Szturz P.,Interni Hematoonkologicka Klinika |
Moulis M.,Ustav Patologie |
Adam Z.,Interni Hematoonkologicka Klinika |
Slaisova R.,Radiologicka Klinika |
And 5 more authors.
Klinicka Onkologie | Year: 2011
Backgrounds: Castleman disease is a rare non-clonal lymphoproliferative disorder with the etiopathogenesis not yet thoroughly clarified. Clinically, either unicentric (localized) or multicentric (generalized) forms are recognized while, histopathologically, hyaline-vascular, plasma-cell and mixed variants of the disease exist. These types vary one from another in their clinical courses and, importantly, in methods of therapeutic management. While the unicentric hyaline-vascular form usually manifests as benign growth of a single lymph node and treatment response to complete surgical excision reaches up to 100%, the multicentric plasmocellular variant is an aggressive disease with generalized symptoms, laboratory abnormalities and the need for systemic therapy. Aim: The paper provides an overview of information on Castleman disease from its clinical and histopathological signs to diagnostic and therapeutic options. It deals with the role of cytokines and HHV-8 virus infection in the disease pathophysiology and is supplied with ample pictorial documentation of radiographic findings including ultrasonography, computed tomography and hybrid imaging by positron emission tomography (PET) in combination with simultaneously taken full-body computed tomography (CT) scans, the so called PET/CT. We also present photographs of histological specimens taken from an HIV and HHV-8 negative patient with the plasmocellular multicentric form. Conclusions: Consequent to its low incidence, Castleman disease is often misdiagnosed or diagnosed with a delay. Therefore, it is always necessary to include this rare condition in differential diagnostics of lymphadenopathy, microcytic anemia as well as B-symptoms (night sweats, fevers and weight loss). In conclusion, we also stress the significance of full-body PET/CT scanning during staging and treatment response evaluation.
Weinberger V.,Gynekologicko Porodnicka Klinika |
Minar L.,Gynekologicko Porodnicka Klinika |
Brancikova D.,Interni Hematoonkologicka Klinika
Ceska Gynekologie | Year: 2012
Breast cancer is the most common cancer of women in the Czech Republic. According to data from the National Cancer Registry (NOR) in 2007, the incidence is 122.7 cases of breast cancer in 100 000 women per year. Czech Republic occupies in the incidence of women brest cancer 26th place in the world and 17th place in Europe. Annualy it has been diagnosed 6500 these tumors, 1700-1900 women die for him in the Czech Republic (data from 2007). The incidence show long-term upward trend (an increase of 32% in 2007 compared to 1995), while mortality has long been stabilized. In this favorable outcome ivolved the introduction of systematic screeningu mammography in women over 45 years of age, diagnosis of early stages of disease, effective adjutant therapy and treatment of metastatic disease. In an international comparison of mortality, the Czech Republic belongs to 71th place in the world and the 27th place in Europe. Malignant neoplasm of breast cancer often affects women of working age, nearly 43% of patients are younger than 60 years. Long-term increasing incidence and stable mortality lead to a further increase in prevalence, which in 2007 reached more than 55000 women living with breast cancer or its history. The various modalities of treatment include surgery, radiotherapy, systemic chemotherapy, hormonal therapy and targeted biological therapy. Fastest-growing issues in breast cancer management is the sentinel nodes and non-invasive breast cancers. This entity was newly assigned and represented by a lobular carcinoma in situ (LCIS) and ductal carcinoma in situ (DCIS). DCIS as noninvasive cancer of the breast is generally 15-20% of breast cancers diagnosed and the number is growing.
