The journal Transfuze a hematologie dnes (Transfusion and Haematology Today) is the official medium of the Society for Transfusion Medicine, the Czech Society of Haematology, and the Czech Society for Thrombosis and Haemostasis of the Czech Medical Society of J. E. Purkyně. Publisher of the journal is the Czech Medical Society of JE Purkyně. Issuer is the company Care Comm. Issues published so far and information about the journal can be found at the following address (after free registration): https://www.prolekare.cz/casopisy/transfuze-hematologie-dnes
Treatment of Relapsed/Refractory Diffuse Large B-Cell Lymphoma and the Real-World Data Perspective
Management of relapsed/refractory diffuse large B-cell lymphoma (R/R DLBCL) has undergone significant transformation in recent years. Second-line therapy has shifted from conventional salvage regimens toward immunotherapy-based approaches. CAR T-cell therapy is now the preferred option for patients with early relapse or primary refractory disease, while salvage chemoimmunotherapy followed by autologous stem cell transplantation consolidation remains reserved for younger, fit individuals with late relapses. Bispecific antibodies (BiAbs) combined with chemotherapy (i.e., glofitamab plus GemOx) could be effective alternatives for transplant-ineligible patients. Additional novel options include Pola-BR and tafasitamab-lenalidomide, particularly for elderly or frail individuals. In third-line and later settings, treatment is increasingly individualized. CAR T-cell therapy remains the preferred modality if not previously administered; otherwise, BiAbs (glofitamab, epcoritamab, odronextamab), antibody-drug conjugates (loncastuximab tesirine, brentuximab vedotin), and lenalidomide-based regimens are considered. Real-world data from the NiHiL registry support the clinical utility of these novel agents and demonstrate improved survival outcomes following their integration into routine clinical practice.
Copyright (c) 2025 Transfuze a hematologie dnes
What can we expect from venetoclax + azacitidine (Ven + Aza) treatment in patients with acute myeloid leukemia (AML): Results from the VIALE-A study and post-hoc analyses.
In 2025, the combination of azacitidine (Aza) and venetoclax (Ven) is the standard treatment for older patients with acute myeloid leukemia (AML) who are not candidates for intensive induction chemotherapy. Ven + Aza has been shown to increase complete remission rates and prolong overall survival. The current treatment regimen is based on the results of the VIALE-A study.
Copyright (c) 2026 Transfuze a hematologie dnes
Úvod do současné léčby první linie akutní myeloidní leukémie
Akutní myeloidní leukémie (AML) je onemocnění vznikající v důsledku proliferace klonálních hematopoetických buněk. AML je nejčastěji diagnostikována u starších osob s mediánem věku 66 - 68 let. I přes zavedení řady nových léků jsou výsledky léčby neuspokojivé. Odhadované 5leté přežití je přibližně 30-35 %, kdy u mladších pacientů se pohybuje okolo 50 %, u starších pacientů nad 60 let je méně než 20 %. V posledních 10 letech byly popsány molekulárně-genetické patologické změny AML což vedlo k lepšímu pochopení biologického chování AML, ale také k identifikaci terapeutických cílů a vývoji nových cílených terapií. Od roku 2017 bylo schváleno 12 nových léků nebo kombinovaných režimů pro léčbu. V tomto sdělení jsou popsány nové aspekty první linie léčby AML (problematika APL není do sdělení zahrnuta).
akutní myeloidní leukémie, indukční terapie, intenzivní léčba, neintenzivní léčba
Antimykotická profylaxe u pacientů s akutní myeloidní leukémií léčených venetoklaxem s azacytidinem
Invazivní mykotické infekce (invasive fungal diseases, IFD) představují závažnou příčinu morbidity a mortality u pacientů s akutní myeloidní leukémií (AML) léčených intenzivně, zejména po indukční chemoterapií a alogenní transplantaci krvetvorných buněk. Kombinace bcl-2 inhibitoru venetoklaxu s hypometylační látkou azacytidinem (VEN+AZA) se stala standardní léčbou u pacientů nevhodných pro intenzivní chemoterapii významně zlepšující jejich výsledky, a to především s následným provedením alogenní transplantace. Terapie však může být spojena s protrahovanou neutropenií a dalšími faktory významně zvyšujícími riziko IFD. Publikované retrospektivní práce udávají velmi různorodou četnost IFD (0,5 %- 26 %) a efekt antimykotické profylaxe dané velkou heterogenitou hodnocených kohort, mírou použití antimykotické profylaxe a jejího typu, definicí IFD a dalšími proměnnými. Většina prací nicméně neprokázala vliv profylaxe na frekvenci IFD, i když musí být zohledněno jejich čistě retrospektivní hodnocení. Navíc v rámci lékových interakcí může představovat kombinace VEN a azolové antimykotikum určité nebezpečí ve smyslu navýšení toxicity VEN. Na základě dostupných dat není nutné profylaxi všeobecně indikovat, avšak pouze u definovaných rizikových skupin pacientů (relabovaná/refrakterní AML, IFD v anamnéze, protrahovaná hluboká neutropenie). Cílem tohoto článku bylo shrnout dostupná data o významu a bezpečnosti antimykotické profylaxe u pacientů s AML léčených režimem VEN+AZA. Souhrnná práce zdůrazňuje potřebu prospektivních studií a rizikové stratifikace k detailnímu posouzení role AFP v této kohortě pacientů.
