血清乳酸脱氢酶水平与初诊老年多发性骨髓瘤病人预后的相关性分析

目的探讨血清乳酸脱氢酶(LDH)与初诊老年多发性骨髓瘤(MM)临床指标的相关性及其预后意义。方法回顾性分析我院2009年7月至2016年1月年龄≥65岁的78例初诊老年MM病人的临床资料,并绘制病人的Kaplan-Meier生存曲线,分析初诊时血清LDH水平与病人预后的关系。根据病人的LDH水平分为LDH正常组和LDH升高组,并根据荧光原位杂交技术检测结果对60例资料完整的MM病人进行修正的国际分期系统(R-ISS)分期,比较其与国际分期系统(ISS)分期对预后判断的准确性。结果所有病人中位随访时间为16.5个月,初诊时LDH水平升高者占11.5%(9/78)。LDH正常组中位生存期(OS)和中位无进展生存期(PFS)分别为44.0个月、23.0个月,LDH升高组分别为14.0个月、12.0个月,2组间比较,差异有统计学意义(P<0.01)。COX多因素回归分析显示,LDH水平升高是老年MM病人OS的独立不良预后因素(HR=5.998,95%CI2.454~14.664,P<0.001)。另外,ISS分期Ⅱ期和Ⅲ期病人的中位OS差异无统计学意义(44.0个月比39.0个月,P=0.713),中位PFS差异也无统计学意义(26.0个月比20.0个月,P=0.569);而R-ISS分期Ⅱ期和Ⅲ期病人的中位OS差异有统计学意义(44.0个月比15.5个月,P<0.001),中位PFS差异无统计学意义(21.0个月比14.0个月,P=0.097)。结论LDH水平是判断老年MM病人预后的重要指标,基于其基础上的R-ISS分期在预后判断中要优于ISS分期。     

Staging of chronic lymphocytic leukemia

One-hundred and eighty-eight patients with chronic lymphocytic leukemia were analyzed for prognosis based on Rai's staging system. It was found that stages I and II were not homogeneous as to prognosis. Stage II patients presenting with isolated splenomegaly had a long survival and were pooled with stage 0 patients (low risk group, 30% of cases, relative death rate 0.24, median survival greater than 10 yr). Stages I and II patients with a lymphocyte count higher than 40 x 10(9)/liter had a short survival and were pooled with stages III and IV patients (high risk group, 39% of cases, relative death rate 1.91, median survival 3.3 yr). Stages I and II patients with a lymphocyte count lower than 40 x 10(9)/liter made up an intermediate or standard risk group (31% of cases, relative death rate 1.00, median survival 6.2 yr). This modified staging system applied successfully to both old and young patients (more and less than 60 yr old, respectively).     

Prognostic factors in agnogenic myeloid metaplasia: a report on 195 cases with a new scoring system [see comments]

We studied the survival of 195 patients with agnogenic myeloid metaplasia (AMM) diagnosed between 1962 and 1992 in an attempt to stratify patients into risk groups. Median survival was 42 months. Adverse prognostic factors for survival were age > 60 years, hepatomegaly, weight loss, low hemoglobin level (Hb), low or very high leukocyte count (WBC), high percentage of circulating blasts, male sex, and low platelet count. A new scoring system based on two adverse prognostic factors, namely Hb < 10 g/dL and WBC < 4 or > 30 x 10(3)/L, was able to separate patients in three groups with low (0 factor), intermediate (1 factor), and high (2 factors) risks, associated with a median survival of 93, 26, and 13 months, respectively. An abnormal karyotype (32 cases of 94 tested patients) was associated with a short survival, especially in the low-risk group (median survival of 50 v 112 months in patients with normal karyotype). The prognostic factors for acute conversion were WBC > 30 x 10(3)/L and abnormal karyotype. Thus, hemoglobin level and leukocyte count provide a simple prognostic model for survival in AMM, and the adverse prognostic value of abnormal karyotype may be related to a higher rate of acute conversion.     