Suppression of alternative immunoglobulin pairs correlates with the MGUS risk degree more significantly than suppression of polyclonal immunoglobulin levels - Partial results from HevyLite™ examination in MGUS [Suprese alternativního páru imunoglobulinu koreluje s mírou rizikovosti MGUS výrazněji nežli suprese hladin polyklonálních imunoglobulinů - dílč í výsledky vyšetření HevyLiteTM u MGUS]
Pika T.,III. Interni Klinika Nefrologicka |
Lochman P.,Oddeleni Klinicke Biochemie |
Klincova M.,Laborator Experimentalni Hematologie A Bunecne Imunoterapie |
Maisnar V.,II. Interni Klinika Oddeleni Klinicke Hematologie |
And 5 more authors.
Klinicka Biochemie a Metabolismus | Year: 2012
Introduction: Monoclonal gammopathy of undetermined significance (MGUS) indicates an asymptomatic and potentially malignant state characterised by benign clonal proliferation of plasma cells secerning (discharging) monoclonal immunoglobulin (MIG, M-protein) detectable in serum and/or urine in the absence of malignant lymphocyte proliferation. It has been observed that in some patients, MGUS transforms into one of the malignant forms of monoclonal gammopathy. The wellknown factors determining the risk degree of progression include in particular the quantity, M-protein type and the ratio of immunoglobulin free light chains (FLC) allowing the efficient stratification of MGUS patients. In addition, several authors consider the present suppression of polyclonal immunoglobulin levels to be a potential factor of progression. Aim: The study aimed at conducting a pilot analysis of levels of immunoglobulin heavy/light chains pairs (HLC) in a group of MGUS patients and at comparing the results with the M-protein levels detected by gel electrophoresis. Furthermore, the polyclonal immunoglobulin levels and the levels of HLC alternative pairs were to be compared with a view to verify the degree of immune paresis depending on the MGUS risk degree. Methods: The analysed set comprised 148 serum samples of MGUS patients (102 IgG, 28 IgA, 18 IgM type) who were stratified into 4 risk groups (low, low-intermediate, high-intermediate, and high risk of transformation) according to the levels, M-protein type and FLC index values. FLC levels, polyclonal immunoglobulin levels, and HLC levels were determined by means of the SPA Plus turbidimeter platform. In the statistical analysis, Spearmans rank correlation and Mann-Whitney U Test with Bonferroni correction were applied. Results: When comparing the HLC levels, the IgG MGUS group revealed strong correlation with M-protein levels both in the case of IgGκ (r = 0.698, p = < 0.0001), and in the case of IgGλ (r = 0.847, p = < 0.0001), the summation of both pairs of the IgG isotype correlated considerably with the total levels of IgG in serum (r = 0.893, p = < 0.0001). Similar results were acquired in MGUS with the IgA isotype of MIG. In case of the IgAκ isotype pair, medium-strong correlation with M-protein levels was detected (r = 0.601, p = 0.018), and strong correlation in case of the IgAλ (r = 0.733, p = 0.004), whereas the summation of both HLC pairs showed very strong correlation with levels of the total IgA (r = 0.941, p = < 0.0001). Moreover, analyses of IgM type patients revealed very strong correlation of IgMκ levels (r = 0.904, p = 0.001) or IgMλ (r = 0.783, p = 0.013) and M-protein levels. Again, the strongest correlation was found between the summation of both isotype pairs and the total levels of IgM immunoglobulin in serum (r = 0.967, p = < 0.0001). Comparison of the suppression degree of polyclonal immunoglobulin levels in individual risk classes in the IgG MGUS group brought significantly higher levels of IgA immunoglobulin in patients with low or low-intermediate risk as opposed to the group with high-intermediate risk (p = 0.0001 and p = 0.047 respectively). When analysing the MGUS group with IgA isotype, notably higher levels of IgM immunoglobulin were evident in the group with low-intermediate risk than in patients with high-intermediate or high risk (p = 0.035 and p = 0.017 respectively). In addition, the levels of alternative isotype pairs were compared among the individual risk groups of the IgG MGUS group. Patients with dominant IgGκ secretion showed significantly higher levels of the IgGλ alternative pair in the group of low and low-intermediate risk than in the group of high-intermediate risk (p = 0.003 and p = 0.006 respectively). In case of dominant IgGλ secretion, considerably higher IgGκ levels were detected in patients with low or low-intermediate risk than in the group of high-intermediate risk (p = 0.0006 and p = 0.009 respectively). Conclusion: The analysis confirmed a very close correlation between M-protein levels and HLC determination which appears to be an auxiliary or alternative method of M-protein determination with an advantage in case of atypical electrophoresis results (in particular in IgA isotypes) or by very low M-protein concentrations with the benefit of Igκ/Igλ index calculation. The discovered connection between the degree of immune suppression depending on the MGUS risk level and especially the clearly visible benefit of determining the alternative HLC pairs contributes with another aspect to understanding the links between the biology, behaviour, and the potential malignant evolution of MGUS with the advantage of obtaining a well-measurable parameter. The use of the SPA Plus platform seems to be fast, effective, with high prognostic value and, last but not least, even with favourable economic aspects.