Venetoclax plus azacitidine as a bridge treatment to allogeneic stem cell transplantation in acute myeloid leukaemia patients
Allogeneic hematopoietic cell transplantation is the most effective form of consolidation therapy for patients with acute myeloid leukemia. The combination of venetoclax and azacitidine represents an alternative to initial therapy with lower toxicity and high rate of achieved remissions, enabling subsequent transplantation even in patients initially unsuitable for intensive therapy. The article describes the possibilities of using the combination of venetoclax and azacitidine as a bridge to allogeneic hematopoietic cell transplantation.
acute myeloid leukaemia, venetoclax, azacitidine, bridging therapy, allogeneic transplantation.
Principles of Effective Hemotherapy Interdisciplinary consensus statement
Effective hemotherapy is a therapeutic procedure in which blood components and SD plasma are applied with maximum safety and treatment effectiveness, while minimizing known risks. Important therapeutic procedures include Patient Blood Management, which represents simple and cost-effective evidence-based recommendations, divided into three thematic areas: correction of preoperative anaemia, reduction of intraoperative and postoperative blood loss, and guidelines for the indication of transfusion. A consensually developed framework recommendation by professional societies defines the basic types of blood components and plasma derivatives intended for transfusion, their general indications in children and adults, their selection according to blood group compatibility, as well as possible adverse effects after their administration.
effective hemotherapy, Patient Blood Management, blood components, plasma SD, indication for transfusion, adverse effects of transfusion
Recommendation of the Society for Transfusion Medicine No. 20 Cold red blood cell antibodies testing before cardiothoracic surgery on cardiopulmonary bypass
Cold red blood cell antibodies are usually clinically insignificant. However, they may have an adverse clinical impact in patients undergoing cardiac surgery with the use of cardiopulmonary bypass with cooling of the patient's blood. We present consensus recommendation for the screening and titration of cold red blood cell antibodies in these patients.
cold RBC antibodies, cardiac surgery, cardiopulmonary bypass
Folikulární lymfom: proměna léčebného paradigmatu směrem k chemo-free přístupům
Léčba folikulárního lymfomu (FL) se v posledních letech významně mění směrem k biologicky cíleným, chemo‑free přístupům. U pacientů s nízkou nádorovou náloží zůstává standardem sledování („watch‑and‑wait“) nebo monoterapie rituximabem, zatímco symptomatické onemocnění vyžaduje imunochemoterapii (nejčastěji bendamustin-rituximab, R‑CHOP, obinutuzumab‑bendamustin). U nemocných s relabovaným onemocněním se uplatňují režimy založené na protilátkách (rituximab‑lenalidomid (R²), tafasitamab‑R²), malé molekuly (zanubrutinib‑obinutuzumab) a epigenetická léčba (tazemetostat). Nové na T‑lymfocytech založené terapie – bispecifické protilátky (mosunetuzumab, epcoritamab, odronextamab) a CAR‑T buňky (axi‑cel, tisa‑cel, liso‑cel) – dosahují léčebných úspěchů i u vysoce předléčených nemocných. Moderní monitorovací metody, zejména PET/CT a ctDNA, umožňují přesnější hodnocení léčebné odpovědi a otevírají cestu k individualizované terapii. Trendem je časově omezená, méně toxická léčba s cílem dosažení dlouhodobé remise („funkčního vyléčení“) při zachování kvality života.
folikulární lymfom, imunochemoterapie, bispecifické protilátky, buněčná terapie, funkční vyléčení
Dopis redakci - prosím bez abstraktu.
