Survival in chronic myelogenous leukemia: Influence of treatment and extent of disease at diagnosis

Monfardini, S.; Gee, Timothy; Fried, Jerrold; Clarkson, Bayard D.

doi:10.1002/1097-0142(197303)31:3<492::aid-cncr2820310302>3.0.co;2-4

Cited by 60 publications

(6 citation statements)

References 21 publications

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“…12 In the same review, the median survival of patients with Ph negative, bcr/abl positive CML was 50 months, a survival similar to that reported for patients with Ph positive CML. [53][54][55][56][57][58][59][60][61] This finding provides further support for the notion that Ph negative, bcr/abl positive CML is indistinguishable biologically and clinically from Ph positive CML. 14,20,32,51,62 In patients with Ph negative CML, we 22 and others 16,24 have reported features that distinguished Ph negative CML from Ph positive CML: older age, male predominance, higher incidences of anemia and thrombocytopenia, low incidence of basophilia, and lower WBC counts.…”

Section: Discussionmentioning

confidence: 57%

Characteristics and outcome of patients with Philadelphia chromosome negative,bcr/ablnegative chronic myelogenous leukemia

Onida

Ball

Kantarjian

et al. 2002

Cancer

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BACKGROUNDUp to 5% of patients with chronic myelogenous leukemia (CML) do not have the Philadelphia (Ph) translocation t(9;22)(q34;q11) or a bcr/abl molecular rearrangement. Although the diagnostic criteria of this entity are still under debate, there is general agreement that patients with Ph negative, bcr/abl negative CML have a severe clinical course that is not affected significantly by current treatment options.METHODSA population of 76 patients with bcr/abl negative CML who had received minimal or no previous therapy was characterized carefully with the intent of investigating clinical and hematologic variables and their association with survival by univariate, correlation, and multivariate analyses. A group of 73 patients with Ph negative CML who were not tested for the bcr/abl rearrangement (bcr/abl unknown) was analyzed separately and used for extension of the analysis.RESULTSIn the bcr/abl negative patient population, the median overall survival was 24 months. At the time of the analysis, 38 patients (50%) had died, and blastic transformation preceded death in 31%. Chromosomal abnormalities were found in 30% of the 76 patients, with trisomy 8 the most common abnormality. Complex chromosomal abnormalities were rare, and monosomy 7 was not observed. Survival was not affected significantly by treatment. Multivariate analysis identified older age (> 65 years), anemia (hemoglobin < 10 g/dL), and severe leukocytosis (white blood cells > 50 × 109/L) as variables with independent prognostic significance for poor survival. A prognostic scoring system stratified patients into a low‐risk group (53%) and a high‐risk group (47%), with median survivals of 38 months and 9 months, respectively.CONCLUSIONSBcr/abl negative CML is a distinct clinical entity associated with very poor prognosis. Two risk categories are identifiable using a simple scoring system based on age, hemoglobin level, and leukocyte number. Cancer 2002;95:1673–84. © 2002 American Cancer Society.DOI 10.1002/cncr.10832

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Section: Discussionmentioning

confidence: 57%

Characteristics and outcome of patients with Philadelphia chromosome negative,bcr/ablnegative chronic myelogenous leukemia

Onida

Ball

Kantarjian

et al. 2002

Cancer

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“…However, in the pooled series patients presenting with an H b level higher than 10 g/dl survived slightly longer than the others. A relationship of H b to survival was suggested by Theologides (1972) and Jacquillat et a1 (1975), but not by Monfardini et al(1973). We attempted to include H b level in the panel ofprognostic factors, but the ensuing prognostic classification became less sharp.…”

