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  • 1
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    Evaluation of Current Clinical Models for Risk of Progression from Monoclonal Gammopathy of Undetermined Significance to Multiple Myeloma or Related Malignancies in 2028 Persons Followed in the Czech Republic
    American Society of Hematology ; 2014
    In:  Blood Vol. 124, No. 21 ( 2014-12-06), p. 3376-3376
    Details
    In: Blood, American Society of Hematology, Vol. 124, No. 21 ( 2014-12-06), p. 3376-3376
    Abstract: Introduction: Monoclonal gammopathy of undetermined significance (MGUS) is a non-malignant condition associated with a risk of progression to multiple myeloma (MM) or related disorders. There are currently 2 clinical models predicting progression from MGUS to MM. The Mayo Clinic model uses levels and type of serum monoclonal protein (M-protein) and serum free light chain ratio (sFLC). The Spanish PETHEMA model uses flow cytometry of bone marrow plasmocytes (BMPC) and the presence of DNA aneuploidy. Purpose: The primary end point was to estimate the cumulative risk of hematologic disorders occurring during the follow-up of our cohort. The secondary end points were: to validate known clinical models suggested by the Mayo Clinic group and the Spanish PETHEMA group for the risk of progression from MGUS to MM or related malignancies and to establish a new risk model by the Czech Myeloma Group (CMG model) with better prediction of low-risk MGUS group. Group: Data for this study were obtained from the Registry of Monoclonal Gammopathies (RMG) acquired from hematologic centers of the Czech Republic. MGUS diagnosis was made according to IMWG criteria. In total, 2028 persons with MGUS were enrolled in the RMG study from May 2007 to June 2013. A total of 93% (1887/2028) of persons were evaluated. Results: 1887 MGUS persons were followed with median 4 years. Malignancies developed in 8.6% (162/1887) cases; MM occurred in 77.2% (125/162) of persons. The risk of progression was 1.5% at 1 year, 7.6% at 5 years and 16.5% at 10 years after diagnosis. The key predictors factors of progression were as follows: age ≥ 69 years, serum M- protein concentration ≥ 1.5 g/dL, BMPC 〉 5%, pathological sFLC ratio ( 〈 0.26 or 〉 1.65), immunoparesis of polyclonal immunoglobulins, levels of serum hemoglobin at baseline 〈 12.0 g/dL and the presence of normal plasma cells (nPC) in bone marrow ≤ 5 % identified by multiparametric flow cytometry techniques. Distribution of MGUS persons according to risk groups based on the Mayo Clinic model confirmed predictive power of Mayo Clinic model based on our data although isotype of M- protein was not found as independent predictor. At 10 years, no-risk group had 4.9% risk of progression compared to 16.3%, 24.6%, and 54.9% in groups with 1, 2 or 3 risk factors, respectively (p 〈 0.001). MGUS group with 1, 2 and 3 risk factors in comparison to the reference group without any risk factor had HR( 2.59 [95% CI: 1.39- 4.84]; p= 0.003, HR 4.79 [95% CI: 2.56-8.93] ; p 〈 0.001, HR 12.97 [95% CI: 5.52-30.48];p 〈 0.001), retrospectively. Immunoparesis instead of DNA aneuploidy was used together with the presence of abnormal plasma cells (aPCs) to validate the modified PETHEMA model. The rates of progression at 3 years were 2.5%, 8.1% and 28.0% for groups with neither, one or both risk factors, respectively (p 〈 0.001). MGUS group with 1 and 2 risk factors in comparison to the reference group without any risk factor had HR (3.98 [95% CI: 1.60-9.91]; p= 0.003, HR 14.23 [95% CI: 2.86-70.76] ; p 〈 0.001), retrospectively. Based on the 5 parameters with independent predictive value in the univariate analysis (immunoparesis, serum M-protein quantity ≥ 1.5 g/dL, BMPC 〉 5%, abnormal sFLC ratio and serum level of hemoglobin 〈 12.0 g/dL) we proposed a new CMG model. The created CMG model clearly detected MGUS persons at low risk 86.6% (828/956) with the risk of progression 5.6% at 5 years better than previously described models. As expected, the number of MGUS persons with the highest risk of progression was limited to 3.7% only (35/956), with the risk of progression 31.9% at 5 years. The MGUS