Stem cell transplantation in chronic lymphocytic leukemia

Despite the significant progress that has been made in recent years in the treatment of chronic lymphocytic leukemia (CLL), mainly due to the introduction of monoclonal antibodies, allogeneic hematopoietic cell transplantation remains the only method that could potentially cure CLL. However, because of high peritransplant mortality, this method is reserved only for patients with high-risk CLL. Autologous transplantation is currently not recommended for patients with CLL due to the lack of advantage over the standard first-line FCR immunochemotherapy and the lack of efficacy in high-risk CLL

Description of invariant NKT cells.

Recently discovered subpopulations belonging to cell groups called “invariant” NKT, also referred to as type I NKT or classical NKT cells, appear to have a unique impact on the tumor microenvironment. Considering the characteristics of secreted cytokines, production of chemokines and the capability of cell cytotoxic activity, these cells have a significant function in being immune to bacteria, parasites, viruses, autoimmune and tumor diseases. The profile of secreted cytokine is probably decisive in reference to the distinct functions of the subpopulation, some of them exhibit immunosuppressive activity inhibiting an anticancer immune response, and the other stimulate the organisms for eradicating the tumor. Currently, it has been discovered six fundamental, functionally distinct subpopulations of iNKT cells (NKT1, NKT2, NKT10, NKT17, NKTreg, NKTFH).Recently discovered subpopulations belonging to cell groups called “invariant” NKT, also referred to as type I NKT or classical NKT cells, appear to have a unique impact on the tumor microenvironment. Considering the characteristics of secreted cytokines, production of chemokines and the capability of cell cytotoxic activity, these cells have a significant function in being immune to bacteria, parasites, viruses, autoimmune and tumor diseases. The profile of secreted cytokine is probably decisive in reference to the distinct functions of the subpopulation, some of them exhibit immunosuppressive activity inhibiting an anticancer immune response, and the other stimulate the organisms for eradicating the tumor. Currently, it has been discovered six fundamental, functionally distinct subpopulations of iNKT cells (NKT1, NKT2, NKT10, NKT17, NKTreg, NKTFH)

Skuteczność ruksolitynibu w leczeniu chorych na mielofibrozę z chorobami współistniejącymi

Myelofibrosis (MF) is a heterogenous Philadelphia-myeloproliferative neoplasm that is characterized by bone marrow fibrosis and impaired hematopoiesis. Clinical hallmarks of MF are increased splenomegaly, cytopenias and general symptoms. Deregulation of JAK–STAT signaling pathway plays a key role in the pathogenesis of MF. Ruxolitinib is a selective and oral JAK1/JAK2 inhibitor that in clinical trials demonstrated significant efficacy in the reduction of clinical symptoms, improved the quality of life and increased overall survival of patients with MF.Mielofibroza (MF) jest heterogennym nowotworem układu krwiotwórczego, cechującym się brakiem chromosomu Filadelfia i włóknieniem szpiku oraz wynikającym z tego upośledzeniem hematopoezy. Objawami klinicznymi MF są powiększenie śledziony, cytopenie oraz objawy ogólne. W patogenezie MF szczególną rolę odgrywają zaburzenia szlaku JAK–STAT. Ruksolitynib jest selektywnym, doustnym inhibitorem JAK1/JAK2, który w badaniach klinicznych wykazał istotną skuteczność w zmniejszeniu objawów klinicznych, poprawie jakości życia oraz wydłużeniu całkowitego przeżycia chorych na MF

Assessment of Red Blood Cell Distribution Width as a Prognostic Marker in Chronic Lymphocytic Leukemia