Peripheral blood monoclonal plasma cells as a predictor of survival in patients with multiple myeloma [see comments]

The purpose of this study was to quantitate the number and labeling index of monoclonal plasma cells in the blood of patients with newly diagnosed multiple myeloma (MM) to learn if these values were independent prognostic factors for survival. Patients were candidates for this study if they had untreated myeloma requiring therapy, were evaluated at our institution between 1984 and 1993, and had a sample of blood analyzed with a sensitive immunofluorescence technique for monoclonal plasma cells and the blood B-cell labelling index (BLI). The % blood monoclonal plasma cells (%BPC) and the BLI were analyzed along with stage, marrow plasma cell LI, % marrow plasma cells, calcium, creatinine, albumin, beta-2-microglobulin, and C-reactive protein as univariate and multivariate factors for survival. Eighty percent of the 254 patients accrued to this study had monoclonal BPC detected. The median % BPC was 6% and 57% (144 of 254) of patients had a high number (> or = 4%). Patients with > or = 4% BPC had a median survival of 2.4 years vs 4.4 years for those with < 4% BPC (P < .001). The BLI was also prognostic (P = .008). In a multivariate analysis, the % BPC, age, albumin, stage, marrow plasma cell LI, and the BLI were independent factors for survival. The %BPC and the marrow plasma cell LI best separated the group into low, intermediate, and high risk myeloma with median survivals of 52, 35, and 26 months, respectively. Patients with high %BPC were less likely to have lytic bone disease from their MM (P = .002). The %BPC and the BLI are independent prognostic factors for survival and are useful in identifying patients as low, intermediate, and high risk. Clonal cells in the blood should be quantified in future clinical trials for myeloma.     

Prognostic significance of stratification systems in multiple myeloma. I. Risk categories (good and poor risk)

A group of 193 patients with multiple myeloma (MM) consisting of cases treated only symptomatically or by nonsystematic therapy with Cyclophosphamide or Melphalan (1959-76), and of patients given systematic polychemotherapy with intensive supportive treatment (1976-84) were subjected to prognostic analysis of the importance of MM stratification into two categories, poor and good risk. The evaluation included only hitherto untreated patients with MM of the IgG, IgA, and Bence-Jones' types. All the three evaluated stratification systems (ALGB, NCI-SECSG and CALGB) were found in the present study to show a good, equally significant relation to the prognosis of the disease. The survival median of poor risk patients in terms of the used classification system for the 1959-76 subgroup was 5-6 months, for the 1976-84 subgroup 20-22 months. In the good risk category it was 24-27 months in the 1959-76 subgroup and 47-50 months in the 1976-84 subgroup. Permanent validity of the initial prognosis and in the poor risk category safe coverage of patients with a high risk of early death was proved. Good agreement of the studied stratification systems with the clinical staging system of Durie and Salmon [14] (most of the Stage III patients consisted of poor risk patients) was recorded, which, however, was not the case with the staging system of Merlini, Waldenstr?m and Jayakar [27]. The CALGB system is considered the most suited to the needs of clinical practice.     

Prognostic factors in idiopathic myelofibrosis: a simple scoring system with prognostic significance

In a prognostic univariate analysis of a series of 80 patients with idiopathic myelofibrosis the Hb-concentration, the platelet count and osteomyelosclerosis emerged as factors with prognostic significance. A Hb-concentration less than 10 g/dl was associated with a significantly shorter survival than a Hb-concentration greater than or equal to 10 g/dl. A platelet count less than 100 x 10(9)/l also implied a significantly shorter survival. Patients with osteomyelosclerosis on X-ray of the skeleton had a significantly better prognosis as compared to those without osteomyelosclerosis. In a multivariate regression analysis the Hb-concentration consistently emerged as an important prognostic parameter, whereas the platelet count was only of prognostic significance within the first 6 months from diagnosis and the presence of osteomyelosclerosis emerged as a favourable parameter at 3 and 5 years. Based upon the above parameters and spleen size, a prognostic scoring system was designed which categorized the patients into three prognostic groups with highly different survival times (low risk group = 69 months; intermediate risk group = 33 months; high risk group = 4 months).     

