The aim of this study was to determine the thrombogenicity of lupus anticoagulant (LA) antibodies using a modified thrombin generation assay (TGA) with the addition of activated protein C (APC) in a group of 85 patients with LA-positive samples. Of these, 58 patients had clinical manifestations of antiphospholipid syndrome (APS) according to the Sydney criteria classification, i.e., each patient had thrombosis or foetal loss, and 27 patients did not show any clinical manifestations of APS. A comparison of the two groups' TGA results revealed statistically significant differences (Fisher's test p = 0.0016). The group of patients exhibiting clinical manifestations of APS showed higher thrombogenicity in 56.9% of patients, while the group of patients not yet exhibiting clinical manifestations of APS showed higher thrombogenicity in 25.9% of patients. There were no significant differences in the specificity of the TGA test between the groups of patients exhibiting similar clinical manifestations. Receiver operating characteristic curve analysis showed a more significant relationship (p = 0.0060) for TGA than for LA titre (p = 0.3387). These data suggest that the determination of LA thrombogenicity with the TGA assay leads to an increased prediction of the manifestation of a thromboembolic event. Our findings appear to be particularly relevant for the prediction of thrombotic events in patients with laboratory-expressed APS and no clinical manifestations.

• Publikační typ
• časopisecké články 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

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.

• MeSH
• dospělí MeSH
• faktor VIII * terapeutické užití   MeSH
• hemofilie A * farmakoterapie   MeSH
• krvácení * chemicky indukované   epidemiologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• následné studie MeSH
• registrace * MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH

Mnohočetný myelom je krevní nádorové onemocnění charakterizované proliferací klonálních plazmatických buněk v kostní dřeni a přítomností paraproteinu v séru a/nebo moči. V posledních letech se pro diagnostiku i pro celý průběh léčby dostává do popředí celotělová zobrazovací metoda pozitronová emisní tomografie (PET) kombinovaná s výpočetní tomografií (PET/CT). Její výhodou je zachycení metabolicky aktivních charakteristických ložisek a dobrá detekce extramedulárních i paramedulárních lézí. Následující text shrnuje společné stanovisko odborníků České společnosti nukleární medicíny ČLS JEP (ČSNM ČLS JEP) a České myelomové skupiny (CMG) jak k provedení vyšetření PET/CT, tak k jeho hodnocení a reportování. Cílem je sjednotit metodiku vyšetření PET/CT napříč pracovišti v ČR, která bude srozumitelná a jednoznačná.

Multiple myeloma is a blood cancer characterized by proliferation of clonal plasma cells in the bone marrow and the presence of paraprotein in the serum and/or urine. In recent years, whole-body positron emission tomography (PET) imaging combined with computed tomography (PET/CT) has become a preferred imaging technique for diagnosis and treatment. It has the benefit of detecting metabolically active characteristic focuses and good detection of extramedullary and para-medullary lesions. The following text presents the joint standpoint of the representatives of the Czech Society of Nuclear Medicine ČLS JEP (CSNM ČLS JEP) and the Czech Myeloma Group (CMG) regarding PET/CT examination per se as well as its evaluation and reporting. The aim is to standardize the methodology of PET/CT examination across the Czech Republic, which will be clear and simple to understand.

• MeSH
• fluorodeoxyglukosa F18 MeSH
• lidé MeSH
• mnohočetný myelom * diagnostické zobrazování   patologie   MeSH
• PET/CT * metody   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• směrnice pro lékařskou praxi MeSH
• Geografické názvy
• Česká republika MeSH

Mnohočetný myelom je krevní nádorové onemocnění charakterizované proliferací klonálních plasmatických buněk v kostní dřeni a přítomností paraproteinu v séru a/nebo moči. V posledních letech se pro diagnostiku i pro celý průběh léčby dostává do popředí celotělová zobrazovací metoda pozitronová emisní tomografie (PET) kombinovaná s výpočetní tomografií (PET/CT). Její výhodou je zachycení metabolicky aktivních charakteristických ložisek a dobrá detekce extramedulárních i paramedulárních lézí. Následující text shrnuje společné stanovisko odborníků České společnosti nukleární medicíny ČLS JEP z. s. (ČSNM ČLS JEP) a České myelomové skupiny (CMG) jak k provedení vyšetření PET/CT, tak k jeho hodnocení a reportování. Cílem je sjednotit metodiku vyšetření PET/CT napříč pracovišti v ČR, která bude srozumitelná a jednoznačná.