Early Isolated Central Nervous System Relapse of Mantle Cell Lymphoma Successfully Treated with Ibrutinib and Chimeric Antigen Receptor T-Cell Therapy – A Case Report
Mantle cell lymphoma (MCL) is a relatively rare and clinically distinct subtype of non-Hodgkin lymphoma. Involvement of the central nervous system is an uncommon manifestation at both diagnosis and recurrence. Central nervous system (CNS) relapse more frequently occurs in the context of aggressive disease behavior and portends a poor prognosis. However, advances in therapeutic strategies for MCL have opened the possibility of achieving durable remission, even in this high-risk presentation. We report a case of a patient who developed an early, isolated CNS relapse following autologous stem cell transplantation. The patient was successfully treated with a sequential treatment protocol combining chemoimmunotherapy, ibrutinib and chimeric antigen receptor T-cell therapy.
Treatment of Patients with Relapsed or Refractory Diffuse Large B-Cell Lymphoma in the Czech Republic in 2025
Diffuse large B-cell lymphoma (DLBCL) represents a heterogeneous group of diseases within the category of non-Hodgkin lymphomas, generally associated with a relatively good prognosis. First-line treatment cures approximately 60% of patients. About 40% of patients, however, experience relapse or have primary refractory disease. The prognosis for these patients was historically very poor. In the treatment of relapsed/refractory DLBCL, the timing of first-line treatment failure is currently a key factor. If the failure occurs within 12 months after completion of first-line therapy and the patient is eligible for CAR-T therapy, axicabtagene ciloleucel (axi-cel) or lisocabtagene maraleucel (liso-cel) should be administered. If the relapse occurs after more than 12 months and the patient is suitable for high-dose therapy, salvage immunochemotherapy followed by autologous stem cell transplantation is indicated. If the patient is not eligible for such high-dose therapy, immunochemotherapy remains the standard second-line treatment. In third and later lines of therapy, more treatment options are now available. These include the bispecific antibody glofitamab. If the patient has not previously received CAR-T therapy, CAR-T treatment is also an option. In cases where these treatment modalities fail, loncastuximab tesirine is available in the Czech Republic for third and later lines of therapy. Other treatment options include the bispecific antibody epcoritamab, the combination of tafasitamab + lenalidomide, or the Pola-BR regimen. However, these treatments are not currently reimbursed, and approval must be requested from the relevant health insurance company under Paragraph 16. A very promising approach is the combination of glofitamab with the GEMOx regimen for second and later lines, based on data from the STARGLO clinical trial. However, this treatment is also not yet reimbursed in the Czech Republic. Looking ahead, it is expected that these new treatment modalities will shift into earlier lines of therapy, but results from ongoing clinical trials must be awaited. Nonetheless, it can be stated that the prognosis for patients with relapsed/refractory DLBCL has significantly improved in recent years.
lymphoma, relapse, DLBCL, CAR-T, axi-cel, liso-cel, bispecific antibodies, glofitamab, prognosis
Guidelines for the management of adverse events following CD19 CAR-T cell therapy in adult patients with lymphomas and acute lymphoblastic leukemia: consensus of expert panel from certified centers in Czechia and Slovakia for 2025
Autologous chimeric antigen receptor T-cells (CAR-T cells) have become a standard of care for hematological malignancies, particularly non-Hodgkin B-cell lymphomas and acute B-lymphoblastic leukemia. An expert panel from all certified centers in Czechia and Slovakia presents these guidelines for the grading, prophylaxis and treatment of common adverse events. These include cytokine release syndrome, neurotoxicity syndrome, hematological toxicity, infections, and other less frequent but specific toxicities. These guidelines are adapted to the local state-of-the-art and are supplemented with concise summary tables designed for use in daily clinical practice.
Generační změna terapie klasického Hodgkinova lymfomu
Klasický Hodgkinův lymfom (cHL) je v moderní hematoonkologii často vnímán jako příběh úspěchu, a to zejména u pacientů mladších 60 let, kde současné léčebné postupy umožňují vyléčení u přibližně 80-90 % nemocných v závislosti na klinickém stadiu. Tento pokrok je výsledkem dlouhodobého výzkumu a zavedení účinných kombinací chemoterapie a radioterapie. Za touto statistikou se však skrývají významné výzvy: akutní i pozdní toxicita standardní léčby a omezené možnosti efektivní terapie pro starší pacienty či nemocné s relabujícím/refrakterním (R/R) průběhem. Klasický HL tak zůstává dynamickým polem vývoje, kde hledání rovnováhy mezi účinností a tolerancí léčby pokračuje. V posledních letech jsme svědky zásadní generační změny v terapeutickém přístupu k léčbě tohoto typu lymfomu, která je poháněna rozvojem cílené léčby a imunoterapie. Nové léky, jako je imunokonjugát (brentuximab vedotin) a inhibitory kontrolních bodů imunity (tzv. immune checkpoint inhibitory - nivolumab, pembrolizumab), změnily léčebné paradigma zejména ve 3. a vyšší linii léčby a postupně v současné době nacházejí nově své místo i v 1. a 2. linii. Tento posun přináší nejen nové možnosti pro zvýšení účinnosti terapie především u starších nemocných a u R/R formy nemoci, ale také potenciál pro snížení toxicity a zlepšení kvality života mladších pacientů. Cílem tohoto sdělení je shrnout současné poznatky o implementaci inovativních přístupů do 1. a 2. linie léčby cHL a diskutovat jejich dopad na klinickou praxi v kontextu měnícího se terapeutického paradigmatu v rámci moderní personalizované medicíny v českých podmínkách.
