achieved CR (16.5 months for 17 normal patients vs 6.9 months for 42 abnormal patients, p = 0.0002). This was independent of age, WBC, leukemia cell mass, or FAB morphology. Within the lowest C1q quartile (less than 60 micrograms/ml), 43% of the patients have not relapsed with a minimum follow-up of 18 months compared to only 6-14% for the three higher quartiles. We conclude that host immunity as assessed by CIC levels has little effect on the initial response to therapy but may play a role in maintaining remission in AML.
Leukemia 1991 Feb
PMID:Circulating immune complexes correlate with remission duration in acute myeloid leukemia. 202 Jan 95

We treated 47 adult patients with de novo myelodysplastic syndrome (MDS) by an anthracycline-AraC regimen. Median age was 54, and M/F 1.3. At diagnosis, 26 patients had refractory anaemia with an excess of blasts in transformation (RAEB-T) three had refractory anaemia (RA), 11 had refractory anaemia with excessive blasts (RAEB) and seven had chronic myelomonocytic leukaemia (CMML). Treatment was started within 3 months of diagnosis in 30 patients, and after more than 3 months in the 17 remaining patients. At the onset of treatment, 16 patients had progressed to acute myeloid leukaemia (AML). Twenty-two patients (47%) reached complete remission (CR), 10 (21%) had hypoplastic death and 15 (32%) had resistant disease. Median actuarial disease-free interval was 11 months. Median actuarial survival was 14 months from diagnosis and 10 months from the onset of treatment. A significantly higher CR rate was found in patients with RAEB-T at diagnosis (69% v 19% in patients with other FAB subtypes: P = 0.008), and in patients treated within 3 months of diagnosis. Using multivariate analysis, RAEB-T at diagnosis emerged as the most powerful prognostic factor of CR achievement. Karyotype was the only significant prognostic factor of disease-free interval, with a median of 16.5 months in patients with normal karyotype versus 4 months in patients with normal findings (P = 0.018). A subgroup of 15 patients with RAEB-T at diagnosis and normal karyotype, who had a CR rate of 80% and a median actuarial disease-free interval of 18 months, could be identified. Our results confirm that, overall, intensive chemotherapy has limited efficacy in MDS, especially when compared with allogeneic bone marrow transplantation (BMT). Relatively favourable results were obtained in our patients with RAEB-T at diagnosis, however, particularly those with normal karyotype. In that subgroup, intensive chemotherapy may be recommended, especially before BMT, as a high risk of relapse after BMT in patients with RAEB-T allografted as first line therapy has been reported.
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PMID:Prognostic factors in adult de novo myelodysplastic syndromes treated by intensive chemotherapy. 202 75

The need for reproducibility in the classification of acute leukaemia has made it necessary to incorporate information derived from new techniques which have become essential for the study of these disorders. In addition to classic morphology and cytochemistry (FAB proposals), it is necessary to add immunology and cytogenetics (MIC proposals), as well as to investigate further the biological and diagnostic significance of molecular events. As a result of these investigations a new group of leukaemias merit recognition as distinct entities. These include three types of ALL with specific chromosome abnormalities, namely, i) t (9;22), ii) t (4;11) and iii) t (1;19) and four subtypes of AML, i) with minimal differentiation or AML-M0, ii) with basophilic precursors or M2Baso, iii) AML (M4/M5) with t (8;16) and iv) AML with trilineage myelodysplasia. Biphenotypic acute leukaemia constitutes also a distinct entity with features of ALL and AML and represents a malignancy probably affecting multipotent stem cells. We propose an objective evaluation system for biphenotypic leukaemias based on a score in which the various lineage markers are graded according to their known specificity.
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PMID:A classification of acute leukaemia for the 1990s. 203 64

