Successful treatment of relapsed Waldenström's macroglobulinemia with proteasome inhibitors (bortezomib and subsequently ixazomib) in combination with rituximab and dexamethasone. A case report and review of the of proteasome inhibitors in Waldenström's….

Background: Waldenström's macroglobulinemia (WM) is a very rare disease with an incidence 10times lower than that of multiple myeloma. The incidence of WM is also significantly lower than that of the other CD20+ low-grade lymphomas. The rarity of WM is the reason why registration studies of new drugs used for multiple myeloma or the more common CD20+low-grade lymphomas do not cover WM.

The treatment combination of obinutuzumab, bendamustine and dexamethasone achieved a deeper response than the previous line of treatment in five patients with Waldenström's macroglobulinemia.

Background: Rituximab is already a standard part of the treatment of patients with Waldenström's macroglobulinemia. However, a small proportion of patients develop intolerance to rituximab during administration or the treatment is not very effective. In these patients, we are faced with the question of whether another anti-CD20 monoclonal antibody can be used and what result will be achieved.

Treatment of retroperitoneal fibrosis with rituximab, cyclophosphamide and dexamethasone, followed by rituximab and dexamethasone maintenance, achieved disappearance of pathological PET accumulation of FDG and regression of fibrotic masses after 4 months….

Background: Idiopathic retroperitoneal fibrosis is characterized by the development of inflammatory infiltrates with marked fibrosis along the large retroperitoneal vessels. Rituximab in combination with glucocorticoids constitute an effective therapy, but the responses are not long-lasting. In other similar situations, addition of cyclophosphamide to the combination achieved longer and deeper responses.

Efficacy of ixazomib, lenalidomide, dexamethasone regimen in daratumumab-exposed relapsed/refractory multiple myeloma patients: A retrospective analysis.

We performed retrospective analysis of relapsed/refractory multiple myeloma (RRMM) patients previously exposed to daratumumab treated with ixazomib, lenalidomide, dexamethasone (IRd) regimen in real clinical practice. Our aim was to evaluate efficacy of IRd in these patients and select a subset of patients that would benefit from this treatment the most. In total, we analyzed 43 daratumumab-exposed RRMM patients treated in our center.

Therapy of Castleman's disease with siltuximab - case report and review of literature.

Background: Idiopathic multicentric Castleman disease (iMCD) is characterized by constitutional symptoms, enlarged lymph nodes and laboratory test abnormalities, which are primarily related to the overproduction of interleukin-6 (IL-6). This form (iMCD) was treated earlier with cytostatics used for lymphoma, later with bio-logic therapy as rituximab, immunodulatory drugs and proteasome inhibitors, and in the last years with an anti-IL-6 antibody, siltuximab. Siltuximab is a human-mouse chimeric immunoglobulin G1k monoclonal antibody against human IL-6 approved in the European Union for the treatment of iMCD.

[Successful therapy of retroperitoneal fibrosis due to IgG4-related disease with rituximab, cyclophosphamide and glucocorticoids followed by maintenance therapy wit ritutixmab].

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications, which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. Common symptoms at onset include lower back, abdominal or flank pain, and constitutional symptoms such as malaise, fever, and anorexia and weight loss.

Waldenström's macroglobulinemia - clinical symptoms and review of therapy yesterday, today and tomorrow.

Background: Waldenström macroglobulinemia (WM) is a lymphoplasmocytic lymphoma with immunoglobulin M monoclonal protein. The incidence of this disease is very low (0.4/100,000), so that this disease can be regarded as an orphan's disease.

Daratumumab with lenalidomide and dexamethasone in relapsed or refractory multiple myeloma patients - real world evidence analysis.

We performed real world evidence (RWE) analysis of daratumumab, lenalidomide and dexamethasone (Dara-Rd) versus lenalidomide and dexamethasone (Rd) treatment in relapsed/refractory multiple myeloma patients (RRMM). In total, 240 RRMM patients were treated with Dara-Rd from 2016 to 2022 outside of clinical trials in all major Czech hematology centers. As a reference, 531 RRMM patients treated with Rd were evaluated.

