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1.
Ann Hematol ; 101(4): 789-798, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35061088

RESUMO

Mature T cell lymphomas (MTCLs) have worse prognosis, and in contrast to B cell lymphomas, there is no universal marker like CD20 with exception of ALK and CD30, which are present in proportion of MTCL only. Up to now, ALK is traditionally associated with good prognosis in ALCLs, and there are some evidences that CD30-positive T cell or B cell lymphomas have better prognosis. In our retrospective, population-based analysis, we analyzed the real clinical value of ALK and CD30 in the most frequent MTCL subtypes. Between 2000 and 2017, we identified 732 patients with newly diagnosed ALCL, AITL, or PTCL-NOS. Among them, 207 ALCL patients were with known ALK, whereas 61 AITL and 238 PTCL-NOS with known CD30 expression. There were 69/207 (33.3%) ALK + ALCLs, who displayed better 5-year PFS (65.6% vs. 36.2%) (p .001) and 5-year OS (71.5% vs. 45.9%) (p .002) compared to ALK - ; ALK + patients were significantly younger (median 48 vs. 60 years; p < 0.001). For patients ≥ 60 years, 5-year PFS (38.5% vs. 31.2%) and 5-year OS (38.5% vs. 39.6%) were similar between ALK + vs. ALK - patients. For AITL and PTCL-NOS, there were 44/61 (72.1%) and 120/238 (50.4%) CD30 + samples, and difference in CD30 expression was significant (p .02). AITL patients had 5-year OS of 43.8% vs. 55.7% (p 0.848) and 5-year PFS of 36.7% vs. 29.4% (p .624) for CD30 + vs. CD30 - patients, whereas PTCL-NOS had 5-year OS of 35.7% vs. 34.3% (p .318) and 5-year PFS of 29.3% vs. 22.5% (p.114) for CD30 + vs. CD30 - cases. We conclude that ALK in ALCLs (≥ 60 years) and CD30 expression in PCTL-NOS and AITL have only limited prognostic value.


Assuntos
Antígeno Ki-1 , Linfoma de Células T Periférico , República Tcheca , Humanos , Linfoma de Células T Periférico/patologia , Prognóstico , Receptores Proteína Tirosina Quinases , Estudos Retrospectivos
2.
Rozhl Chir ; 97(3): 133-138, 2018.
Artigo em Tcheco | MEDLINE | ID: mdl-29589457

RESUMO

Hirschsprungs disease (HD) in adults is extremely rare, only three publications in Czech and Czechoslovak journals making reference to the condition after childhood. We present two cases of adult patients with HD. The first case is a 46-year-old male patient suffering from chronic constipation since childhood and diagnosed with megacolon at the age of 16; however, no further detailed diagnosis was done. At the age of 41, he developed a sigmoid perforation due to fecaloma and underwent urgent rectosigmoid resection and colostomy. 5 months later, Swensons coloanal anastomosis with diverting ileostomy was performed. Postoperative course was uneventful. He has two bowel movements a day. 7 years after the Swensons procedure, he also underwent adhesiolysis for acute bowel obstruction. His daughter was operated on due to HD at 16 days of age. The second case is a 57-year-old male patient. He suffered from chronic constipation and megacolon since 2 years of age and was diagnosed with congenital megacolon at the age of 19. However, no detailed diagnostics followed. He had a long interval between stools of up to 14 days. He underwent colonoscopy and, with a diagnosis of resistant Crohns disease, was referred to a surgical department where he was diagnosed with HD. Left hemicolectomy was first performed, followed by Swensons procedure with diverting ileostomy. All postoperative courses were uneventful. Currently he passes one or two soft stools a day. Adult HD is extremely rare. However, adult surgeons should consider it in case of refractory constipation since childhood associated with megacolon. Diagnosis is based on contrast radiography and rectal biopsy. Both Swensons and Duhamels procedures are suitable for surgical management. Left hemicolectomy with colonic rotation and coloanal anastomosis and/or proctocolectomy with J-pouch anastomosis are indicated in advanced forms of non-functional megacolon.Key words: adult Hirschsprungs disease - megacolon surgical therapy.


