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

Zespół hemofagocytowy indukowany terapią hormonalną – studium przypadku klinicznego

Zespół hemofagocytowy (ang. – HLH) charakteryzuje się nieprawidłową aktywacją układu immunologicznego, u podłoża której leżą zmiany genetyczne lub nabyte zaburzenia cytotoksyczności limfocytów T i NK. Objawy kliniczne są niespecyficzne i różnorodne, a postawienie rozpoznania, pomimo dostępności badań dodatkowych, jest niezwykle trudne. W artykule zaprezentowano przypadek kliniczny chorej, u której pierwotnie w obrazie klinicznym dominowała nawracająca gorączka oraz zmiany skórne sugerujące rozpoznanie rumienia guzowatego. Pomimo rozpoczęcia steroidoterapii oraz stosowania empirycznej antybiotykoterapii stan pacjentki nie ulegał poprawie. Do objawów klinicznych dołączyły się splenomegalia oraz zaburzenia w badaniach dodatkowych: trójukładowa cytopenia, hiperferrytynemia, hipertriglicerydemia, hipofibrynogenemia. Ponadto w obrazie histopatologicznym szpiku kostnego stwierdzono obecność hemofagocytów. Na podstawie obrazu klinicznego oraz badań dodatkowych postawiono rozpoznanie HLH. W terapii zastosowano chemioterapię zgodnie z protokołem HLH-2004, uzyskując całkowitą remisję

Expression of CD1d molecules on B cells in patients with chronic lymphocytic leukemia

Chronic lymphocytic leukemia (CLL) which is the most common leukemia in adults in Western countries still remains incurable. There is an intense search for the prognostic markers that might facilitate the treatment of patients according to individual prognosis. The aim of the presented study was to evaluate the expression of CD1d molecule on peripheral blood B cells from 70 untreated patients with CLL and 20 healthy donors. The samples were analyzed by flow cytometry directly after preparation.The results of the study showed that the median percentage of CD1d-positive B cells was significantly lower in peripheral blood of patients with CLL than in healthy subjects from control group. Additionally, the percentage of CD1d+ B cells in CLL patients varied in patients with different Rai stages. We have also observed significant differences in CD1d expression depending on CD38 or ZAP70 expression. Moreover, patients with CLL had lower percentages of iNKT cells than healthy donors and the percentage of CD1d+/CD19+ cells inversely correlated with the percentage of iNKT cells. Our results suggest the important role of CD1d molecule in the development and progression of CLL, as well as its potential prognostic significance

Ekspresja cytokin wewnątrzkomórkowych w limfocytach T u chorych na przewlekłą białaczkę limfocytową

Functional disorders of T lymphocytes play an essential role in abnormal immune response in patients with chronic lymphocytic leukemia. The aim of this study was to assess the profile of cytokines expressed by T cells derived from patients with CLL. We have demonstrated that the intracellular levels of IL-2, IL-4, IFN-γ, TNF, IL-6 and IL-10 were significantly higher in T cells of CLL patients than in healthy donors. Moreover, the percentages of CD4+/CD3+/TNF+, CD4+/CD3+/IFN-γ+, and CD4+/CD3+/IL-2+ cells were significantly higher in ZAP-70-positive patients compared with ZAP-70-negative ones. Likewise, significantly higher percentages of CD4+/CD3+/TNF+, CD4+/CD3+/IFN-γ+ cells were observed in CD38-positive than in CD38-negative cases. What is more, there was a significant difference in median percentage of CD3+/CD4+ cells expressing TNF, IL-4, IFN-γ, IL-2 or IL-6 between patients carrying the 11q22.3 deletion and/or the 17p13.1 deletion and patients without these genetic aberrations. Our results confirm the functional disorders of T cells in CLL and their influence on the clinical course of the disease

Tie-2 expressing monocytes in chronic lymphocytic leukemia

IntroductionIn peripheral blood, monocytes form a heterogeneous population of cells. One particular subset of circulating monocytes is expressing the angiopoietin receptor Tie-2 (Tie2-expressing monocyte; TEM). TEM are characterized by tumor promoting properties. However, the role of TEM in chronic lymphocytic leukemia (CLL) immunopathogenesis remains undefined.Material and MethodsHere, we evaluated the monocytes with Tie-2 expression (CD14+Tie-2+) in peripheral blood of CLL patients (n=55) and normal subjects (n=15) by flow cytometry. We investigated possible associations between TEM and poor prognostic factors such as CD38 or ZAP-70 expression, Rai stage and unfavorable cytogenetic abnormalities. Moreover, we investigated the association of TEM percentage with CD14++CD16+ monocytes and Treg percentages.ResultsWe found that CLL patients had a higher percentage of CD14+Tie-2+ monocytes compared to normal controls. The percentage of TEM was positively associated with ZAP-70 expression as well as with unfavourable cytogenetic changes: del(17p) and/or del(11q). The frequency of TEM increased with the disease stage. We showed no correlation between the percentage of TEM and CD38 expression. The percentage of TEM at diagnosis was associated with white blood cell count as well as with the percentages of CD19+CD5+ lymphocytes and Tregs. The majority of CD14+Tie-2+ cells belonged to the intermediate monocytes subset (CD14++CD16+) while fewer of them were among the classical (CD14++CD16−) or non-classical monocyte (CD14+CD16++) subsets. TEM and CD14++CD16+ monocytes have a proangiogenic activity, suppress T-cell activation and promote Treg expansion. The results suggest that monitoring of TEM number and function may provide useful information in determining disease activity

Evaluation of the prognostic and predictive value of free light chains in patients with chronic lymphocytic leukemia – preliminary results