Kala Z.,Chirurgicka klinika |
Prochazka V.,Chirurgicka klinika |
Cervinek L.,Interni hematoonkologicka klinika
Endoskopie | Year: 2012
Laparoscopic splenectomy is a safe procedure for various pathological conditions of the spleen and of haematopoiesis. Contraindications to laparoscopic approach depend, in part, on the centre's experience. Absolute contraindications are poor internal condition, portal hypertension, and massive splenomegaly. The most frequent indication is idiopathic thrombocytopenic purpura. Treatment of thrombocytopenia always begins with conservative first-line approaches: administration of corticosteroids and intravenous immunoglobulins. Splenectomy thus remains second-line treatment in a small proportion of patients. Postoperative complications are uncommon. In addition to the cosmetic effect, there is a considerably lower risk of large incisional hernia than in laparotomic approach in corticosteroid-dependent patients. Following the procedure, it is always necessary to prevent thromboembolic disease by administering low-molecular-weight heparin.
Szturz P.,Interni Hematoonkologicka Klinika |
Adam Z.,Interni Hematoonkologicka Klinika |
Sediva A.,Ustav Imunologie |
Fojtik Z.,Interni Hematoonkologicka Klinika |
And 5 more authors.
Klinicka Onkologie | Year: 2011
Backgrounds: The most important diagnostic criteria for Schnitzler syndrome include chronic urticaria, the presence of monoclonal IgM immunoglobulin, marked inflammation (leukocytosis, elevated CRP and erythrocyte sedimentation rate), subfebrile temperatures or fevers and bone and joint pains. It is a rare idiopathic disease that may lead to potentially life-threatening complications such as development of secondary amyloidosis or transformation into malignant lymphoproliferation. Schnitzler syndrome should be included in differential diagnostics of chronic urticaria and fevers of unknown origin. The diagnostic algorithm is based on clinical presentation and serum and urine electrophoreses to detect monoclonal components. Blockade of interleukin-1 (IL-1), key cytokine in the pathogenesis of the disease, dominates current therapeutic protocols. Anakinra (Kineret™), recombinant human IL-1 receptor antagonist, is the most widely used treatment option. According to literature, disease remission was obtained in all treated patients. Therefore, anakinra represents a significant diagnostic possibility to differentiate Schnitz-ler syndrome from e. g. monoclonal gammopathy of unknown significance (MGUS) associated with urticaria of different aetiology. Biological therapy with rilonacept (Arcalyst™) and canakinumab (Ilaris™) represents a new treatment alternative for patients, allowing prolonged dosing intervals of 1 and 8 weeks, respectively (compared to 24 hours with anakinra). The review article also presents findings of various imaging methods (conventional radiography, computed tomography, traditional bone scintigraphy) and photographs of patients with Schnitzler syndrome before and after anakinra therapy. Design: The aim of the review is to draw attention to the existence of this rare autoinflammatory and potentially pre-malignant condition, present a simple diagnostic algorithm and provide an overview of therapeutic options for the patients. Conclusions: Malign potential of Schnitzler syndrome, possible development into systemic amyloidosis and the fact that patients are frequently referred to oncology clinics for differential diagnostics of monoclonal gammopathy, are the main reasons why clinical oncologists should be aware of Schnitzler syndrome.