Assuntos
Doença de Hirschsprung , Adulto , Canal Anal , Anastomose Cirúrgica , Doença de Hirschsprung/complicações , Doença de Hirschsprung/diagnóstico , Doença de Hirschsprung/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade
3.
Klin Onkol ; 29(4): 295-302, 2016.
Artigo em Tcheco | MEDLINE | ID: mdl-27534788

RESUMO

BACKGROUND: Recent advances in the use of the imaging modalities, especially PET/CT, and their utilization for determining clinical stage (CS) and assessment treatment response (TR) in malignant lymphomas, along with development of prognostic tools and new treatment modalities, formed the basis for the revised criteria for evaluating CS and TR (published as the Lugano classification, 2014). MATERIALS AND METHODS: The authors summarize the new Lugano recommendations (published in 2014) and the changes from the criteria published in 2007. Moreover, discussion of the changes places emphasis on practical use. The practicality of the Lugano classification, 2014 was the subject of consensus meeting at the annual meeting of the Cooperative Lymphoma Study Group (CLSG) in March 2015. This study reports the final consensus. The CLSG recommends use of the Lugano classification, 2014, but recommends some modifications. CONCLUSIONS: Standardization of the criteria used to determine CS and TR in malignant lymphomas has led to improvements in initial staging and assessment of TR. The criteria are helpful for unifying response assessment in clinical trials and simplify the work of regulatory agencies (e.g., the EMA and the Czech State Institute for Drug Control) when registering new drugs. It also allows evaluation of treatment outcomes outside clinical trials, for example within the CLSG prospective registry of patients with newly diagnosed lymphoma. KEY WORDS: malignant lymphoma - computed tomography - positron emission tomography - staging - treatment responseThis work was supported by the grant Prvouk P27/2012 of the Third Faculty of Medicine, Charles University in Prague and by the grant of the Czech Lymphoma Study Group No. NT12193-5/2011.The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.Submitted: 24. 1. 2016Accepted: 16. 2. 2016.


Assuntos
Linfoma/diagnóstico por imagem , Guias de Prática Clínica como Assunto , República Tcheca , Gerenciamento Clínico , Humanos , Linfoma/patologia , Linfoma/terapia , Estadiamento de Neoplasias , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada
4.
Neoplasma ; 60(1): 68-73, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23067219

RESUMO

Diffuse large B-cell lymphoma (DLBCL) consists of at least two biologically and pathogenetically different subtypes, the germinal centre B-cell (GCB) and the activated B cell type (ABC). It has been suggested that immunohistochemistry can discriminate these subtypes as well. The aim of this study was to verify the validity of the most commonly used Hans algorithm in patients with DLBCL treated with anthracycline- based chemotherapy with rituximab. Immunohistochemical staining using standard protocols was performed on formalin fixed paraffin-embedded tissues. CD20, CD5, CD23, BCL2, CD10, BCL6, MUM1 and Ki67 antibodies were applied. Out of 120 examined cases 52 patients were evaluated as GCB type and 68 patients as having non-GCB, out of a set of 99 patients treated with immunochemotherapy 45 patients with GCB and 54 patients with non-GCB DLBCL were identified. In this set of patients, there was no statistically significant difference neither in overall survival (OS) (HR 1.47 95% CI 0.51-2.63; p=0.45) nor in progression free survival (PFS) (HR 1.57, 95 % CI 0.76-3.22; p=0.731) between both groups.