CAR-T lymphocytes have become an integral part of the treatment arsenal for hematological malignancies, including B-non-Hodgkin's lymphomas (B-NHL). Currently, we have four registered anti-CD19 products available for the treatment of B-NHL. For the treatment of large B-cell lymphomas (LBCL) and follicular lymphoma (FL), axi-cel, liso-cel, and tisa-cel are available, while brexu-cel is registered for the treatment of mantle cell lymphoma (MCL). Registered indications are mostly for the treatment of 3rd and subsequent lines, but for LBCL we can use axi-cel and liso-cel already in the second line. The presented work addresses: 1. general selection of suitable patients in terms of age and comorbidities, 2. pre-treatment examinations, 3. estimating the risks of adverse effects, 4. the adverse effects, 5. expected outcomes, 6. decision-making algorithms or elements of decision-making algorithms for each malignancy. In the chapter about LBCL, case report is included. The main aim of the work is to assist clinicians who first diagnose relapsed patients and decide on their referral to specialized centers.
Modern therapeutic approaches in the treatment of relapsed and refractory marginal zone lymphoma
Marginal zone lymphomas (MZL) are a group of indolent B-cell lymphomas originating from B-lymphocytes of the marginal zone of lymphoid follicles of lymph nodes, spleen, or extranodal tissues. The group includes five distinct clinical entities, which together represent the third most common group of non-Hodgkin lymphomas in Czech Republic. In recent years, there has been a significant broadening of treatment options beyond conventional immunochemotherapy for relapsed refractory (R/R) disease. In the MAGNIFY Phase IIIb multicenter study, the combination of lenalidomide and rituximab followed by rituximab maintenance therapy in 76 patients with R/R MZL after failure of ≥ 1 prior line of therapy.achieved an overall response rate (ORR) of 64 % (49/76), complete remission (CR) in 39 % (30/76), and a median progression-free survival (PFS) of 41.2 months. Bruton's tyrosine kinase inhibitors, particularly zanubrutinib, have significantly expanded the treatment options for R/R disease. In the MAGNOLIA phase II, zanubrutinib led to an ORR of 68.2 % (45/66) and CR of 25.8 % (17/66) in patients who had received at least one prior line of anti-CD20 therapy. The two-year estimated PFS) was 70.9 %, and the toxicity profile was favorable. Therapy with T lymphocytes with chimeric antigen receptors also appears highly promising, particularly the liso-cel, which achieved an ORR of 95.5 % (63/66), a CR of 62.1 % (41/66) and an estimated 2year PFS of 85.7 % in R/R patients with a median of 3 previous lines of treatment in the TRANSCEND FL trial. Data on the efficacy and safety of bispecific antibodies in MZL is currently limited, with relatively the most information available on odronextamab. In the phase II ELM-2 trial, odronextamab monotherapy led to an ORR and CR of 79.3 % (23/29) in patients after ≥ 2 lines of systemic therapy.
Concomitant BCR::ABL positive chronic myelogenous leukemia and Ph-negative myeloproliferative neoplasm – case reports of a single centre institution
Chronic myeloid leukemia (CML) is defined by the BCR::ABL1 fusion resulting from t(9;22)(q34;q11). According to the 5th edition of the WHO classification of Haematolymfoid Tumours it belongs to the group of myeloproliferative neoplasms (MPN) with polycythaemia vera, essential thrombocythaemia, primary myelofibrosis, chronic neutrophilic leukaemia, chronic eosinophilic leukemia, juvenile myelomonocytic leukaemia and myeloproliferative neoplasms, not otherwise specified. Rare cases with co-occuring BCR::ABL1 positive CML and Ph negative MPN have been identified. We present a detailed description of clinical, cytomorphological and molecular characteristics of 4 cases of BCR::ABL1 positive CML patients with coexiting Ph negative MPN with driver mutations.
