The myelodysplastic syndrome-comorbidity index provides additional prognostic information on patients stratified according to the revised international prognostic scoring system.

نویسندگان

  • Esther Zipperer
  • Nina Tanha
  • Corinna Strupp
  • Andrea Kündgen
  • Kathrin Nachtkamp
  • Judith Neukirchen
  • Barbara Hildebrandt
  • Rainer Haas
  • Norbert Gattermann
  • Ulrich Germing
چکیده

The myelodysplastic syndromes are a heterogeneous group of disorders of the hematopoietic stem cell and stem cell niche. The revised version of the International Prognostic Scoring System (IPSS-R), which is based on disease-related factors, was recently published. In 2010, the MDS-specific Comorbidity Index (MDS-CI) was developed by the Italian MDS study group and was validated using data of the Düsseldorf MDS-Registry. It uses patientrelated factors. We wondered if the MDS-CI adds any prognostic information to the IPSS-R. Our retrospective study included 1161 patients from the Düsseldorf MDSRegistry who received best supportive care or disease-specific therapy but not allogeneic transplantation. Diagnoses were made according to the WHO 2008 classification. In addition, patients with chronic myelomonocytic leukemia (CMML) and refractory anemia with excess blasts in transformation (RAEB-T) were included. For each patient to be included in the study, a complete set of comorbidity factors had to be evaluable. Data of 504 patients of our cohort had been used as the validation cohort for the development of the MDS-CI. The study received local ethics committee approval. Patients ́ data are summarized in Table 1. According to the MDS-CI, median survival times were 39, 24 and 15 months, for the low-risk, intermediate-risk and high-risk groups, respectively (P<0.001). The most frequent comorbidities were cardiac diseases (37%), followed by solid tumors (10%), and pulmonary (9%), renal (7%) and hepatic diseases (4%). Male patients had more comorbidities than female patients (P=0.001). Cardiac and pulmonary diseases were more frequent in males than in females: 42% vs. 30% (P<0.001) and 11% vs. 6% (P=0.002), respectively. Survival of male patients in the whole cohort (P=0.002) and in the MDS-CI low-risk group was worse than survival of female patients (P=0.02). The IPSS-R assessed at diagnosis was available for 506 patients. Median survival times were 105, 70, 36, 14 and 8 months for the very low-risk, low-risk, intermediate-risk, high-risk and very high-risk groups, respectively; overall survival time was 37 months (P<0.001). The IPSS-R low-risk group was divided by the MDS-CI into three different risk groups with survival times of 92, 63 and 36 months, respectively (P<0.0001). Combining IPSS-R very lowand low-risk patients together (n=221) produced significantly different median survival times; 98, 70 and 45 months, respectively (P=0.005) (Figure 1). Patients assigned to the intermediate, highand very high-risk groups were combined (n=285) and this produced significantly different median survival times; 22, 21 and 7 months, respectively (P=0.017) (Figure 1). In the Cox regression model in categorical analysis, the five risk groups of the IPSS-R are included into the model, as well as the MDS-CI lowand intermediate-risk groups. In the forward stepwise multivariate Cox regression analysis, IPSS-R and MDS-CI provided independent prognostic information. A total of 859 out of 1161 patients (74%) died during follow up. The exact cause of death could be ascertained in 516 patients. Of these, 402 deaths were disease-related (78%); in the other 114 patients, death was not diseaserelated (22%). Interestingly, in the MDS-CI low-risk group, the death of 84% of patients was disease-related: 75% of patients in the intermediate-risk group and 67% of those in the high-risk group (P=0.002). In a large cohort of 506 MDS patients, we were able to show that, in univariate analyses, the MDS-CI allows further stratification of the IPSS-R low-risk group and the combined group of the very lowplus low-risk groups and the intermediateplus high-risk groups. In multivariate analyses, the MDS-CI provides prognostic stratification independently of the IPSS-R. We also showed that in the MDS-CI high-risk group, the proportion of patients who died from disease-related causes is smaller than in the MDS-CI low-risk group, underlining the importance of assessing and potentially treating comorbidities. Our group was the first to systematically examine MDS patients according to comorbidities using CCI and HCTCI and evaluate the relationship to the IPSS. We reported that the HCTCI is superior to CCI in the multivariate analysis including the IPSS. Since both scores were not optimally suited for MDS patients, the Italian study group developed the MDS-CI, which was validated using data from our Registry.We can now confirm that the MDS-CI is suitable for all MDS patients. Breccia et al. compared the three mentioned comorbidity scores. Sperr et al. used CCI and HCTCI, and in their cohort, the HCTCI further stratified the IPSS lowand IPSS intermediate-risk groups. More recently, the Austrian MDS-Group published a score which is made up of both patient-related and disease-related factors with a comorbidity score according to HCTCI, ferritin, IPSS and age. The authors showed that this score provides four independent risk groups. In summary, a lot of work has been done to show that comorbidities are important for MDS-patient outcome. But do we really need another score? When you ask clinicians if they classify patients according to any comorbidity score they usually answer “No”, except in patients for whom allogeneic transplantation is being programmed. We propose to use the MDS-CI for assessment of comorbidities in the future since it has shown its high stability in