Folber F.,Interni hematoonkologicka klinika |
Letalova E.,Interni hematoonkologicka klinika |
Doubek M.,Interni hematoonkologicka klinika
Onkologie | Year: 2011
Philadelphia chromosome positive adult acute lymphoblastic leukemia with BCR/ABL fusion gene is an aggressive hematological malignancy most common in the elderly. Both chemotherapy and a tyrosine kinase inhibitor are engaged in the treatment. The best outcome can eventually be achieved using allogeneic hematopoietic stem cell transplantation. However, only a minority of patients can undergo such intensive therapy. Disease relapse is associated with an extremely dismal prognosis.
Results of nutritional risk screening in patients with lime cancer predict tumor response to therapy [VÝsledek screeningu nutričního rizika u pacientů s karcinomem plic predikuje odpověd' nádoru na léčbu]
Illa P.,Klinika Nemoci Plicnich a TBC |
Tomiskova M.,Klinika Nemoci Plicnich a TBC |
Skrickova J.,Klinika Nemoci Plicnich a TBC |
Tomiska M.,Interni Hematoonkologicka Klinika
Studia Pneumologica et Phthiseologica | Year: 2013
Background: Malnutrition in cancer patients may be associated with poor tolerance of chemotherapy and lower response rate after oncological treatment. Methods: Nutritional Risk Screening NRS 2002 adapted for oncological patients was used to assess the risk of undernutrition in a group of 188 lung cancer patients. The risk was evaluated on a 6-point scale according to common signs of nutritional status and tumor and its treatment risk factors. A score of 3 and more (called "nutritional risk") means a significant risk of malnutrition and poor treatment outcome. Results: There were 133 (70.7%) men and 55 (29.3%) women with a mean age of 64.9 years. Tumor stages from I to IV were classified in 10.6% (I), 4.3% (II), 9.6% (IIIA), 25.0% (IIIB) and 48.6% (IV) patients. Non-small cell lung cancer was diagnosed in 66.0% and small cell lung cancer in 27.7% of patients. Acceptable NRS score of 0-2 points was found in 50.6%, while in 45.3% the 3-5 score suggested the risk of malnutrition ("nutritional risk"). Unexpectedly, the toxicity of anticancer treatment was not significantly different between the subgroups ("acceptable score" vs. "nutritional risk") with a mean toxicity on the 0-4 point scale of 1.6 (SD 1-3; 95% Cl 1.3-2.0) in patients with higher NRS scores as compared to 1.2 (SD 1.2; 95% Cl 1.0-1.5) in the lower NRS score group (p = 0.061). The rate of treatment response (RR) evaluated by imaging techniques was significantly higher in patients with the "acceptable score" (1-2 points) compared to those with a "nutritional risk" (3-5 points) (57% vs. 30%; p = 0.001). The overall survival rate was significantly higher in lung cancer patients treated with cytostatics and the "acceptable score" as compared to patients at a "nutritional risk" (13.5 months vs. 7.9 months; p = 0.001). Conclusion: Nutritional risk screening is a significant predictor of tumor response in lung cancer patients. Early detection of malnutrition is important to determine the prognosis of cancer patients as well as to plan effective supportive care.