Multiple myeloma is a blood cancer characterized by proliferation of clonal plasma cells in the bone marrow and the presence of paraprotein in the serum and/or urine. In recent years, whole-body positron emission tomography (PET) imaging combined with computed tomography (PET/CT) has become a preferred imaging technique for diagnosis and treatment. It has the benefit of detecting metabolically active characteristic focuses and good detection of extramedullary and paramedullary lesions. The following text presents the collective opinion of the representatives of the Czech Society of Nuclear Medicine ČLS JEP z.s. (CSNM ČLS JEP) and the Czech Myeloma Group (CMG) on PET/CT as well as on its evaluation and reporting. The aim is to standardize the methodology of PET/CT examination across the Czech Republic, which will be clear and simple to understand.

• Klíčová slova
• extramedulární ložiska, paramedulární ložiska,
• MeSH
• kritéria léčebné odpovědi MeSH
• lidé MeSH
• mnohočetný myelom * diagnostické zobrazování   MeSH
• PET/CT metody   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• směrnice pro lékařskou praxi MeSH

Antiphospholipid syndrome (APS) is a hypercoagulable state accompanied by the presence of heterogeneous antiphospholipid antibodies (aPL), which nonspecifically affect hemostasis by the presence of lupus anticoagulans (LA), anticardiolipin antibodies (aCL), antibodies against β2-glycoprotein-I (anti-β2GPI), but also non-criteria antibodies such as antibodies against β2-glycoprotein-I domain I (anti-DI), anti-phosphatidylserine/prothrombin (anti-PS/PT), anti-annexin V, and many others. The main target of the antibodies is the activated protein C (APC) system, the elimination of which can manifest itself as a thrombotic complication. The aim of this study was to determine the thrombogenicity of antibodies using a modified protein C-activated thrombin generation assay (TGA) on a group of 175 samples suspected of APS. TGA was measured with/without APC and the ratio of both measurements was evaluated (as for APC resistance), where a cut-off was calculated ≤4.5 (90th percentile) using 21 patients with heterozygous factor V Leiden mutation (FV Leiden heterozygous). Our study demonstrates the well-known fact that multiple positivity of different aPLs is a more severe risk for thrombosis than single positivity. Of the single antibody positivity, LA antibodies are the most serious (p value < 0.01), followed by aCL and their subgroup anti-DI (p value < 0.05). Non-criteria antibodies anti-annexin V and anti-PT/PS has a similar frequency occurrence of thrombogenicity as LA antibodies but without statistical significance or anti-β2GPI1 positivity. The modified TGA test can help us identify patients in all groups who are also at risk for recurrent thrombotic and pregnancy complications; thus, long-term prophylactic treatment is appropriate. For this reason, it is proving increasingly beneficial to include the determination antibodies in combination with modified TGA test.

• MeSH
• antifosfolipidové protilátky MeSH
• antifosfolipidový syndrom * komplikace   MeSH
• antikardiolipinové protilátky MeSH
• beta-2-glykoprotein I MeSH
• fosfatidylseriny MeSH
• lidé MeSH
• protein C MeSH
• protrombin MeSH
• těhotenství MeSH
• trombin MeSH
• trombóza * etiologie   MeSH
• Check Tag
• lidé MeSH
• těhotenství MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

OBJECTIVES: This study compared the use of bortezomib in different combination regimens in newly diagnosed multiple myeloma (NDMM) patients who were transplant ineligible. PATIENTS AND METHODS: We analyzed data from the Registry of Monoclonal Gammopathies (RMG) of the Czech Myeloma Group (CMG) to provide real-world evidence of outcome for 794 newly diagnosed MM transplant ineligible patients. The most frequently used regimen was VCd (bortezomib-cyclophosphamide-dexamethasone) (47.5%) over VMP (bortezomib-melphalan-prednisone) (21.7%), BDd (bortezomib-doxorubicin-dexamethasone) (9.8%), and VTd (bortezomib-thalidomide-dexamethasone) (2.9%). RESULTS: The overall response rate (ORR) was 69.2% (478/691), including 12.6% (≥ CR); 34.7% very good partial responses (VGPR); and 21.9% partial responses (PR). Among triplet regimens, VMP was the most effective regimen compared to VCd, BDd, and VTd. Median PFS was 22.3 vs. 18.5 vs. 13.7 vs. 13.8 mo, (P = .275), respectively, and median OS was 49 vs. 41.7 vs. 37.9 vs. 32.2 mo (P = .004), respectively. The most common grade 3-4 toxicities were anemia in 17.4% and infections in 18% of patients. CONCLUSION: Our study confirmed that bortezomib-based treatment is effective and safe in NDMM transplant ineligible patients, especially VMP, which was identified as superior between bortezomib-based induction regimens not only in clinical trials, but also in real clinical practice.

