- MeSH
- lidé MeSH
- mnohočetný myelom * epidemiologie terapie MeSH
- rizikové faktory MeSH
- věkové faktory MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- dopisy MeSH
- Geografické názvy
- Skandinávie a severské státy MeSH
- MeSH
- lidé MeSH
- mnohočetný myelom * epidemiologie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- směrnice MeSH
Smoldering multiple myeloma (SMM) is an asymptomatic precursor to active multiple myeloma (MM). The aim of this study was to report clinical characteristics and outcomes of patients with SMM stratified based on their risk of progression to MM using the Mayo 20/2/20 criteria. Data were leveraged from the Czech Myeloma Group Registry of Monoclonal Gammopathies (RMG). Key outcomes included progression-free survival from SMM diagnosis to active MM diagnosis or death (PFS), progression-free survival from SMM diagnosis to progression on first line (1 L) MM treatment or death (PFS2), and overall survival (OS). Of 498 patients, 174 (34.9%) were classified as high risk and 324 (65.1%) as non-high risk. Median follow-up was approximately 65 months. During follow-up, more patients in the high-risk vs non-high-risk group received 1 L MM treatment (76.4% vs 46.6%, p < 0.001). PFS, PFS2, and OS were significantly shorter in high-risk vs non-high-risk patients (13.2 vs 56.6 months, p < 0.001; 49.9 vs 84.9 months, p < 0.001; 93.2 vs 131.1 months, p = 0.012, respectively). The results of this study add to the growing body of evidence that patients with high-risk vs non-high-risk SMM have significantly worse outcomes, including OS.
- MeSH
- doba přežití bez progrese choroby MeSH
- doutnající mnohočetný myelom * diagnóza epidemiologie terapie MeSH
- lidé MeSH
- mnohočetný myelom * diagnóza epidemiologie terapie MeSH
- registrace MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Česká republika MeSH
Studies of survival in hematological malignancies (HMs) have generally shown an improvement over time, although most of these studies are limited by a short follow-up period. Using the NORDCAN database with data from Denmark, Finland, Norway and Sweden, we follow periodic increases in relative survival in seven HMs through half a century up to 2015-2019. Five-year survival improved in all seven HMs, reaching 90% for Hodgkin lymphoma (HL), myeloproliferative neoplasias and chronic lymphocytic leukemia (CLL), 60% for multiple myeloma (MM) and chronic myeloid leukemias (CMLs), 50% for the myelodysplastic syndromes and 30% for acute myeloid leukemia (AML). Improvements in survival over 50 years ranged from 20% to more than 50% units across the different HMs. The likely reasons for such progress include earlier diagnoses, improved risk stratification and advances in treatment. We observed differing temporal trends in improvements in survival. The gradual increases observed in HL, CLL and AML highlight the impact of optimization of existing therapies and improvements in diagnostics and risk stratification, whereas the rapid increases observed in the CMLs and MM highlight the impact of novel therapies. Recent therapeutic advances may further improve survival in HMs where survival remains low such as in AML.
- MeSH
- akutní myeloidní leukemie * terapie MeSH
- chronická lymfatická leukemie * MeSH
- hematologické nádory * epidemiologie terapie MeSH
- Hodgkinova nemoc * MeSH
- lidé MeSH
- mnohočetný myelom * epidemiologie terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Geografické názvy
- Skandinávie a severské státy MeSH
- MeSH
- antitumorózní látky škodlivé účinky MeSH
- chronická myeloidní leukemie epidemiologie farmakoterapie MeSH
- hematologické nádory * epidemiologie farmakoterapie klasifikace MeSH
- inhibitory tyrosinkinasy škodlivé účinky terapeutické užití MeSH
- lidé MeSH
- mnohočetný myelom epidemiologie farmakoterapie prevence a kontrola MeSH
- nežádoucí účinky léčiv klasifikace MeSH
- terciární prevence MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
Multiple myeloma (MM) is a hematological malignancy caused by the clonal expansion of plasma cells. The incidence of MM worldwide is increasing with greater than 140 000 people being diagnosed with MM per year. Whereas 5-year survival after a diagnosis of MM has improved from 28% in 1975 to 56% in 2012, the disease remains essentially incurable. In this review, we summarize our current understanding of MM including its epidemiology, genetics and biology. We will also provide an overview of MM management that has led to improvements in survival, including recent changes to diagnosis and therapies. Areas of unmet need include the management of patients with high-risk MM, those with reduced performance status and those refractory to standard therapies. Ongoing research into the biology and early detection of MM as well as the development of novel therapies, such as immunotherapies, has the potential to influence MM practice in the future.