Introductionκ and λ serum free light chains (sFLCs) are produced during physiological lymphopoesis by plasmocytes and B lymphocytes in a constant ratio related to heavy chains. The measurement of sFLC plays an important role in the diagnosis and monitoring of patients with multiple myeloma (MM). The first reports suggested that sFLC disturbances might have prognostic value also in patients with chronic lymphocytic leukemia (CLL). Aim of the study: The aim of the study was to evaluate the relationship between sFLC concentration and recognized prognostic factors and clinical course of CLL. Materials and methods: The sFLC concentration was measured using a latex-enhanced immunoassay in 59 patients with newly diagnosed CLL. The relationship between sFLC concentration and time to start of the treatment (TFT), the response rate to therapy (ORR) and overall survival (OS) was assessed. ResultsA significant correlation was found between sFLC κ concentration and the clinical stage of leukemia according to Rai classification, β-2 microglobulin concentration, LDH activity, CD38 expression, as well as between sFLC λlevel and β-2 microglobulin concentration and platelet count (PLT ). There was also a correlation between the values of summated κ and λ and the clinical stage of disease according to Rai classification, β-2 microglobulin concentration, CD38 expression, white blood cells count (WBC), lymphocyte count (ALC) and hemoglobin (Hgb) concentration. The κ/λ ratio (FCLR) values were significantly different in the CD38+ and CD38- population. SummarySimple and reproducible clonality index, which constitutes the sFLC concentration assessment, can be an attractive, potential prognostic marker in patients with CLL, however further studies are needed on a larger group of patients especially in relation to the predictive value of sFLC

Analiza apoptozy ex vivo komórek B i T z krwi obwodowej i szpiku kostnego chorych na przewlekłą białaczkę limfocytową

In this study we analyzed selected parameters of apoptosis in leukemic cells from peripheral blood and bone marrow of patients with chronic lymphocytic leukemia (CLL). The percentage of apoptotic leukemic B cells (Δψmlow/CD19+) was significantly lower in peripheral blood (median: 0.99%) than in bone marrow (median: 1.41%) (pW badaniach przeprowadzono analizę wybranych parametrów apoptozy komórek białaczkowych krwi obwodowej i szpiku chorych na przewlekłą białaczkę limfocytową (PBL). Odsetek białaczkowych limfocytów B ulegających spontanicznej apoptozie ex vivo (Δψmlow/CD19+) był większy w szpiku (mediana: 1.41%) w porównaniu z krwią obwodową (mediana: 0.99%) (

Early and late follow-up of patients with Hodgkin’s lymphoma. Recommendations of the Polish Lymphoma Research Group

Optymalny sposób monitorowania chorych na chłoniaka Hodgkina (HL) po zakończeniu leczenia przeciwnowotworowego nie jest do końca ustalony i opiera się w głównej mierze na praktyce klinicznej. Przez pierwsze lata obserwacji największy nacisk kładzie się na wykrycie ewentualnej wznowy, następnie większe znaczenie ma monitorowanie późnych powikłań terapii. W artykule przedstawiono dostępne zalecenia oraz rekomendacje monitorowania chorych po leczeniu chłoniaka Hodgkina przygotowane przez Polską Grupę Badawczą Chłoniaków.Post-treatment follow-up of patients with Hodgkin’s lymphoma has not yet been fully optimised and is still basedmainly on clinical practice and experience. During the first years of follow-up, the principal aims are to detectrelapse and monitor any post-treatment complications or side effects. Such as they are, current guidelines onfollow-up are herein considered and discussed, together with those now recommended by the Polish LymphomaResearch Group

TP53 polymorphism in plasma cell myeloma

Introduction. Significant and accessible predictive factors for bortezomib treatment in plasma cell myeloma (PCM) are still lacking. TP53 codon 72 polymorphism (P72R) results in proline (P) or arginine (R) at 72 amino acid position, which causes synthesis of proteins with distinct functions. The aims of our study were to: 1) analyze whether this polymorphism is associated with an increased risk of PCM; 2) study whether the P72R polymorphism affects overall survival (OS) among PCM patients; 3) assess the possible association of the P72R polymorphism with sensitivity to bortezomib in cell cultures derived from PCM patients. Material and methods. Genomic DNA from newly diagnosed 59 patients (without IgVH gene rearrangements and TP53 deletions) and 50 healthy blood donors were analyzed by RFLP-PCR to identify TP53 polymorphism. Chromosomal aberrations were detected by use of cIg-FISH. The lymphocyte cell cultures from a subgroup of 40 PCM patients were treated with bortezomib (1, 2 and 4 nM). Results. The P allele of the P72R polymorphism was more common than the R allele in PMC patients compared to controls (39% vs. 24%), and the difference was significant (p = 0.02). The PP and PR genotypes (in combina­tion) were more frequent among cases than in controls (65% vs. 42%, OR = 2.32, p = 0.04). At the cell culture level and 2 nM bortezomib concentration the PP genotype was associated with higher necrosis rates (10.5%) compared to the PR genotype (5.7%, p = 0.006) or the RR genotype (6.3%, p = 0.02); however, no effect of genotypes was observed at bortezomib concentrations of 1 and 4 nM. The shortest OS (12 months) was observed in patients with the PP genotype compared to patients with the PR or RR genotypes (20 months) (p = 0.04). Conclusions. The results suggest that P72R polymorphisms may be associated with an increased PCM risk and may affect OS of PCM patients. However, we saw no consistent results of the polymorphism effect on apoptosis and necrosis in cell cultures derived from PCM patients. Further studies are need in this regard