Panovska A.,Interni hematoonkologicka klinika |
Doubek M.,Interni hematoonkologicka klinika
Onkologie (Switzerland) | Year: 2013
Chronic lymphocytic leukemia (CLL) is a low-grade lymphoma, typically found in eldery people. CLL is the most common leukemia in the Western World, it makes up almost 30% of all leukemias. Pathogenesis of CLL is still unknown, however, an antigen stimulation, apoptotic defects and the role of microenvironment are often discussed. At present, new prognostic factors are being analyzed in order to define a risk subgroup of patients that would benefit from early and intensive start of therapy. Treatment of CLL has made a great progress in the last decade. While a few years ago chlorambucil was a golden standard in the CLL therapy, currently, the combination of a chemotherapy backbone with the monoclonal antibody has opened up new horizons for CLL therapy. Patients treated with chemoimmunotherapy can achieve years of complete remission of CLL. Allogeneic stem cell transplantation still represents the only curative approach.
Holanek M.,Interni Hematoonkologicka Klinika |
Hajek R.,Interni Hematoonkologicka Klinika
Klinicka Onkologie | Year: 2010
Lenalidomide is a promising new drug in the treatment of patients with multiple myeloma. The analogue of thalidomide was created with the intention of improving the anticancer activity, its immunomodulatory properties as well as reducing the toxicity of the preparation. The mechanism of antitumor action of the preparation appears to be the effect on cells, tumour microenvironment and in particular the regulation of cytokinine production. Performed clinical studies Phase I and II have shown efficacy in patients with relapsed multiple myeloma. Subsequently, large randomized trials demonstrated the benefit of combined therapy in relapsed/refractory multiple myeloma patients with a combination of lenalidomide plus dexamethasone compared with dexamethasone treatment alone. Patients treated with a combination of lenalidomide and dexamethasone had significantly higher therapeutic response and overall survival than patients treated with dexamethasone alone. Profound toxicity seems to be the myelosuppressive effect of lenalidomide. The work gives a summary of the information available on lenalidomide in the treatment of multiple myeloma.
PubMed | Interni hematoonkologicka klinika
Type: Journal Article | Journal: Klinicka onkologie : casopis Ceske a Slovenske onkologicke spolecnosti | Year: 2011
The most important diagnostic criteria for Schnitzler syndrome include chronic urticaria, the presence of monoclonal IgM immunoglobulin, marked inflammation (leukocytosis, elevated CRP and erythrocyte sedimentation rate), subfebrile temperatures or fevers and bone and joint pains. It is a rare idiopathic disease that may lead to potentially life-threatening complications such as development of secondary amyloidosis or transformation into malignant lymphoproliferation. Schnitzler syndrome should be included in differential diagnostics of chronic urticaria and fevers of unknown origin. The diagnostic algorithm is based on clinical presentation and serum and urine electrophoreses to detect monoclonal components. Blockade of interleukin-1 (IL-1), key cytokine in the pathogenesis of the disease, dominates current therapeutic protocols. Anakinra (Kineret), recombinant human IL-1 receptor antagonist, is the most widely used treatment option. According to literature, disease remission was obtained in all treated patients. Therefore, anakinra represents a significant diagnostic possibility to differentiate Schnitzler syndrome from e.g. monoclonal gammopathy of unknown significance (MGUS) associated with urticaria of different aetiology. Biological therapy with rilonacept (Arcalyst) and canakinumab (Ilaris) represents a new treatment alternative for patients, allowing prolonged dosing intervals of 1 and 8 weeks, respectively (compared to 24 hours with anakinra). The review article also presents findings of various imaging methods (conventional radiography, computed tomography, traditional bone scintigraphy) and photographs of patients with Schnitzler syndrome before and after anakinra therapy.The aim of the review is to draw attention to the existence of this rare autoinflammatory and potentially premalignant condition, present a simple diagnostic algorithm and provide an overview of therapeutic options for the patients.Malign potential of Schnitzler syndrome, possible development into systemic amyloidosis and the fact that patients are frequently referred to oncology clinics for differential diagnostics of monoclonal gammopathy, are the main reasons why clinical oncologists should be aware of Schnitzler syndrome.