group with 5 risk factors had 63 times higher hazard of progression compared to reference MGUS group (HR 63.17 [95% CI: 13.99-285.36]; p 〈 0.001). Conclusion: In the large cohort of MGUS persons, we confirmed validity of previously considered clinical models for the risk of progression from MGUS to MM by the Mayo Clinic group and the Spanish PETHEMA group (model used for SMM). New CMG model for the risk of progression from MGUS to MM or related malignancies was established with an advantage for better identification of MGUS persons at low risk (87% of persons with risk of progression below 10% in 5 years) as well as few persons at the highest risk of progression. As a consequence, limited evaluation and visits can be planned in majority of MGUS persons in follow-up. Acknowledgments: This work was supported by grants NT13492-4, NT14575-3 and by EU FP7/2007-2013; grant n° 278570. Disclosures No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V124.21.3376.3376
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2014
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  • 2
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    A first Czech analysis of 1887 cases with monoclonal gammopathy of undetermined significance
    Wiley ; 2017
    In:  European Journal of Haematology Vol. 99, No. 1 ( 2017-07), p. 80-90
    Details
    In: European Journal of Haematology, Wiley, Vol. 99, No. 1 ( 2017-07), p. 80-90
    Abstract: Monoclonal gammopathy of undetermined significance ( MGUS ) is a premalignant condition with a risk of malignant conversion. Patients and methods With the aim to estimate the cumulative risk MGUS progression to hematologic malignancies, we analyzed a nationwide population‐based cohort of 1887 MGUS patients from the Czech Registry of Monoclonal Gammopathies ( RMG ) between 2007 and 2013. Results During the follow‐up period (median 4 years; range 0.6‐34.8), progression to hematologic malignancies was observed in 8.6% (162 of 1887) of patients. Factors associated with progression were as follows: M‐protein concentration ≥1.5 g/ dL , pathological sFLC ( 〈 0.26 or 〉 1.65) ratio, bone marrow plasma cells ( BMPC s) in cytology 〉 5%, immunoparesis, age ≥69 years, and the level of serum hemoglobin at baseline 〈 12.0 g/ dL . Combining these factors, we propose a new risk model ( CMG model). The risk of progression at 10 years was 1.6%, 16.9%, 22.9%, 39.4%, and 52.3%, respectively, if 0 (reference group), one, two, three, or four to five risk factors are present ( P 〈 .001) with HR 63 times higher compared to the reference MGUS group. Conclusion The new CMG model was established with an advantage for better identification of MGUS patients at low risk.
    Type of Medium: Online Resource
    ISSN: 0902-4441 , 1600-0609
    URL: Issue
    URL: Article
    DOI: 10.1111/ejh.2017.99.issue-1
    DOI: 10.1111/ejh.12894
    Language: English
    Publisher: Wiley
    Publication Date: 2017
    detail.hit.zdb_id: 2027114-1
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  • 3
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    Bortezomib (Velcade) in the Treatment of Relapsed or Refractory Multiple Myeloma: Analysis of Safety and Efficacy of Bortezomib From Registry of Monoclonal Gammopathy of Czech Myeloma Group
    American Society of Hematology ; 2012
    In:  Blood Vol. 120, No. 21 ( 2012-11-16), p. 1872-1872
    Details
    In: Blood, American Society of Hematology, Vol. 120, No. 21 ( 2012-11-16), p. 1872-1872
    Abstract: Abstract 1872 Background: Bortezomib (Velcade) is one of the most effective treatment options in the treatment of relapsed or refractory multiple myeloma (RRMM). In the Czech Republic, it has been available since 2004. Registry of monoclonal gammopathy (RMG) of the Czech Myeloma Group contains information of more than 90% of patients in the Czech Republic treated with novel drugs.Aims: The aim of this retrospective analysis was to verify the therapeutic efficacy and safety of bortezomib in the treatment of RRMM in the Czech Republic. Methods: Before inclusion to RMG, all persons signed the informed consent form. In total, 1469 MM patients treated with bortezomib were evaluated from the RMG between June 2004 and December 2011.A total of 51.5% (750/1469) RRMM patients were analyzed with follow-up ≥6 months from the start of first administration of bortezomib. 