A new staging system to predict prognosis of patients with multiple myeloma in an era of novel therapeutic agents

Various prognostic markers for multiple myeloma (MM) have been identified, and stratification using these markers is considered important to optimize treatment strategies. The international staging system (ISS) is now a widely accepted prognostic staging system for MM patients; however, its validity is controversial in the era of new therapeutic regimens, since ISS had been established before introduction of new agents. We retrospectively reviewed prognostic factors in order to seek out an alternative staging system more suitably applied to MM patients treated with novel agents. We analyzed 178 newly diagnosed MM patients who received either conventional chemotherapy without novel agents (CT; n = 79) or chemotherapy with novel agents (NT; n = 99). Although median overall survival (OS) of patients treated with CT is significantly different depending on stages of ISS, ISS had no effect on OS among patients treated with NT. Meanwhile, we identified hemoglobin (Hb) and plasmacytoma as independent risk factors for OS in patients who received NT. Using these two parameters, we stratified NT patients into three stages; stage 1 (Hb≥10 g/dL and absence of plasmacytoma), stage 2 (not stage 1 or 3), and stage 3 (Hb <10 g/dL and presence of plasmacytoma). We found that there were significant differences in median OS among the three stages (8.13, 5.95, and 2.45 yr for stages 1, 2, and 3, respectively). This preliminary study suggests that this alternative staging system based on Hb and plasmacytoma is a simple and useful way to predict prognosis of MM patients in the novel agent era.     

Significance of the preoperative neutrophil-to-lymphocyte ratio in the prognosis of patients with gastric cancer

AIM: To investigate the significance of the preoperative neutrophil-to-lymphocyte ratio(NLR) in the prognosis of patients with gastric cancer(GC). METHODS: The clinical data of 291 GC patients were analysed retrospectively; these patients were divided into two groups according to their preoperative NLR: a high-NLR group(NLR ≥ 3.5, 131 cases) and a low-NLR group(NLR 3.5, 160 cases). The clinicopathological characteristics and five-year survival rates of the two groups were compared. The NLR and other clinicopathological factors were subjected to univariate and multivariate survival analysis to evaluate the effects of the NLR on the prognosis of GC patients. RESULTS: The lowest preoperative NLR among the 291 patients was 0.56, whereas the highest preoperative NLR was 74.5. The mean preoperative NLR was 5.99 ± 8.98. Age, tumour size, T staging, tumour-node-metastasis(TNM) staging and platelet count were significantly different between the highand low-NLR groups(P 0.05). The five-year survival rate of the high-NLR group was 17.0%, which was significantly lower than that of the low-NLR group(43.6%; 17.0% vs 43.6%, P 0.05). The univariate analysis results showed that the five-year survival rate was related to age, tumour size, T staging, N staging, TNM staging, carcinoembryonic antigen value and NLR(P 0.05). Multivariate analysis results showed that the NLR was an independent risk factor that likely affected the five-year survival rate of GC patients(P = 0.003, HR = 0.626, 95%CI: 0.460-0.852). CONCLUSION: The preoperative NLR could be used as a prognostic factor for GC patients; in particular, a high NLR corresponded to poor prognosis of GC patients.     