- MeSH
- cyklin D1 genetika MeSH
- exozom genetika MeSH
- genetická predispozice k nemoci MeSH
- histondemethylasy genetika MeSH
- imunoterapie metody MeSH
- lidé MeSH
- míra přežití MeSH
- mnohočetný myelom diagnóza epidemiologie genetika terapie MeSH
- mutace MeSH
- nádorové biomarkery genetika MeSH
- plazmatické buňky imunologie patologie MeSH
- represorové proteiny genetika MeSH
- rizikové faktory MeSH
- transkripční elongační faktory genetika MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- přehledy MeSH
- MeSH
- křehkost diagnóza epidemiologie MeSH
- lidé MeSH
- mnohočetný myelom diagnóza epidemiologie MeSH
- osmdesátníci MeSH
- proporcionální rizikové modely MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- dopisy MeSH
- MeSH
- lidé MeSH
- mnohočetný myelom * diagnóza epidemiologie terapie MeSH
- následné studie MeSH
- společnosti lékařské MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- směrnice pro lékařskou praxi MeSH
Monoklonální gamapatie jsou heterogenní skupinou onemocnění, pro kterou je typická proliferace klonu diferencovaných B-lymfocytů produkujících tzv. monoklonální imunoglobulin (MIG). Monoklonální imunoglobulin se může skládat jak z intaktní imunoglobulinové molekuly nebo také jen z jejích strukturálních komponent, tj. lehkých řetězců kappa či lambda, vzácněji z těžkých řetězců. Nejčastější monoklonální gamapatií je monoklonální gamapatie nejasného významu (MGUS). V posledních letech nabývá na významu včasná diagnostika MGUS vzhledem k stoupající četnosti monoklonálních gamapatií obecně, a zvláště té nejzávažnější – mnohočetného myelomu, která je patrná v České republice z dostupných dat Národního onkologického registru. Předmětem našeho sdělení je skutečnost, že právě ve východních Čechách (a to především na Trutnovsku a Jičínsku) je podle těchto dat zaznamenána nejvyšší frekvence výskytu maligních lymfoproliferací. Z tohoto důvodu jsme se zaměřili na diagnostiku a následné sledování pacientů s MGUS, kteří byli zachyceni laboratoří OKB Oblastní nemocnice v Trutnově v rámci rutinního vyšetření za období trvající 10 let. Naším cílem bylo vyhodnocení souboru pacientů s nově diagnostikovanými monoklonálními gamapatiemi nejasného významu z pohledu četnosti jejich transformace do obrazu maligní lymfoproliferace, se zaměřením zejména na výskyt mnohočetného myelomu v okrese Trutnov.
Monoclonal gammopathy are a heterogeneous group of disorders. They are characterized by proliferation of clonal differentiated B-cells producing the monoclonal immunoglobulin (MIG). Monoclonal immunoglobulin consists of both an intact immunoglobulin molecule or also only of its structural component, i.e. light chain kappa or lambda, rarely heavy chain. The most common monoclonal gammopathy is monoclonal gammopathy of unknown (undetermined) significance. Every patient with MGUS should be monitored regularly. In recent years, the early diagnosis of MGUS has become increasingly important due to the frequency of monoclonal gammopathy in general and especially the most serious of them - multiple myeloma. A higher incidence of monoclonal gammopathies and especially MM in Czech Republic is apparent from the available data of the National Cancer Registry. The subject of our communication is the fact, that in East Bohemia region (especially in the Trutnov and Nachod regions), according to these data, the highest frequency of malignant lymphoproliferative disorders is recorded. Therefore, we focused on diagnostics and monitoring over time in patients with MGUS, who were detected by the department of clinical biochemistry of the regional hospital in Trutnov as part of a routine examination. In a 10- year follow-up of patients with newly diagnosed monoclonal gammopathy of undetermined significance we monitored their further development over time and also on the frequency of transformation in malignant lymphoproliferative disorders, especially multiple myeloma in the district Trutnov.
The etiology of multiple myeloma (MM) is poorly understood. Summary data from genome-wide association studies (GWASs) of multiple phenotypes can be exploited in a Mendelian randomization (MR) phenome-wide association study (PheWAS) to search for factors influencing MM risk. We performed an MR-PheWAS analyzing 249 phenotypes, proxied by 10 225 genetic variants, and summary genetic data from a GWAS of 7717 MM cases and 29 304 controls. Odds ratios (ORs) per 1 standard deviation increase in each phenotype were estimated under an inverse variance weighted random effects model. A Bonferroni-corrected threshold of P = 2 × 10-4 was considered significant, whereas P < .05 was considered suggestive of an association. Although no significant associations with MM risk were observed among the 249 phenotypes, 28 phenotypes showed evidence suggestive of association, including increased levels of serum vitamin B6 and blood carnitine (P = 1.1 × 10-3) with greater MM risk and ω-3 fatty acids (P = 5.4 × 10-4) with reduced MM risk. A suggestive association between increased telomere length and reduced MM risk was also noted; however, this association was primarily driven by the previously identified risk variant rs10936599 at 3q26 (TERC). Although not statistically significant, increased body mass index was associated with increased risk (OR, 1.10; 95% confidence interval, 0.99-1.22), supporting findings from a previous meta-analysis of prospective observational studies. Our study did not provide evidence supporting any modifiable factors examined as having a major influence on MM risk; however, it provides insight into factors for which the evidence has previously been mixed.