Red blood cell distribution width (RDW) is a quantitative measure of the variability in size of circulating erythrocytes. It was recently reported that RDW is a prognostic factor for infection diseases, cardiovascular and pulmonary diseases, as well as some neoplasms. Moreover, RDW is remarkably strong predictor of longevity, including all causes of death, for adults aged 45 years and older. To explain this occurrence it was proposed that persistent IGFs/mTOR signaling is one of the factors that play a role in affecting the RDW and mortality.The above observations induced us to analyze the prognostic role of RDW in chronic lymphocytic leukemia (CLL) being the most frequent type of adult leukemia in Western countries. The obtained results have shown that RDW may be considered as a potential CLL prognostic marker. Elevated RDW level at the moment of diagnosis was associated with advanced disease and presence of other poor prognostic factors. It is also connected with overall survival indicating shorter time in patients with elevated RDW. It is possible that the presently observed correlation between mortality and RDW of the CLL patients is affected by their metabolic (IGF-1/mTOR driven)- rather than chronological- aging. The patients with high level of RDW are expected to have an increased persistent level of IGF-1/mTOR signaling. Within the framework of personalized therapy, these CLL patients therefore would be expected to be more sensitive to the treatment with mTOR inhibitors

Chest pain in the course of multiple myeloma - a clinical case study

Introduction: Multiple myeloma (MM) is a rare blood cell proliferative disease characterized by the accumulation and proliferation of monoclonal plasmocytes. Clinical picture of MM includes bone pain, underlying osteolytic lesions, osteopenia or osteoporosis that often lead to pathological fractures. Aim: To draw attention to the unusual cause of chest pain and the holistic approach to analgesic therapy in patients with MM. Case report: A clinical case of a 66-year-old patient with chest pain intensified when moving and deep breathing was presented and cardiological and gastroenterological reasons were excluded. Initially, non-steroidal analgesics and weak opioids were used in the treatment with good effect, however, as time was passing the pain symptoms progressed. Diagnostic imaging was complemented by computed tomography which revealed massive destructive changes within the ribs with the presence of soft tissue masses infiltrating adjacent muscles. Based on additional tests, the patient was diagnosed with MM. Optimization of analgesic therapy has brought permanent pain relief and improved his quality of life. Summary: The modern approach to anelgesia in patients with MM includes not only the use of analgesics, but also radiotherapy, bisphosphonates/zoledronic acid, orthopaedic treatment and chemotherapy

Intraventricular treatment of secondary central nervous system lymphoma – Case study and literature overview

Secondary nervous system lymphoma (SCNSL) is a rare extranodal form of non-Hodgkin lymphoma (NHL). This applies to a particular form of lymphoma that does not originally derive from the central nervous system (CNS); it can be both an isolated form of relapse or a systemic part of disease progression. Due to poor prognosis and a lack of established algorithms of therapeutic procedures, it is a big challenge for physicians from many specializations. In our study, we present an interesting case of a patient with a relapsed form of SCNSL for whom a unique form of treatment was used – intraventricular administration of rituximab and methotrexate

Skuteczne leczenie ibrutynibem chorego na przewlekłą białaczką limfocytową z obecnością delecji 17p – opis przypadku

W pracy przedstawiono przypadek 62-letniego chorego na PBL z obecnością delecji 17p, u którego zastosowanie immunochemioterapii nie przyniosło oczekiwanego efektu. Dopiero leczenie ibrutynibem doprowadziło do remisji częściowej choroby (zmniejszenia limfadenopatii, normalizacji obrazu morfologii krwi)

Central nervous system involvement during clinical course of chronic lymphocytic leukaemia

Central nervous system involvement (CNS) of chronic lymphocytic leukemia (CLL) is a rare complication. Some analyses of autopsy studies suggest that the complication remains subclinical or is under-diagnosed. The symptoms of patients with leukemic CNS involvement are heterogeneous and nonspecific. Due to the low incidence, there are no treatment guidelines for this problem. Intrathecal chemotherapy and cranial irradiation are often performed

Central nervous involvement by chronic lymphocytic leukaemia

Inclusion of the central nervous system (CNS) in the course of chronic lymphocytic leukaemia (CLL) is rare. At the moment no risk factors or proven treatment methods are known. The disease is described both in its early phase and during its acceleration period, thus it has been suggested that there might be independent mechanisms influencing the development of this condition. As there are no unified diagnostic procedure algorithms each patient needs to be assessed individually. CLL can manifest mostly in elderly people, for whom a possibility of development of neurological disorders with their aetiology different from leukaemia, should also be taken into consideration. The thesis presents a group of seven patients with CLL with CNS infiltration. Patients with prolymphocytic leukaemia, Richter's transformation and the original location of leukemic infiltration within the eye socket constitute an especially interesting case