Section: Propiostic Factorsmentioning

confidence: 99%

Staging of Chronic Myeloid Leukaemia

Tura¹,

Baccarani²,

Corbelli

1981

Br J Haematol

113

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The prognostic value of nine clinical and haematological features, recorded at diagnosis in chronic myeloid leukaemia (CML), was analysed in two distinct series of patients. One series (116 cases) was collected at a single hospital over a 12-year period. The second series (139 cases) was collected from a multicentre trial over a 20-month period. Six features were associated with a poor prognosis: splenomegaly (more than 15 cm below the costal margin), hepatomegaly (more than 6 cm below the costal margin), thrombocytopenia (< 150 X 10(9)/l) or thrombocytosis (> 500 X 10(9)/l), a leucocyte count above 100 X 10(9)/l, peripheral blood non-granulated precursors (blast cells) above 1%, and peripheral blood granulated precursors (promyelocytes and myelocytes) above 20%. Depending on the number of negative prognostic factors, patients were divided into three categories: group I (0 or 1 factor), group II (2 or 3 factors) and group III (4,5 or 6 factors). Survival was significantly different in the three groups (P < 0.0005), and this was independent of age (below and above 50). The prognostic value of the classification was confirmed in a third series of 153 patients. We suggest that this classification provides a useful tool to identify prognostic categories in CML, and thus allows a proper allocation of patients to different therapies.

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“…'- 17 Several recent studies have analyzed the prognostic factors in Ph-positive CML resulting in statistical models that predict patients' o~t c o r n e .~~~~" Variables adverse for survival included, among others, old age, the presence of organomegaly, weight loss, anemia, thrombocytopenia or thrombocytosis, and high blast or basophil counts in the peripheral blood and bone marrow. [2][3][4][5][6]10 Ph-negative CML represents only 10% to 15% of the total CML population. "-'8 A careful analysis of this entity has been hampered by its rarity and the small number Of patients referred to any single institution.…”

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Clinical and prognostic features of philadelphia chromosome-negative chronic myelogenous leukemia

Kantarjian

Keating

Walters

et al. 1986

Cancer

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Between 1965 and 1982, 105 patients with a diagnosis of Philadelphia chromosome-negative chronic myelogenous leukemia were referred to our institution with minimal or no prior therapy. The median age was 63 years and 64% were males. The overall median survival from time of referral was 14 months; 53% of patients survived 1 year and only 10% survived beyond 5 years. At the time of analysis, 92 patients (88%) were dead, 56% of deaths being preceded by a blastic crisis. Compared with Philadelphia chromosome positive disease, patients with Philadelphia chromosome-negative chronic myelogenous leukemia were older and had a significantly higher incidence of anemia, thrombocytopenia, monocytosis, marrow blasts, decreased marrow megakaryocytes and a lower incidence of basophilia and thrombocytosis. Chro-mosomal abnormalities occurred in 33% of patients and consisted most frequently of trisomy 8, or an additional chromosome C, loss of the Y chromosome, or abnormalities in chromosomes #5 and #7. Of nine pretreatment characteristics significantly associated with poor survival, a multivariate analysis identified four to have independent additive prognostic significance: severe thrombocytopenia, hemoglobin levels less than 10 g/dl, increasing peripheral blasts and promyelocytes, and age 60 years or older. Monocytosis was not of prognostic significance. The derived prognostic model divided patients into three risk groups, low, intermediate, and high, with median survivals of 36,16, and 3 months, respectively. The authors conclude that Philadelphia chromosome-negative chronic myelogenous leukemia is a distinct entity among the myeloproliferative syndromes with characteristic clinical and laboratory features and a poor prognosis. Prognostic factors and related risk categories were demonstrated within this disease entity.

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Survival in chronic myelogenous leukemia: Influence of treatment and extent of disease at diagnosis

Cited by 60 publications

References 21 publications

Characteristics and outcome of patients with Philadelphia chromosome negative,bcr/ablnegative chronic myelogenous leukemia

Characteristics and outcome of patients with Philadelphia chromosome negative,bcr/ablnegative chronic myelogenous leukemia

Staging of Chronic Myeloid Leukaemia

Clinical and prognostic features of philadelphia chromosome-negative chronic myelogenous leukemia

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