chronic myeloid leukemia, tyrosinkinase inhibitors, myelofibrosis, ruxolitinib, splenomegaly
Moderní strategie léčby CLL: význam fixních režimů a jejich opětovného využití
The introduction of fixed-duration treatment regimens represents a significant advancement in managing chronic lymphocytic leukemia (CLL), as it allows the achievement of deep remissions without the need for continuous medication. Combinations of targeted agents—particularly venetoclax with obinutuzumab or a Bruton tyrosine kinase inhibitor—have demonstrated high efficacy and a favorable safety profile while offering patients the benefit of so-called "treatment holidays." This approach is not only clinically effective but also cost-efficient. An increasingly discussed topic is the potential for re-administration of these regimens in the event of relapse—so-called retreatment. Data from the Murano study and real-world experience suggest that retreatment with venetoclax-based regimens may be effective and well tolerated in selected patients, mainly when relapse occurs after a prolonged treatment-free period. This strategy is already reflected in both Czech and international clinical guidelines, which consider retreatment a reasonable option for patients whose initial response lasted at least 1–2 years. In summary, fixed-duration regimens represent a practical, modern approach to CLL therapy, and the possibility of their repeated use opens new perspectives for sequential treatment strategies in clinical practice. However, as current evidence is derived mainly from retrospective analyses and small patient cohorts, prospective studies with well-defined criteria and long-term follow-up are essential to confirm this approach's efficacy and safety definitively.
Spontánní remise u akutní myeloidní leukémie
SUMMARY Acute Myeloid Leukaemia (AML) is a malignant hematopoietic disorder with high mortality, which remains challenging to treat despite advances in therapy, particularly in older patients with comorbidities and those at high risk. The vast majority of patients require intensive treatment, and achieving consolidation of remission and cure often necessitates allogeneic hematopoietic stem cell transplantation. Nevertheless, there are extremely rare cases of spontaneous remission of AML, which are typically of short duration. Hypotheses regarding the underlying mechanism of spontaneous remission frequently highlight an immune-mediated effect, often following an infection. This study focuses on a review of available case reports describing instances of spontaneous remission between 2004 and 2023. In total, we identified 27 cases in patients with a median age of 59 years (range 28–77 years), 19 of whom were male. The most common subtypes of AML were M2 and M4, with a normal karyotype being the most frequently reported cytogenetic finding. The duration of spontaneous remission ranged from 0.3 to 120 months, with a median of 6 months. We also briefly describe our own experience with a case of spontaneous remission in a 75year-old woman with a cytogenetic finding of t(9;11)(p21;q23) and trisomy 8, whose spontaneous remission has currently lasted 48 months.
spontaneous remission, acute myeloid leukaemia
Recommendations for chronic lymphocytic leukaemia diagnosis and therapy
The treatment of chronic lymphocytic leukaemia (CLL) has changed dramatically in recent years. New oral signalling pathway targeted inhibitors have significantly improved the prognosis of patients with CLL. Currently, two main groups of small molecules are used, namely Bruton's tyrosine kinase (BTK) inhibitors and BCL-2 inhibitors. However, the results of new clinical trials directly impacting routine clinical practice are rapidly accumulating. We therefore present updated recommendations for the diagnosis and treatment of CLL, based on a comprehensive analysis of the current literature and following the principles of evidence-based medicine.
MYC gene and its abnormalities with a focus on aggressive B-cell lymphomas
The MYC gene encodes the Myc protein, which is one of the most important transcription factors regulating a wide range of cellular functions, including cell proliferation, differentiation and apoptosis. Deregulation of the MYC gene occurs at various levels and its increased expression is associated with a variety of cancers. Deregulation of the gene through chromosomal rearrangement is typical for B-cell lymphomas and is usually accompanied by a more aggressive nature of the disease with an unfavourable prognosis. B-cell lymphomas with a proven MYC gene rearrangement show high variability in the localization of breakpoints in the 8q24 region. The rearrangement involves various translocation partners, both immunoglobulin (IG) genes and non-immunoglobulin (non-IG) genes. The involvement of IG genes as translocation partners of MYC is considered a negative prognostic factor. Co-occurring aberrations of other oncogenes have a significant impact on the prognosis of patients with MYC gene rearrangements. The presence or absence of MYC, BCL2 and BCL6 gene rearrangements, together with morphological assessment, is the defining criterion for the classification of aggressive B-cell lymphomas according to the 5th revised WHO classification (2022). The most suitable method for detecting rearrangements is fluorescence in situ hybridization (FISH), which can be used both on native material and on tissue sections from paraffin blocks. This review discusses the physiological function of the MYC gene, its deregulation, types of chromosomal aberrations involving MYC gene and their role in cancer pathogenesis with a focus on B-cell lymphomas.