Tumor necrosis correlates with angiogenesis and is a predictor of poor prognosis in malignant mesothelioma

OBJECTIVES: Malignant mesothelioma (MM) is a fatal tumor of increasing incidence related to asbestos exposure. Microscopic tumor necrosis (TN) is a poor prognostic factor in solid tumors, but it has not been characterized in MM. We wished to evaluate the incidence of TN in MM and its correlations with clinicopathologic factors, angiogenesis, and survival. METHODS: TN was graded in 171 routine formalin-fixed, paraffin-embedded hematoxylin-eosin-stained tumor sections by two independent observers. Angiogenesis was assessed by the microvessel count (MVC) of CD34 immunostained sections. TN was correlated with survival by Kaplan-Meier and log-rank analysis, and stepwise, multivariate Cox models were used to compare TN with angiogenesis and established prognostic factors and prognostic scoring systems. RESULTS: TN was identified in 39 cases (22.8%) and correlated with low hemoglobin (p = 0.01), thrombocytosis (p = 0.04), and high MVC (p = 0.02). TN was a poor prognostic factor in univariate analysis (p = 0.008). Patients with TN had a median survival of 5.3 months vs 8.3 months in negative cases. Independent indicators of poor prognosis in multivariate analysis were nonepithelioid cell type (p = 0.0001), performance status > 0 (p = 0.007), and increasing MVC (p = 0.004) but not TN. TN contributed independently to the European Organisation for Research and Treatment of Cancer (EORTC) [p = 0.03] and to the Cancer and Leukemia Group B (CALGB) [p = 0.03] prognostic groups in respective multivariate Cox analyses. CONCLUSIONS: TN correlates with angiogenesis and is a poor prognostic factor in MM. TN contributes to the EORTC and CALGB prognostic scoring systems.     

Platelet mass has prognostic value in patients with myelodysplastic syndromes

Platelet mass (mean platelet volume x platelet count) can be derived from data obtained from the routine full blood count and separates patients with myelodysplastic syndromes (MDS) at diagnosis into three distinct prognostic groups: low platelet mass group - median survival 5 months and 5-year survival 0%; intermediate platelet mass group - median survival 30 months and 5-year survival 34%; high platelet mass group median survival - not reached at 82 months follow-up with a 5-year survival of 82%. These data provide a simple rapid prognostic index at the time of diagnosis in MDS.     

A new prognostic system for multiple myeloma based on easily available parameters

The prognostic significance of different presenting features in 180 patients with multiple myeloma (MM) from a single institution was analysed. Out of eight variables isolated from the univariate analysis only two (blood urea and serum albumin), were significant in the multivariate model. Derived from these two simple variables, the relative risk of each patient was calculated, and subsequently two subpopulations of patients could be recognized. The first group included patients with a very active myeloma and a high risk of death soon after diagnosis, their median survival being of only 11.6 months, and the second one comprised patients with low risk of death during the first year and a median survival of 28 months. A hazard function derived from two-thirds of the patient population (training group) was successfully validated in the remaining subset of patients (test group). Finally, the three major available myeloma staging systems (Durie & Salmon's, Merlini et al's, and the one proposed by the British Medical Research Council) were tested in the present series, and only the latter one showed prognostic validity.     

Chronic idiopathic myelofibrosis: prognostic impact of myelofibrosis and clinical parameters on event-free survival in 122 patients who presented in prefibrotic and fibrotic stages

In chronic idiopathic myelofibrosis (CIMF) the factors predicting survival in patients who were already in the fibrotic stage have been well documented by numerous studies. Prefibrotic stages were only rarely evaluated so that the prognostic impact of myelofibrosis is currently not well known. Also predictive factors for disease-related events were not included in those studies. Thus, we evaluated the prognostic impact of myelofibrosis and other histopathological (megakaryocytes, blasts) and clinical [age, gender, splenomegaly, chemotherapy, hemoglobin (Hb), leukocyte, and platelet count] parameters in 122 patients in fibrotic and prefibrotic stages of CIMF on event-free survival. The statistical analysis was performed using the univariate log-rank test and the multivariate recursive partition and amalgamation (RECPAM) approach. In 62 patients disease-related events occurred during a mean observation period of 58 months. In univariate analysis they were associated with blast increase in the bone marrow. In RECPAM analysis a shorter event-free survival was found in anemic patients (mean: 9.3 months). In nonanemic patients older than 60 years, advanced myelofibrosis was associated with a shorter event-free mean survival of 23.2 months versus 69.3 months in less advanced cases. A slight or moderate myelofibrosis was not found to have a prognostic impact on event-free survival. The longest event-free survival was found in nonanemic patients who were younger than 60 years (mean: 185 months), regardless of the grade of myelofibrosis. Thus, we found that the most relevant prognostic parameter for event-free survival in CIMF were the Hb value, age, and grade of myelofibrosis.     