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

The hematopoietic stem cell transplantation comorbidity index is of prognostic relevance for patients with myelodysplastic syndrome.

We studied the impact of comorbidities on survival and evaluated the prognostic utility of comorbidity scores in MDS patients, who received best supportive care and were assessable according to the Charlson Comorbidity Index (CCI) and the Hematopoietic Stem Cell Transplantation Comorbidity Index (HCTCI): 171 patients were identified in the Duesseldorf MDS Registry. The HCTCI captured more comor...

متن کامل

Myeloid Cell Leukemia-1 Gene Expression and Clinicopathological Features in Myelodysplastic Syndrome

Background and Aims: Myeloid cell leukemia-1 (Mcl-1) plays a pivotal role in the survival of hematologic and solid tumors, and is known as a substantial oncogene. Studies have demonstrated the altered expression of Mcl-1 has been linked to malignancy development and poor prognosis. In this research, we have studied the expression of Mcl-1 mRNA in myelodysplastic syndrome (MDS) patients and det...

متن کامل

High flow cytometric scores identify adverse prognostic subgroups within the revised international prognostic scoring system for myelodysplastic syndromes.

The estimation of survival of myelodysplastic syndromes (MDS) and risk of progression into acute myeloid leukaemia is challenging due to the heterogeneous clinical course. The most widely used prognostic scoring system (International Prognostic Scoring System [IPSS]) was recently revised (IPSS-R). The aim of this study was to investigate the prognostic relevance of flow cytometry (FC) in the co...

متن کامل

Predictive factors for the outcome of allogeneic transplantation in patients with MDS stratified according to the revised IPSS-R.

Approximately one-third of patients with myelodysplastic syndrome (MDS) receiving allogeneic hematopoietic stem cell transplantation (HSCT) are cured by this treatment. Treatment failure may be due to transplant complications or relapse. To identify predictive factors for transplantation outcome, we studied 519 patients with MDS or oligoblastic acute myeloid leukemia (AML, <30% marrow blasts) w...

متن کامل

Impact of the revised International Prognostic Scoring System, cytogenetics and monosomal karyotype on outcome after allogeneic stem cell transplantation for myelodysplastic syndromes and secondary acute myeloid leukemia evolving from myelodysplastic syndromes: a retrospective multicenter study of the European Society of Blood and Marrow Transplantation.

The aim of this study was to determine the impact of the revised 5-group International Prognostic Scoring System cytogenetic classification on outcome after allogeneic stem cell transplantation in patients with myelodysplastic syndromes or secondary acute myeloid leukemia who were reported to the European Society for Blood and Marrow Transplantation database. A total of 903 patients had suffici...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • Haematologica

دوره 99 3  شماره 

صفحات  -

تاریخ انتشار 2014