MYC gene, Burkitt lymphoma (BL), diffuse large B-cell lymphoma (DLBCL), high-grade B-lymphoma (HGBL), fluorescence in situ hybridization (FISH)
Automated digital cytomorphology increases the reliability of bone marrow diagnostics
Thrombotic microangiopathies represent a heterogeneous group of diseases of various etiology, whose common feature is damage to the endothelium of small blood vessels. Typical laboratory findings include thrombocytopenia and microangiopathic hemolytic anemia, along with signs of organ damage (most commonly affecting the kidneys). In the differential diagnosis, it is necessary to differentiate, among other things, autoimmune diseases with multi-organ failure. This case report presents the case of a young woman who developed multi-organ failure in the early postpartum period. For this patient, it was the first manifestation of systemic lupus erythematosus. Despite prompt diagnosis and treatment, the course of hospitalization was complicated—both due to the underlying disease and due to severe infections that required adjustments to her immunosuppressive therapy. The patient was discharged to outpatient care after nearly four months of intensive treatment, and her condition continues to improve.
Successful treatment of MDS-IB2 and chronic lymphocytic leukemia with venetoclax - case report
In this case report, we present the case of a 61-year-old female patient with chronic lymphocytic leukemia (CLL) and myelodysplastic syndrome with increased blasts 2 (MDS-IB2). The rare coincidence of these two diseases has made it possible to reaveal the therapeutic potential of venetoclax in patients with high-risk MDS. While venetoclax is a common therapeutic option in the diagnosis of CLL, it is still the subject of clinical trials in the management of MDS treatment. Due to the presumed therapeutic overlap of the mentioned BCL2 inhibitor, it was chosen in the first line for the treatment of CLL in our case report. Although the treatment was initially complicated by severe prolonged pneumonia, after a short duration of venetoclax and azacitidine both diseases were in remission and the patient was able to undergo allogeneic hematopoietic cell transplantation (HSCT). Apart from corticosensitive acute GvHD of the skin and liver, the HSCT was performed without the feared infectious complications. Even 35 months after the HSCT, the complete treatment response with 100% chimerism persists.
Results after first-line treatment of non-Hodgkin and Hodgkin lymphoma treated between 2013 and 2022 in Department of Hematooncology, Faculty Hospital in Ostrava, Czech Republic.
Lymphomas are a heterogenous group of blood malignancies, having different histopathological, immunohistochemical and clinical features, as well as different prognosis and therapy. In our retrospective analysis, we put forward results after 1st line of therapy of 668 patients with non-Hodgkin lymphoma (NHL) and 142 patients with Hodgkin lymphoma (HL) treated between 2013 and 2022 in Department of Hematooncology, Faculty Hospital in Ostrava, Czech Republic. We focus mainly on diffuse large B-cell lymphoma (DLBCL), follicular lymphoma (FL) and HL with major interest in overall response rate (ORR), progression free survival (PFS) and overall survival (OS). In the DLBCL group, 82% (221/274) patients received R-CHOP/ R-CHOP-like treatment. The complete remission (CR) rate in this cohort was 80% (177/221), median PFS and OS was 122 months and five-year PFS and OS was 61% and 66%, respectively. Low IPI risk was associated with better PFS and OS, which was also partially true for GCB (Germinal Centre B-cell like) immunophenotype. Of 134 patients with FL, 60% (80/134) received chemotherapy in combination with anti-CD20 monoclonal antibody as 1st line treatment, with 95% ORR (76/80) and 76% CR (62/80). The median PFS and OS was not reached for the whole group. The five-year PFS and OS was 63% and 86%, respectively. Better survival rate was observed in patients with localized Lugano stage at the time of diagnosis and low FLIPI. Of 142 patients with HL, 96% (136/142) had classical HL (cHL). For this group, median PFS and OS was not reached. The five-year PFS and OS was 75% and 86%, respectively. The respective five-year PFS and OS in early stages was 94% and 100%, in intermediate stages 84% and 90%, in advanced stages 68% and 81%. Our observed results of lymphoma patients with standard treatment are comparable to results from clinical trials and other authors’ analyses from daily clinical practice.