Staging multiple myeloma patients with active disease using serum levels of beta2m-free HLA class I heavy chain together with IgM or platelet count

Purpose: In multiple myeloma (MM), serum beta-2-microglobulin (β2m)-free heavy chains (FHC) of HLA class I has been shown to reflect disease activity. We investigated the possibility of stratifying patients with active disease according to FHC and other clinical parameters.Experimental design: We studied 146 patients with MM, including 100 at diagnosis, 31 in relapse and 15 unresponsive to therapy. Univariate and multivariate analyses were used to assess the prognostic significance of FHC together with continuous variables (age, albumin, creatinine, hemoglobin, erythrocyte sedimentation rate, β2m, calcium, IgM, platelet count) and categorical variables (Durie–Salmon disease stage, gender, bone lesion burden, heavy and light chain isotypes of M-component, clinical status). Survival tree analysis on significant variables was used to develop an MM staging system.Results: FHC, IgM, platelet count and hemoglobin were independent predictors of prognosis. Survival tree analysis of these variables defined 2 three-risk-group staging systems involving FHC and either IgM or platelet count. Median survival for FHC/IgM stages II and III was 41.5 and 27.8 months, whereas it was not reached for stage I patients (p < 0.0001). In the FHC/platelets system, median survival was 93.2 (stage I), 44.1 (stage II) or 27.8 (stage III) months (p < 0.0001). Similar results were obtained for the 117 MM patients without renal insufficiency (FHC/IgM p < 0.0001; FHC/platelets p = 0.001). For the 100 patients at diagnosis, FHC/IgM (p = 0.001) was more effective than FHC/platelets (p = 0.04).Conclusions: The independent prognostic markers FHC, IgM and platelets provide two staging systems unaffected by renal insufficiency. Both are effective in evaluating MM patients with active disease.     

IgD型多发性骨髓瘤18例临床特征和生存分析

目的探讨IgD型多发性骨髓瘤(MM)的临床表现与实验室检查特点、对治疗的反应、生存及预后情况。方法回顾性分析中山大学附属第一医院1996-01-01—2010-05-30收治的18例IgD型MM患者的临床资料。结果 IgD型MM占所有MM的4.4%,起病中位年龄54岁(36～70岁)。18例IgD型MM中,IgD-λ型16例(88.9%),IgD-κ型2例(11.1%);ISS分期中,Ⅲ期10例(占55.6%);DS分期中,18例患者均为Ⅲ期;2例(11.1%)合并淀粉样变,4例(22.2%)伴有髓外浸润;2例(11.1%)起病时有高钙血症;10例(55.6%)有肾功能损害。10例接受≥4个疗程方案化疗,总有效率60.0%;达平台中位时间为5.2个月,中位疾病进展时间为7.8个月,中位生存时间为13.0个月。结论 IgD型MM是一种侵袭性疾病,诊断时多处于疾病晚期,临床表现和实验室检查与其他亚型MM有较大差异,对治疗反应差,生存期短,预后差。     

Prognostic factors in elderly multiple myeloma patients aged 65 years or older: Comparison with nonelderly patients with multiple myeloma