Assuntos
Algoritmos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Centro Germinativo/patologia , Linfoma Difuso de Grandes Células B/mortalidade , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos Monoclonais Murinos/administração & dosagem , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Feminino , Seguimentos , Humanos , Técnicas Imunoenzimáticas , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/patologia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prednisona/administração & dosagem , Prognóstico , Rituximab , Taxa de Sobrevida , Vincristina/administração & dosagem , Adulto Jovem
5.
Cesk Patol ; 48(4): 198-206, 2012 Oct.
Artigo em Tcheco | MEDLINE | ID: mdl-23121029

RESUMO

Histiocytic necrotizing lymphadenitis / Kikuchi-Fujimoto disease (HNL/K-F) is being recognized with an increasing frequency not only in the East Asia but also on the American continents and in the Europe. Still the diagnostics of HNL/K-F is not easy and difficulties with its proper classification persist. In a group of 19 patients diagnosed primarily or as consults at our department there were 12 woman and 7 men. An average age at diagnosis was 28 years, median 25 years. Cervical lymph nodes were involved in 18 patients. Bilateral lymphadenopathy was present in one patient, the remaining 17 were unilateral. Inguinal lymph node was affected in one patient. In one other patient there were enlarged retroperitoneal lymph nodes simultaneously with a cervical lymphadenopathy. The size of the lymph nodes varied between 5 mm to 32 mm. The subclassification showed the necrotizing type in 14 patients, in one there was a predominant xanthomatous tissue reaction around the necrotic areas (xanthomatous type), and in 4 patients the disease was recognized as the proliferative type without necrosis (in two with a variously intense apoptosis of the proliferating lymphocytes). Of 10 consult cases the tumor was primarily evaluated as B cell lymphoma not otherwise specified (1x), peripheral T cell lymphoma (1x), classical Hodgkin lymphoma of mixed cellularity (1x); two patients were submitted with a differential diagnosis between peripheral T cell lymphoma and HNL/K-F; in one diagnosis of probable EBV lymphadenitis and in one diagnosis HNL/K-F was made. There were no data submitted in the remaining three cases. The authors stress diagnostic features which should lead to the diagnosis of the disease and should prevent unnecessary oncological staging investigations and potential chemotherapy for a lymphoma. Among diagnostic features of HNL/K-F identification of the proliferating cells - CD8 activated lymphocytes with apoptotic decay prevail, there are frequent plasmacytoid monocytes and a striking reaction of macrophages which are CD68/myeloperoxidase positive. There are virtually no neutrophil granulocytes and there is a miminal participation of plasma cells. In case of necrotizing and xanthomatous type infectious causes are to be ruled out as well. In case we still need to distinguish HNL/K-F from a lymphoma PCR analysis of a rearrangement of the immunoreceptor gene in T cell population should be investigated.


Assuntos
Linfadenite Histiocítica Necrosante/patologia , Adolescente , Adulto , Diagnóstico Diferencial , Feminino , Linfadenite Histiocítica Necrosante/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem
10.
Cesk Patol ; 47(3): 84-93, 2011 Jul.
Artigo em Tcheco | MEDLINE | ID: mdl-21887923

RESUMO

A histological picture in pretreatment bone marrow trephine biopsy is an essential part of Ph-negative myeloproliferative neoplasm diagnosis according to WHO classification. Polycythaemia vera is histologically defined as a hypercellular trilinear myeloproliferation. Hypercellular haematopoiesis with granulocytic and megakaryocytic proliferation is typical for primary myelofibrosis. In essential thrombocythaemia the haematopoiesis is normocellular with proliferation of megakaryocytes only. The most important differential diagnostic features are morphology and distribution of megakaryocytes, and presence of fibrosis. In primary myelofibrosis there are typically ,dysplastic" megakaryocytes forming tight (dense) clusters, and variable extent of fibrosis, while mature megakaryocytes forming loose clusters and no fibrosis are found in essential thrombocythaemia. In reactive thrombocytosis and erythrocytosis the number of normally appearing megakaryocytes is not increased and they are not forming clusters. Prodromal (latent) phases of myeloproliferative neoplasms often unrecognized by recent WHO classification criteria are discussed as well as a differential diagnosis of myeloproliferative disorders associated with thrombocytosis.