difuzní velkobuněčný B-lymfom, folikulární lymfom, Hodgkinův lymfom, celkové přežití, doba do progrese, prognóza
Léčba mnohočetného myelomu s projevy choroby z ukládání lehkých řetězců (Light Chain Deposition Disease – LCDD). Kompletní remise s negativitou minimální reziduální nemoci po léčbě daratumumabem, dexametazonem a lenalidomidem. Popis případu a přehled lite
Summary Treatment of multiple myeloma with manifestations of Light Chain Deposition Disease (LCDD). Complete remission with minimal residual disease negativity following treatment with daratumumab, dexamethasone, and lenalidomide. A case report and a review of the literature. Light chain deposition disease (LCDD) is a term used for kidney damage by clonal kappa light chain deposits in an amorphous unorganized form. In the kidneys, it causes a decrease in filtration, nephrotic syndrome with microscopic hematuria and proteinuria. Changes induced by FLC kappa binding to mesangium structures cause irreversible kidney damage. Similar deposits can damage the heart, lungs and liver. LCDD is more commonly diagnosed in patients with non-malignant monoclonal gammopathy who, had it not been for kidney damage, would have met the MGUS diagnosis, than in people who met the criteria for multiple myeloma. The first symptom of the disease in the described case was nephrotic syndrome. Kidney biopsy showed evidence of LCDD-type kidney damage and subsequent haematological examination showed multiple myeloma, although no lytic changes were present. The first treatment line based on bortezomib, completed with high-dose chemotherapy, achieved a 31-month complete remission of the disease. Then, due to increasing FLC kappa concentrations and increasing FLC kappa/lambda ratio, it was necessary to initiate a second line of treatment based on antiCD38 antibody. After 24 months of treatment with daratumumab, lenalidomide and dexamethasone, complete haematological remission was achieved with minimal residual disease negativity. Thanks to the early initiation of second-line treatment, kidney function remained without further deterioration. The fate of the kidneys in patients with LCDD, which causes irreversible kidney damage, depends on early diagnosis of light chain monoclonal gammopathy, i.e. FLC examination in people with oedema of unclear etiology or with increasing creatinine concentration, early histological diagnosis and effective treatment leading to suppression of nephrotoxic light chain formation, i.e. to achieve complete remission with minimal residual disease negativity. It is essential not to forget to perform examinations in patients with fluid retention and oedema, which can prove nephrotic syndrome. And if ti is proven, ask specialists for a differential diagnosis of nephrotic syndrome, which can lead to evidence of kidney damage by monoclonal gammopathy. Key words: Light chain deposition disease, free light chain.
Trends in allogeneic hematopoietic cell transplantations - analysis of the activities of the transplantation center of the University Hospital Pilsen in the period 2001-2021.
Allogeneic hematopoietic cell transplantation (alloHCT) is evolving both in terms of indications and execution. We retrospectively evaluated this evolution in 855 consecutive transplanted patients between 2001 and 2021 by comparing 3 7-year time periods: 2001-2007 (n=257, period/cohort A), 2008-2014 (n=304, period/cohort B) and 2015-2021 (n=294, period/cohort C). There was a significant increase in the median age of patients over the study periods - 50 years (range 20-68) vs. 53 years (19-71) vs. 55 years (20-74), p<0,0001. Similarly, the proportion of patients ≥70 years increased from 0 in A to 23 (8 %) in C, p<0,0001. This reflects a decrease in the use of myeloablative regimens (43 % in period A vs. 24 %/28 % in periods B/C, p<0,0001). Changes in donor type were substantial, with an increase in haploidentical donors in period C: 27 % vs. 1 %/0 % in periods B/A (p<0,0001). Concurrently, the proportion of identical siblings decreased (41 % to 26 % and 9 % respectively, p<0,0001), while the proportion of unrelated donors remained stationary (60 % vs. 73 % vs. 64 %). Bone marrow as a source of hematopoietic cells increased from 11 % to 20 % in period B (p=0,0049), then became stationary (18 %, p=0.943). CNI/MTX as GVHD prophylaxis was the mainstay in all transplant types except haploidentical, where PTCY was used exclusively. However, the use of ATG has increased significantly (from 22 % to a recent 55 %, p<0,0001). In all periods, myeloid malignancies (AML/MDS/MPN) were the main indication, with a steady increase (39 % in period A vs. 62 % in period C, p<0,0001). On the other hand, CML and CLL showed a significant decrease (11 %, 4 % and 1 %, p<0,0001, respectively, 14 %, 13 % and 3 %, p<0,0001). The proportion of other indications - ALL, NHL, MM, MH, MPN - remained essentially the same with non-significant variations between periods (e.g. ALL 9 %, 13 % and 9 %, p=0,1608). The analysis documents a trend to transplant increasingly older patients and a predominance of unrelated or haploidentical transplants. Myeloid malignancies remain the dominant indication, but there has been a significant decline in CML and CLL.
allogeneic hematopoietic stem cell transplantation
Je dlouhodobé podávání interferonů u polycythemia vera spojeno s riziky?