30.6% (450/1469) patients with newly diagnosed MM were excluded from the analysis as well as 18.2% (267/1469) with a short follow-up. Evaluation of treatment response was performed according to the IMWG criteria. Median patient age was 65 years (range 33.9–88.1), median time since starting therapy was 21.5 months (range 6.1– 86.2), median number of previous treatments was 3.0 (range 1.0–8.0). In total, 92% (690/750) patients finished treatment of bortezomib (cycles length 21–28 days with application on days 1, 4, 8,11 or 1, (4), 8, 15 for frail patients). Median number of bortezomib cycles delivered was 6 (0.5–15.5). Results: Assessment of therapeutic response was possible in 92% (690/750) of treated patients. Overall response (ORR) in 57.5% (397/690) patients including 3% sCR, 8% CR, 20.3% VGPR, 26.2% PR. Stable disease was confirmed in 11.4% (79/690) patients and 22.5% (155/690) patients had progressive disease. In 50.1% of responders, first response (≥MR; defined as a ≥25% decrease in the serum MIg) occurred within the first cycle. At the second cycle, 24.2% of responders started to respond. Median time to progression (TTP) for all responders was 12.4 months. Median overall survival after starting bortezomib therapy (OS) was 32.3 months for all responders. Altogether, 692 adverse events (AEs) were documented. The most frequent AEs were: anemia in 62% of patients (462/750); severity of anemia was distributed as follows: 33.3% (250/750) cases of grade 1, 28.3% (212/750) cases of grade 2. Thrombocytopenia grade 3 and 4 was seen in 21.5% (161/750) of patients. Pre-existing peripheral neuropathy (PNP) grade 1–2 was presented in 25.1% (191/750) of patients at the start of bortezomib treatment. After treatment of bortezomib, PNP could be documented in 59.3% (445/750) cases with 16% (71/445) cases of grade 3 and 0.7% (3/445) cases of grade 4 PNP. In subanalysis, groups of patients were compared with relapsed patients who were treated with bortezomib in the second, third or greater-line of therapy. Among these three groups, there were significant differences in the evaluation of ORR (59.8% vs. 53.4% vs. 46.8%, p=0.022), as well as the sCR+CR was dependent on the number of previous treatment lines (15.6% vs 7.3% vs. 1.4%; p 〈 0.001). Median TTP had similar dynamics (14.5 vs. 11.0 vs. 10.0 months; p= 〈 0.001). Altogether, 55.5% (416/750) of patients were pre-treated with thalidomide, while other patients were pre-treated with only conventional cytotoxic agens. Between these two groups we found differences in the achievement of treatment response: sCR+CR (8% vs. 14.1%; p =0.007), median TTP (11.0 vs. 15.0 months; p = 0.001) and median OS from the start of treatment with bortezomib (28.2 vs. 36.5 months; p = 0.001). Autologous stem cell transplantation after treatment with bortezomib was performed in 13.9% (104/750) of patients with positive effect on survival compared to patients without autologous stem cell transplantation (median OS 42.4 vs. 31.2 months; p = 0.007, median TTP 19.4 vs. 11.4 months; p 〈 0.001). Conclusion: Our results show that bortezomib is one of the highly effective drugs for patients with RRMM. In current practice, the benefit (measured TTP) is 10–14 months, according to severity of the disease with a favorable impact on overall survival even in heavily pretreated patients. Implementation of autologous stem cell transplantation in relapsed disease following bortezomib treatment had a beneficial effect on overall survival. Acknowledgments: This work was supported by IGA NT 12130–4 and IGA NT 12215–4 grants. Disclosures: Maisnar: Janssen Cilag: Honoraria; Celgene: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; Bayer (Schering): Honoraria. Hajek:Merck: Honoraria, Membership on an entity's Board of Directors or advisory committees; Celgene: Honoraria, Membership on an entity's Board of Directors or advisory committees; Janssen Cilag: Honoraria.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V120.21.1872.1872