Background:   Although age is a prognostic factor in multiple myeloma (MM), the prognostic factors in elderly MM patients may be different to those in nonelderly MM patients due to the patient's age. The difference in the significance of prognostic factors between elderly MM patients and the nonelderly MM patients was studied.
Methods:   Forty-two elderly MM patients aged 65 years or older were compared with 68 nonelderly MM patients, who were less than 65 years of age. The characteristics of the elderly patients included: aged 65–81 years (median, 72 years); female/male ratio of 22 : 20; 24 IgG type cases, 13 IgA type cases, one non-secretory case and four cases of Bence-Jones type; one case of stage I, 12 cases at stage II and 29 cases at stage III. The prognostic factors were evaluated by means of univariate analysis and Cox's multivariate analysis.
Results:   The median survival time was significantly shorter in the elderly MM patients (24 months) than in the nonelderly patients (50 months) ( P  < 0.01). Of the univariate prognostic factors, corrected serum Ca (cCa), hemoglobin, serum P, bone marrow plasma cell and uric acid were significant prognostic factors in the elderly MM patients, while nine factors including those listed here, were significant in nonelderly controls. Multivariate analysis showed that serum cCa was the only independent prognostic factor ( P  = 0.019) in elderly MM patients, while serum P and bone lesions were significant prognostic factors in nonelderly MM patients.
Conclusion:   Corrected serum c. (cCa) was an independent prognostic factor in elderly MM patients.     

Prognostic value of trisomy 8 in primary myelodysplastic syndrome

Background: According to the International Prognostic Scoring System (IPSS), sole +8 is categorized as intermediate cytogenetic subgroup. But as some myelodysplastic syndrome (MDS) patients with +8 perhaps progress quickly to acute leukaemia and have shorter survival, some reports have suggested that +8 should be categorized into poor risk cytogenetic group. The aim of this study was to clarify the prognostic role of +8 in MDS patients by comparing patients with normal karyotype, 20q‐ and ‐7/7q‐. Methods: The consecutive samples of 435 MDS patients in Shanghai were collected by prospective methods and diagnosed according to World Health Organization classification. Cytogenetic analysis was performed using conventional G‐banding karyotyping and fluorescence in situ hybridization techniques. Prognosis was estimated by univariate Log‐rank method and multivariate Cox proportional hazard models. Results: Of 424 cases completing the cytogenetic analysis, 71 (16.7%) had +8, including 38 patients with sole +8 (9.0%). No significant difference in median survival was observed between patients with sole +8 and that with +8 and one of other abnormalities. The +8 clone size was not linked to survival. The median survival of patients with +8, normal karyotype and complex karyotype was 25 months, 38.1 months and 5.9 months respectively. However, no significant difference was observed between patients with 20q‐ (21.4 months) and ‐7/7q‐ (25.8 months). Trisomy 8 was an independent prognostic factor by Cox regression model. Conclusion: There is no significant difference in prognosis between patients with +8 and patients with 20q‐ or ‐7/7q‐. Trisomy 8, 20q‐ and ‐7/7q‐ are categorized as intermediate cytogenetic risk according to our primary study.     

Lenalidomide with dexamethasone treatment for relapsed/refractory myeloma patients in Korea—experience from 110 patients

We conducted a retrospective analysis of lenalidomide with dexamethasone for patients with relapsed/refractory multiple myeloma (RRMM) who were treated within the Korean patient access program. Lenalidomide has been approved for RRMM for several years in Europe and North America, but has not been accessible to Asian patients in the past. Between 2008 and 2012, 110 patients from 20 hospitals were enrolled. The overall response rate (ORR) was 43.6 % with 15.4 % of very good partial response (VGPR) or better. The median time to progression (TTP) in this heavily pretreated patient population was 8.0 months, and median overall survival (OS) was 23 months. Hematologic toxicities, fatigue, anorexia, and constipation were the most common adverse events. The number of previous treatment lines, previous exposure to thalidomide, refractoriness to thalidomide and bortezomib, pretreatment white blood cell count (WBC), platelet count, t(14;16), and 17p deletion were significant prognostic factors for TTP, and creatinine clearance, refractoriness to thalidomide and bortezomib, performance status, platelet count, and 17p deletion were significant for OS in univariate analysis. In multivariate analysis, WBC and platelet count were significant prognostic factors for TTP and performance status for OS. For Korean myeloma patients, lenalidomide showed considerable efficacy, and toxicities were comparable to the data published in Europe and North America.     