[Therapy of immunoglonuline IgG4 related disease (IgG4-RD)].

Immunoglobulin IgG4 related disease (IgG4-RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the beginning of this century only. Evolving therapy is reviewed in this paper. Glucocorticoids are first choice drug but long administration of glucocorticoids is connected with many adverse effects.

[Langerhans cell histiocytosis (LCH). Overview of symptoms of LCH, which may lead the patients to any of these medical specialists].

Langerhans cell histiocytosis (LCH) is a rare condition with incidence in adults 1-2/1 million, wherein Langerhans cells proliferate abnormally, adversely impacting organs including most frequently bones, skin, lungs, pituitary gland, lymph nodes, gums and other organs. The LCH course varies widely among patients from a self-limiting condition, to one that progresses. But LCH only very rarely culminates in death.

Slow increase of bilirubin concentration during administration of lenalidomide, bortezomib and dexamethasone for multiple myeloma (unmasking previously undiagnosed Gilbert syndrome) and disappearance of necrobiotic xanthogranuloma after complete remission of multiple myeloma.

Background: Lenalidomid ranks among immunomodulatory drugs. There are a few of the more common side effects, like a higher risk of venous trombembolism or diarrhea. Other side effects are rare.

[Multicentric Castlemans disease. Symptoms, diagnostics and therapy].

Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric (UCD) or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8-negative/idiopathic (iMCD).

Necessity of flow cytometry assessment of circulating plasma cells and its connection with clinical characteristics of primary and secondary plasma cell leukaemia.

Plasma cell leukaemia (PCL) is a rare and very aggressive plasma cell disorder. Preventing a dismal outcome of PCL requires early diagnosis with appropriate analytical tools. Therefore, the investigation of 33 patients with primary and secondary PCL was done when the quantity of circulating plasma cells (PCs) using flow cytometry (FC) and morphology assessment was evaluated.

Prognostic significance of lymphocyte patterns in multiple myeloma patients after autologous transplant.

Despite the high efficacy of current induction regimens, most multiple myeloma (MM) patients relapse over time. The link between changes in the immune system and the prognosis of the disease is still not entirely clear. Therefore, we analyzed whether the pattern of bone marrow (BM) lymphocytes during routine BM examination after autologous stem cell transplant (ASCT) is related to disease prognosis or MRD negative complete remission.

[Complete remission of necrobiotic xanthogranuloma after disappearance of monoclonal immunoglobulin induced by bortezomib, lenalidomid and dexamethasone].

Necrobiotic xanthogranuloma (NXG) is a rare chronic condition, belonging to the group non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly monoclonal gammopathy, MGUS and multiple myeloma. The case reported here NXG was diagnosed after 1 years of evolution in patient with asymptomatic multiple myeloma. After treatment with bortezomib, lenalidomid and dexamethasone, there was evident abrupt decrease of monoclonal immunoglobulin to not measurable level (complete remission of multiple myeloma) and in the same time was evident disappearance of cutaneous and hepatic lesions of NXG on FDG-PET/CT.

[Unicentric Castlemans disease. Symptoms, diagnostics and therapy].

Castleman disease (CD) includes a group of rare and heterogeneous disorders with characteristic lymph node histopathological abnormalities. CD can occur in a single lymph node station, which is referred to as unicentric CD (UCD). CD can also involve multicentric lymphadenopathy and inflammatory symptoms - multicentric Castleman disease.

Bortezomib and Thalidomide Treatment Results in Newly Diagnosed Transplant-Ineligible Multiple Myeloma Patients are Comparable in Long-Term Follow-Up.

Background: Thalidomide-and bortezomib-containing regimens are widely used for transplant-ineligible newly diagnosed multiple myeloma patients. The aim of this study was to analyse the efficiency of thalidomide-or bortezomib-based regimens in long-term follow-up.