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2012
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  • 4
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    Prediction of Progression of Smouldering into Therapy Requiring Multiple Myeloma By Easily Accessible Clinical Factors [in 527 Patients]
    American Society of Hematology ; 2014
    In:  Blood Vol. 124, No. 21 ( 2014-12-06), p. 2071-2071
    Details
    In: Blood, American Society of Hematology, Vol. 124, No. 21 ( 2014-12-06), p. 2071-2071
    Abstract: Background Several models predict the progression from smouldering multiple myeloma (SMM) to therapy requiring multiple myeloma (MM). Three models comprise the assessment of tumour mass by different clinical parameters to stratify in risk groups: 1) the Mayo Clinic model uses bone marrow plasma cells percentage (BMPC) and serum monoclonal protein (M-protein), 2) the PETHEMA model uses immunoparesis and the percentage of abnormal plasma cells by flow cytometry, 3) the Heidelberg group assesses tumour mass by either the percentage of malignant plasma using iFISH or the Mayo assessment depicted above, and the presence of chromosomal aberrations associated with adverse prognosis. Besides tumor mass, they find the number of focal lesions in whole body MRI ( 〉 1) as strong prognostic factor. Aim To assess a combination of easily accessible clinical factors identifying patients at ≥ 80% risk of progression to MM requiring treatment within two years from the diagnosis of SMM. Methods Data for this study were obtained from the Registry of Monoclonal Gammopathies (RMG) acquired from hematologic centers of the Czech Republic for 287 SMM patients enrolled from May 2007 to June 2013. A cohort comprising 240 SMM patients from Heidelberg, Germany was used for validation (Neben et al. JCO 2013). Results During the follow up period (median 2.4 years; range 0.6 - 18.0) progression to MM was observed in 51.9% (149/287) patients in the study cohort, representing 16% risk of progression at 1 year, 31.2% at 2 years, 54.8% at 5 years and 73.4% at 10 years. In univariate analysis factors significantly associated with progression were as follows: serum free light chain (iFLC/uFLC) ratio 〉 30 (HR 2.4 [95% CI: 1.4 - 4.1]; p 〈 0.001) plasma cell infiltration in bone marrow cytology ≥ 15% (HR 2.1 [1.5-3.0]; p 〈 0.001), immunoparesis (HR 2.0 [1.3-2.9]; p 〈 0.001), M - protein concentration ≥ 2.3 g/dL (HR 2.00 [1.4-2.7]; p 〈 0.001), beta2 microglobulin ≥ 2.0 mg/l (HR 1.8 [1.2-2.7]; p= 0.001), and thrombocyte count ≤ 250 x 109/l (HR 1.7 [1.1-2.4] ; p= 0.005). In multivariate analysis, 3 parameters showed independent predictive value (immunoparesis, serum M-protein quantity ≥ 2.3 g/dL and iFLC/uFLC 〉 30). Combining these factors, we proposed a new risk model for SMM patients (CMG model). The risk of progression from SMM to MM at 2 years was 18.5%, 20.9%, 41.9% and 78.7% if 0 (reference group), 1, 2 or 3 risk factors are present (p 〈 0.001) (Figure 1) with HR of 1.5 [0.7-2.9]; p=0.283, 2.5 [1.3-5.0] ; p= 0.008, 6.8 [3.0-15.2]; p 〈 0.001, n=139), respectively. The CMG model was validated on 240 SMM patients from Heidelberg published in 2013. The risk of progression from SMM to MM at 2 years was 5.3%, 7.5%, 44.8% and 81.3% if 0, 1, 2 or 3 risk factors were present, respectively (p 〈 0.001) (Figure 1) with HR of 4.2 ([0.5-36.1]; p=0.189), 21.5 ([2.9-159.1] ; p= 0.003, HR 38.6 [4.7- 317.7]; p 〈 0.001, n=113). Conclusion We propose and validate a new risk model for SMM patients with prediction of 80% (78.7% on our CMG model; 81.3% on data from Heidelberg) risk of progression to therapy requiring myeloma within two years based on easily accessible clinical parameters (CMG model). The model could especially be used to identify high-risk patients to be included in early treatment clinical trials. Acknowledgments: This work was supported by grants NT13492-4, NT14575-3 and by EU FP7/2007-2013; grant n°278570 and “OverMyR”, as well as the Deutsche Forschungs-Gemeinschaft (DFG) SFB/TRR79. Figure 1: CMG risk model: CMG cohort of patients and validation cohort of Heidelberg patients Figure 1:. CMG risk model: CMG cohort of patients and validation cohort of Heidelberg patients Disclosures Seckinger: Novartis: Research Funding.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V124.21.2071.2071