Therapy of myeloproliferative diseases with interferons is experiencing a renaissance, there are basically three reasons. Extending the effect of pegylation. Improvement of local tolerance and limitation of cytokine release by pegylation. New and encouraging evidence of efficacy with solid tolerance for pegylated interferons. Individual pegylated interferons differ from each other both from a chemical point of view and also in the three-dimensional structure of positional isomers, while one of the pegylated interferons – ropeginterferon α2b – is made up of a single molecule and has no positional isomers. Isomers of pegylated interferons appear to have different biological activity. The two largest databases of adverse effects, the American FAERS and the European Eudravigilance, show very homogeneously that there are differences in the incidence and severity of adverse effects between pegylated interferons. In addition, these differences confirm the results of controlled phase 2 and 3 clinical trials, which, among other things, monitored the incidence of premature termination of therapy due to adverse effects. Based on the analysis of adverse effects and the results of controlled clinical trials, it can be stated that ropeginterferon α2b has a significantly better tolerability in the indication of polycythemia vera compared to peginterferon α2a and peginterferon α2b, across virtually all classes of adverse effects according to the MedDRA classification. Additionally, a significantly lower proportion of patients have to discontinue ropeginterferon α2b therapy prematurely due to adverse effects compared to peginterferon α2a or peginterferon α2b therapy. Information about the safety of the therapy is important for the doctor in the same way that the information about the clinical effectiveness is important, because the set of this knowledge will help the doctor to make the optimal choice of the drug for the patient.
ukončení (vysazení) terapie z důvodu nežádoucích účinků
Czech and international advancements in the treatment and research of chronic myeloid leukemia
The article highlights a selection from oral presentations held at hematological conferences in the Czech Republic in September 2024, focusing on the treatment and research of chronic myeloid leukemia.
Myelomem indukované kardiovaskulární problémy
S Summary Cardiovascular complications in multiple myeloma Most patients with multiple myeloma (MM) are over 50 years old, with a median age of diagnosis of 70 years and this is the age when cardiovascular disease is common. In patients with MM, the incidence of hypertension has been shown to be higher than in the average population of the same age, and cardiovascular disease is present in 69% of MM patients. Myeloma cells have some pathophysiological mechanisms negatively affecting the cardiovascular system, and the drugs used also have a negative effect. Myeloma cell products can damage the cardiovascular system by the following mechanisms: damage to the heart by light chain deposits in amyloid or amorphous form, hyperviscosity, hyperkinetic heart failure in shunt circulation, pulmonary hypertension, negative effects of anaemia and renal failure on cardiovascular fitness. It is important for patients that their doctor is familiar with these phenomena and adjusts the treatment of MM appropriately so that the benefits always outweigh the side effects. The review summarizes current knowledge of the pathophysiology of cardiovascular complications in patients with symptomatic MM. Keywords: cardiovascular complications of multiple myeloma.
Kardiovaskulární komplikace, které mohou způsobit protimyelomové léky
Cardiovascular complication of anti-myeloma therapy Most patients with multiple myeloma (MM) are over 50 years old, an age when cardiovascular disease is common. Therefore, it is necessary to be aware of the adverse cardiovascular effects of anti-myeloma drugs. Of the classic cytostatics, doxorubicin has a well-known cardiotonic effect. However, high doses of cyclophosphamide, used for collecting hematopoietic stem cells, can also cause cardiovascular complications. Therefore, in case of heart damage caused by AL-amyloidosis, it is recommended to collect stem cells after G-CSF. Doses of melphalan, which are common in hematopoietic stem transplantation, can also have a negative effect on the heart and induce arrhythmias. Of the group of proteasome inhibitors, carfilzomib has the most cardiovascular adverse reactions, after bortezomib they have been described to a much lesser extent, cardiotoxicity is not described for ixazomib. Drugs from the IMiDs group have a predominant procoagulant effect, requiring targeted prophylaxis of thrombosis and pulmonary embolism, but rhythm disturbances have also been described. Treatment with new anti-CD38 monoclonal antibodies is not associated with evident cardiotoxicity, but cardiovascular side effects are described with glucocorticoids, which are the standard premedication for anti-CD antibodies. The aim of this article is to inform about the incidence of these complications associated with antimyeloma treatment. Keywords: cardiovascular complications of multiple myeloma, cyclophosphamide, melphalan proteasome inhibitors, IMiDs drugs, anti-CD38 monoclonal antibodies