Haemophagocytic lymphohistiocytosis: case series. Serum ferritin level as an indicator of treatment effectiveness

Introduction: Haemophagocytic lymphohistiocytosis (HLH) is a medical condition associated with over-activation of the immune system. HLH results from the inactivity of NK cells and uncontrolled activity of cytotoxic T lymphocytes and macrophages with the massive cytokine response.[1] Cytohistological examinations might show hemophagocytes in different tissues (e.g., bone marrow). Exceptionally high level of ferritin is often observed in the laboratory findings. Due to aggressive course of the disease mortality rate is extremely high. Material and methods: We presented three patients with acquired form of HLH treated successfully in Heaematooncology Department of Medical University of Lublin from September 2018 to April 2021. Results: In the first patient, HLH developed during pregnancy. Patient 2 was first hospitalized in the Intensive Care Unit (ICU) and 10 therapeutic plasma exchanges (TPE) were carried out. In both patients, stabilization -of ferritin levels and remission of the disease were achieved soon after the application of the treatment according to the HLH-2004 protocol. Case 3 presents a patient in whom HLH was induced by EBV and SARS CoV-2 infections. In the first stage of the treatment, the patient received drugs according to the HLH-2004 protocol, but the serum ferritin did not normalize. The reinfection with SARS CoV-2 virus occurred again in the course of the treatment. Due to the disease resistance and inability to continue the HLH-2004 protocol, it was decided to start ruxolitinib therapy, which resulted in stabilization of the serum ferritin and improvement of the general condition. The patient was qualified for allogeneic bone marrow transplantation. Conclusions: HLH is a difficult and interdisciplinary diagnostic and therapeutic problem. It is essential to popularize knowledge about fast and targeted diagnostics. Among laboratory finding, the ferritin concentration seemed to be particularly helpful as a predictor of treatment effectiveness. Proper diagnosis and treatment implemented as early as possible could save patients’ lives.Introduction: Haemophagocytic lymphohistiocytosis (HLH) is a medical condition associated with the over-activation of the immune system. HLH results from the inactivity of natural killer cells and uncontrolled activity of cytotoxic T lymphocytes and macrophages, with a massive cytokine response. Cytohistological examinations might show haemophagocytes in different tissues (e.g., bone marrow). Among laboratory findings especially(particularly) high ferritin level is often seen. Due to the aggressive course of the disease mortality rate is extremely high. Case reports: The study presented three patients with an acquired form of HLH treated successfully in the Haemato-Oncology Department Medical University of Lublin from September 2018 to April 2021. In case 1 HLH developed during pregnancy. Patient 2 was first hospitalized in the Intensive Care Unit and 10 therapeutic plasma exchanges were carried out. In both patients, stabilization of ferritin levels and remission of the disease were achieved soon after the application of treatment according to the HLH-2004 protocol. Case 3 presents a patient in whom HLH was induced by Epstein-Bárr virus and severe acute respiratory syndrome coronavirus type SARS-CoV-2 infections. In the first stage of treatment, the patient received drugs according to the HLH-2004 protocol, but the serum ferritin did not normalize. The reinfection with (SARS-CoV-2) virus occurred again during the treatment. Due to the disease resistance and the inability to continue the HLH-2004 protocol, it was decided to start ruxolitinib therapy, which resulted in the stabilization of the serum ferritin and improvement of the general condition. The patient was qualified for allogeneic bone marrow transplantation. Conclusions: HLH is a difficult and interdisciplinary diagnostic and treatment problem. It is necessary to popularize knowledge about fast and targeted diagnostics. Among laboratory finding the ferritin concentration seemed to be especially helpful as a predictor of treatment effectiveness. Proper diagnosis and treatment introduced as early as possible could save patients’ life