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2014
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 5
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    Bleeding pattern and consumption of factor VIII concentrate in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 (Czech National Haemophilia Programme registry data)
    Springer Science and Business Media LLC ; 2023
    In:  Annals of Hematology
    Details
    In: Annals of Hematology, Springer Science and Business Media LLC
    Abstract: The manuscript provides an overview of treatment and its changes in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 using data from the registry of the Czech National Haemophilia Programme (CNHP). Over a 9-year period, we focused on the reduction in the annual bleeding rate (ABR), joint bleeding rate (AJBR) and factor VIII consumption when patients with severe haemophilia A switched from on-demand treatment to prophylaxis. The ABR and AJBR include both patient-reported home treatment and treated hospitalisation episodes. All adult patients with severe haemophilia A were categorised into three groups according to the therapeutic regimen. The first group was patients on prophylaxis during the follow-up period, the second group consisted of patients on on-demand treatment, and the third group was patients who received both treatment regimens during follow-up. With an increase in the proportion of patients with severe haemophilia A on prophylaxis from 37 to 74% between 2013 and 2021, the ABR for all patients with severe haemophilia A decreased approximately 6.9-fold, and the AJBR decreased 8.7-fold. Expectedly, the factor consumption increased by approximately 68.5%. In the group of patients with severe haemophilia A who had switched from an on-demand to a prophylactic regimen, the total number of bleeding events decreased 3.5-fold, and the number of joint bleeding episodes decreased 3.9-fold. Factor VIII consumption increased by 78.4%. Our study supports a previously reported positive effect of prophylaxis on bleeding control. We believe that the substantial improvement in ABR justifies the increased treatment costs.
    Type of Medium: Online Resource
    ISSN: 0939-5555 , 1432-0584
    URL: Article
    DOI: 10.1007/s00277-023-05453-6
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2023
    detail.hit.zdb_id: 1458429-3
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  • 6
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    Rapid Progression of COVID-19-Associated Fatal Capillary Leak Syndrome
    MDPI AG ; 2022
    In:  Infectious Disease Reports Vol. 14, No. 6 ( 2022-11-17), p. 884-888
    Details
    In: Infectious Disease Reports, MDPI AG, Vol. 14, No. 6 ( 2022-11-17), p. 884-888
    Abstract: Several cases of capillary leak syndrome (CLS) related to COVID-19 or vaccination against SARS-CoV-2 have been described in the literature. We present a case of a 42-year-old, previously healthy male, presenting with a mild form of COVID-19, who suddenly developed severe shock with hypotension and severe hemoconcentration within hours of admission to the hospital. Volume resuscitation was not effective, increasing hemoglobin (198 g/L on admission, 222 g/L 9 h later) suggested fluid leak into peripheral tissues. After cardiac arrest, the patient was resuscitated and connected to extracorporeal membrane oxygenation, but died shortly afterwards due to refractory heart failure. Retrospective investigation of blood samples confirmed diagnosis of CLS by progressive hypoalbuminemia (40 g/L on admission, 14 g/L 19 h later) and monoclonal gammopathy kappa (4.7 g/L). Patient’s CLS was triggered by COVID-19, either a first attack of idiopathic CLS called Clarkson’s disease or a COVID-19-induced secondary CLS.
    Type of Medium: Online Resource
    ISSN: 2036-7449
    URL: Article
    DOI: 10.3390/idr14060088
    Language: English
    Publisher: MDPI AG
    Publication Date: 2022
    detail.hit.zdb_id: 2569856-4
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