Pretreatment thrombocytosis as a significant prognostic factor in malignant mesothelioma: a meta-analysis

The current meta-analysis analyzed the prognostic impact of elevated platelet count before the treatment of malignant mesothelioma (MM). We performed a search for articles published up to April 15, 2016 in PubMed, MEDLINE, EMBASE, and Web of Science, which evaluated elevated platelet count and survival outcome of MM. STATA version 12 was used for statistical analysis. The pooled hazard ratios (HRs) and their 95% confidence intervals (CIs) were combined to calculate overall effects. The assessment of heterogeneity was tested by the Cochran Q and I² statistics. The sensitivity and meta-regression analyses were performed to explore the origin of heterogeneity. We analyzed 18 eligible studies (3602 patients) that evaluated the correlation between pretreatment platelet count and overall survival (OS). Elevated platelet count was a prognostic factor of poor OS, with a pooled HR of 1.56 (95% CI = 1.36–1.77). However, significant heterogeneity was observed in the included studies (I² = 86.0%, p < 0.001). Sensitivity and meta-regression analyses were performed to trace the origin of heterogeneity. Only the variable type (multivariable or univariate model) was traced as the origin of heterogeneity. Hence, we conducted a subgroup analysis of variable type. The HR was 1.66 (95% CI = 1.41–1.91) in the multivariable group and no significant heterogeneity was observed (I² = 0.0%, p = 0.476). In conclusion, high pretreatment platelet count resulted in poor OS in MM. Therefore, platelet count could be an adequate and useful factor of prognosis for MM.     

A multivariate analysis of prognostic factors in chronic myeloid leukemia

The prognostic value of different clinical and laboratory findings at diagnosis of chronic myeloid leukemia (CML) was analyzed in a series of 121 cytogenetically studied patients. From the univariate and multivariate analysis of the whole series it was apparent that the minority of Ph1-negative patients (11.5%) could be considered as a poor prognosis group. The analysis was then restricted to the Ph1-positive patients. From a multivariate survival analysis (Cox's regression model) of the latter group the following poor prognosis factors emerged: splenomegaly, hepatomegaly, presence of erythroid precursors in peripheral blood, and bone marrow myeloblasts over 5%. From the contribution of each one of these factors to the regression model, a clinical staging of Ph1-positive CML was derived: stage I (low risk, 32% of patients), including patients with one or no factors; stage II (intermediate risk, 38%), including cases with two factors; and stage III (high risk, 30%), including patients with three or four factors. The difference in survival of the patients at different stages was highly significant (p less than 0.001).     

Significantly better prognosis for patients with primary plasma cell leukemia than for patients with secondary plasma cell leukemia

Plasma cell leukemia (PCL) is a rare variant of multiple myeloma (MM). Patients may either present de novo (primary PCL), or PCL may occur during the course of MM (secondary PCL). We compared the laboratory and clinical findings of both primary and secondary PCL and MM to elucidate their natural history and the relationship among these entities. Ten cases of PCL (7 cases of primary PCL and 3 cases of secondary PCL) and 20 sex- and age-matched cases of MM were compared. The patients with primary PCL showed significantly lower platelet and neutrophil counts in peripheral blood and higher cellularity in bone marrow than patients with MM (p = 0.002, < 0.001 and 0.027, respectively). Immunophenotypic studies showed a different expression of HLA-DR and CD117 antigens among the 3 groups. There was a significant difference in survival between the 3 groups (median survival of primary PCL, secondary PCL and MM = 22.2, 1.3 and 36.4 months, respectively; p = 0.048). The patients with primary PCL showed better prognosis than those with secondary PCL. Primary PCL might be a differently developed disease from MM. In diagnosing PCL, it is important to differentiate primary PCL from secondary PCL for the prediction of prognosis.