• MeSH
• bortezomib terapeutické užití   MeSH
• cyklofosfamid terapeutické užití   MeSH
• dexamethason terapeutické užití   MeSH
• doxorubicin terapeutické užití   MeSH
• lidé středního věku MeSH
• lidé MeSH
• melfalan terapeutické užití   MeSH
• mnohočetný myelom diagnóza   farmakoterapie   mortalita   patologie   MeSH
• prednison terapeutické užití   MeSH
• přežití bez známek nemoci MeSH
• protokoly protinádorové kombinované chemoterapie terapeutické užití   MeSH
• registrace * MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• thalidomid terapeutické užití   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH

Cílem standardu je stanovit základní diagnostické a především terapeutické postupy v péči o pacienty s vrozenou hemofilií a s inhibitorem koagulačního faktoru VIII, resp. faktoru IX. Úprava původního konsenzuálního doporučení vychází z vývoje poznání a reflektuje nová data i vstup nových léčiv, jež mění léčebné přístupy.

The aim of these guidelines is to set-up and standardize diagnostic as well as therapeutic approaches to patients suffering from congenital haemophilia and factor VIII or factor IX inhibitors. Update of the original consensual guidelines is based on evolution in our experience and reflects new available data as well as the advent of new drugs revolutionising our therapeutic approach.

• MeSH
• chirurgie operační MeSH
• faktor VIII analýza   MeSH
• hemofilie A * diagnóza   terapie   MeSH
• inhibitory koagulačních faktorů krev   MeSH
• lidé MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• směrnice pro lékařskou praxi MeSH

Cílem standardu je stanovit základní diagnostické a především terapeutické postupy v péči o pacienty s hemofilií na základě aktualizovaných vědeckých poznatků. Úprava původního konsenzuálního doporučení odráží nová data i příchod nových léků a doporučuje optimální dávkování pro léčbu. Stručně je popsán systém péče o hemofilii v České republice.

The aim of these guidelines is to set-up and standardize diagnostic as well as therapeutic approaches to patients with haemophilia according to updated scientific evidence. Update of the original consensual guidelines reflects new available data plus advent of new drugs and recommends the optimal dosage for treatment. The system for haemophilia care in Czech Republic is briefly described.

• Klíčová slova
• profylaxe,
• MeSH
• hemofilie A * diagnóza   terapie   MeSH
• hemofilie B * diagnóza   terapie   MeSH
• lidé MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• směrnice pro lékařskou praxi MeSH

Antiphospholipid syndrome (APS) is a hypercoagulation condition associated with the incidence of heterogenic antiphospholipid antibodies (aPLs), which non-specifically affect hemostasis processes. APS is clinically manifested by recurrent arterial and venous thromboses and reproduction losses. The aPL antibodies, which may induce clinical manifestations of APS, include criteria antibodies anti-cardiolipin, anti-β2-glycoprotein-I, and lupus anticoagulant, but also non-criteria antibodies, for example anti-β2-glycoprotein-I domain I, anti-phosphatidylserine/prothrombin, anti-annexin V, and many others. APS occurs mostly in patients of younger and middle age, most frequently in females. Laboratory diagnostics of APS are quite difficult, as they include a wide spectrum of examining methods, which are based on various principles of detection and are performed using various laboratory techniques. The objective of the review is to describe the current state of potentially examined biomarkers and methods in APS diagnostics. The aforementioned biomarkers are lupus anticoagulant, anti-β2-glycoprotein-I, anti-cardiolipin, anti-β2-glycoprotein-I domain I, anti-phosphatidylserine/prothrombin, anti-β2-glycoprotein-I IgA, anti-cardiolipin IgA, anti-annexin V and II, anti-prothrombin, anti-cardiolipin/vimentin, anti-protein S/protein C, and antibodies against phospholipid antigens for whose diagnostics we may use some of the methods established for a long time and some of the modern methods-the coagulation method for the determination of lupus anticoagulant (LA), enzyme-linked imunosorbent assay (ELISA), chemiluminescence analysis (CLIA), multiplex fluorescence flow immunoassay (MFFIA), fluorescence enzyme immunoassay (EliA), line immunoassay (LIA), multiline dot assay (MLDA), and thin-layer chromatography (TLC). Conclusion: Antibodies against phosphatidylethanolamine, phosphatidic acid, phosphatidylserine, phosphatidylinositol, cardiolipin/vimentin complex, and annexin V are currently the most studied new markers. However, these assays have not been standardized until now, both from the laboratory and clinical point of view. In this review we summarize the evidence of the most studied aPL markers and their potential clinical significance in seronegative APS (SN-APS).

• Publikační typ
• časopisecké články MeSH
• přehledy MeSH