Materials And Methods: From 2008 to 2012, 142 transplant-ineligible newly diagnosed multiple myeloma patients were analysed retrospectively.

Hypoxia enhances antitumor activity of dichloroacetate.

Unlabelled: It is known that glycolysis contributes to the survival of tumor cells by providing them with energetic and plastic substrates. Dichloroacetate (DCA) as inhibitor of kinase pyruvate dehydrogenase shifts balance of energy metabolism of tumor cells from aerobic glycolysis towards oxidative phosphorylation. The aim of the study was to investigate cytostatic/cytotoxic effect of DCA on glioma C6 cells at the conditions of different oxygenation of the cell incubation medium.

Dynamics of changes of antioxidant system indexes during the growth of two Lewis lung carcinoma variants.

Aim: To study an intensity of prooxidant processes and activity of antioxidant enzymes in tumor tissue of two Lewis lung carcinoma variants (LLC and LLC/R9) differing by their proliferative, metastatic, and angiogenic potential (LLC/R9 as compared to LLC is characterized by lower metastatic activity, higher proliferative and angiogenic potential).

Materials And Methods: The in vitro study was carried out using cultured LLC and LLC/R9 cell lines, and in vivo on 50 female С57/BL6 mice. The indexes of prooxidant processes and activity of antioxidant enzymes have been studied using the methods of experimental oncology, optical spectroscopy, fluorescent spectroscopy, electron paramagnetic resonance, statistical analysis.

[Establishment of in vitro culture, plant regeneration and genetic transformation of Camelina sativa].

The results on in vitro culture establishment, plantlet regeneration and rooting of Camelina sativa cultivar sample Peremozhets and cultivar Mirazh are presented. Effective concentrations of sterilizing agents and duration of plant material treatment were estimated. Phytohormone ratio, sucrose concentration in nutrient medium that induce effective formation of C.

[Peptide components of Geolycosa spider venom modulate P2X receptor activity of rat sensory neurons].

Almost each natural venom comprises a considerable combinatorial library of bioactive substances that have been optimized during evolution. Particular attention is devoted currently on a search for new modulators of ion channels from the venoms of arthropods. We have studied the effect of peptidous compounds of the Lycosa spider venom on the activity of P2X receptors in DRG neurons of rats.

[Adaptation of synaptic transmission to high concentrations of homocysteinic acid].

Prolonged incubation with 25 microM homocysteine acid led to initial suppression of the synaptic transmission, which in 6 out of 12 cases was followed by the restoration of the population spike to values 63% from the control. In the rest of experiments, a transient decrease of the population spike amplitude was observed during wash-out of homocysteine acid. Such a pattern suggests that synaptic transmission has special mechanisms for adaptation to increasing concentrations of the excitatory amino acids which are potentially damaging.

[Interneuronal signaling mediated by the trans-synaptic diffusion of neurotransmitters].

A great number of modern neurophysiological studies give evidence about the possibility of the extrasynaptic diffusion of the released neurotransmitters. Such observations challenge the established view on synapses as isolated communication channels between pre- and postsynaptic neurones. In the present review article, we consider the latest experimental data on the interneuronal cross-talk mediated by extrasynaptic diffusion of L-glutamate (L-Glu) and gamma-amino-butyric acid (GABA).

[Detection of latent disorders of visual function during ambulatory care of children].

For the purpose to select tests for diagnosis of latent disturbances of visual functions, dynamic observation was conducted over 88 healthy school children within 5 years (from the 3d to the 8th form). Once a year, the children underwent examination of visual acuity for far and near, the state of binocular vision on a synoptophore and a color test, the volume of relative and absolute accommodation, muscular balance of eyes for far and near, contrast sensibility, the time of perception of the negative successive contrast, refraction. For the period of 5 years, 7 children developed myopia of low degree, 32 showed transitory or lasting reduction of visual acuity due to spasm of accommodation.