A small number of patients positive for the human immunodeficiency virus (HIV) have been reported as developing acute non-lymphoblastic leukaemia (ANLL) and none has achieved remission despite attempts at treatment. We report on a 34-year-old HIV positive heterosexual intravenous drug user who presented with ANLL (FAB classification M5, Monoblastic) and entered remission following one cycle of cytosine arabinoside and daunorubicin according to the 7-3 protocol of the Australian Leukaemia Study Group (ALSG). This was followed by consolidation of 5-2 as per ALSG and one cycle of maintenance with low dose cytosine arabinoside. Ten months after remission, he relapsed but achieved a second remission with the ALSG 7-3-7 protocol (7-3 plus etoposide) followed by consolidation with 5-2-5. He remained HIV positive but showed little progression towards the acquired immunodeficiency syndrome despite the intense immunosuppression. The duration of his second remission was five months. The patient died of septicaemia during the third attempt at remission induction 18 months after diagnosis. We conclude that HIV seropositivity is not an absolute contraindication to aggressive chemotherapy in those patients who develop ANLL.
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PMID:First and second complete remissions in a HIV positive patient following remission induction therapy for acute non-lymphoblastic leukaemia. 203 79

In 153 consecutive patients with myelodysplastic syndrome (MDS) the prognostic value of FAB-classification, cytogenetics, Bournemouth score, a history of previous radio- or chemotherapy and in vitro bone marrow growth were retrospectively analysed, for both acute nonlymphocytic leukaemia (ANLL) development and survival. Thirty-eight of the 153 patients (25%) showed progression to ANLL, 63 (41%) died during the myelodysplastic phase due to infection or bleeding and three (2%) received allogeneic bone marrow transplantation (BMT). Univariate analysis showed that the FAB-classification, in vitro growth pattern and differentiation, and cytogenetics had a predictive value for ANLL development and survival. The Bournemouth score was predictive only for survival. Most predictive for the development of ANLL were in vitro growth pattern and maturation. Patients with normal in vitro growth progressed to ANLL in 6% of the cases, in patients with hypoplastic or leukaemic growth 32.5% developed ANLL (P less than 0.0001). The ANLL incidence in patients with normally differentiated in vitro colonies was 14.5%, compared with a 52% incidence in cases showing no in vitro cell maturation (P = 0.001). The combination of growth pattern and differentiation revealed an ANLL incidence of 4.2% in cases of normal growth and differentiation, and 60.4% if the in vitro growth and/or differentiation was abnormal (P = 0.006). In vitro maturation was the only parameter predictive for ANLL development in multivariate analysis. From our data it is concluded that the predictive value of in vitro bone marrow culturing in patients with MDS can be increased by including in vitro maturation as a distinct parameter. The in vitro prognostic data can be important in selecting MDS patients for intensive chemotherapy or BMT.
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PMID:In vitro growth pattern and differentiation predict for progression of myelodysplastic syndromes to acute nonlymphocytic leukaemia. 204 79

Clinical and biological features were assessed in 114 consecutive previously untreated adult acute myeloid leukaemia (AML) patients whose diagnosis was based on FAB criteria and detailed immunophenotyping. All patients received standard intensive chemotherapy. The main purpose of this study was to establish the prognostic value, if any, of terminal transferase (TdT) expression in myeloid leukaemia. TdT positive cells (7-80% of total blast cells) were detected in 40% of the cases. Among clinical characteristics, a low lactate dehydrogenase (LDH) (less than 250 I.U.) (P = 0.003), a low initial white blood cell count (less than 10 x 10(9)/l) (P = 0.002), and an absolute neutrophil count (less than 5 x 10(9)/l) (P = 0.02) were associated with TdT-positivity. FAB classification was not predictive of TdT expression, and there was no difference in the distribution of FAB subtypes between the groups. Multivariate analysis combining clinical and laboratory data indicated that a low expression of the monocytic antigen CD14 was predictive of TdT positivity in AML (P = 0.01). Karyotyping showed no difference in the pattern of occurrence of specific abnormalities between the TdT+ and the TdT- group. When clinical and immunophenotype data were included in a prognostic model, the patient's age was highly predictive of response (P less than 0.001), and only the CDw65 antigen contributed to the response model (P = 0.07). TdT+ patients with a low expression of CD11b achieved a higher frequency of response at a borderline level of significance (P = 0.06). Frequency of response to chemotherapy, the response duration or overall survival were not influenced by TdT expression.
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PMID:Terminal transferase expression in acute myeloid leukaemia: biology and prognosis. 204 81


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