Assuntos
Medula Óssea/patologia , Transtornos Mieloproliferativos/patologia , Biópsia , Diagnóstico Diferencial , Humanos , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/genética , Cromossomo Filadélfia , Policitemia Vera/diagnóstico , Policitemia Vera/patologia , Mielofibrose Primária/diagnóstico , Mielofibrose Primária/patologia , Trombocitemia Essencial/diagnóstico , Trombocitemia Essencial/patologia
11.
Vnitr Lek ; 57(2): 189-213, 2011 Feb.
Artigo em Tcheco | MEDLINE | ID: mdl-21416861

RESUMO

In 2009, the recommendations of the Czech Collaborative Group for Ph- Myeloproliferative Diseases (CZEMP) for diagnosis and treatment of BCR/ABL-negative myeloproliferative diseases (MPD), i.e., essential thrombocythemia (ET), polycythaemia vera (PV) and primary myelofibrosis (PMF) were updated and extended. The present article gives the rationale of the recommendations in full detail. The CZEMP diagnostic criteria for ET and PMF are based on histopathological (HP) findings, which must unconditionally be in line with the given clinical and laboratory characteristics of ET or of a certain stage of PMF, respectively. The platelet count is not decisive for diagnosis. In cases lacking an adequately taken and read HP finding, the Polycythemia Vera Study Group (PVSG) criteria are recommended. The diagnosis of typical PV is based on demonstration of the V617F mutation of the JAK2 gene along with a significant increase of red cell parameters. If these are close to borderline, the demonstration of increased total red cell mass (RCM) is required. In atypical cases lacking polyglobulia or elevated RCM, the HP picture of PV (in accordance with WHO description) plus JAK2 V617F mutation is satisfactory for diagnosis, or, in cases lacking JAK2 V617F mutation, the HP picture of PV along with polyglobulia (or increased RCM) is sufficient. The treatment principles of ET and other MPDs with thrombocythemia (MPD-T; i.e., the early stages of PMF and PV) are identical. The patients are stratified by their thrombotic risk (preceding thrombosis, another thrombophilic state, jAK2 mutation), presence of disease symptoms (mainly microcirculatory), platelet count and age. Only patients up to 65 years lacking the above mentioned risks with a platelet count < 1000 x 10(9)/l are considered as low-risk and do not demand cytoreducing therapy. The others are high-risk ones and have an indication for thromboreduction. In patients older than 65 years, the potentially leukemogenic drug hydroxyurea (HU) may be used. In the younger ones, the choice is between anagrelide (ANG) or interferon-alpha (IFN). In high-risk patients, the treatment goal is to maintain platelet counts below 400, and in low-risk ones, below 600 x 10(9)/l. In PV, polycythemia itself is another thrombotic risk factor. The condition is treated by bloodletting or erythrocytaphereses. If hematocrit levels < or =45 are not achieved, cytoreductive therapy using HU in patients over 65 years, or IFN in younger individuals is required. All patients with thrombocythemia in PV are high-risk and have an indication for cytoreduction. Acetylsalicylic acid is given to all patients with MPD-T with platelets < 1000 x 10(9)/l (at higher counts, hemorrhage is imminent), and to all individuals with PV, unless contraindication is present. In case of platelet count normalization, it may be withdrawn in cases of low-risk ET or PMF, not in JAK2+ PV. The treatment of advanced stages of PMF is symptomatic, with substitution of blood derivatives being the basis. The only curative treatment is allogeneic stem cell transplantation, which should not be indicated too early seeing to its risks, but also not too late--we must not allow transition into acute leukemia, which is heralded by blasts in the blood picture. The indication is the presence of any of the following criteria: values of hemoglobin < 10 g/dl, WBC < 4 x 10(9)/l and platelets < 100 x 10(9)/l, any percentage of blasts or > or = 10% immature granulocytes in the differential picture, >1 erythroblast per 100 cells--all at repeated examinations within at least a 2-month interval, and in addition, rapid progression of hepato-/splenomegaly, presence of general symptoms of the disease, portal hypertension and extensive swellings.


Assuntos
Proteínas de Fusão bcr-abl , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/terapia , Humanos , Transtornos Mieloproliferativos/genética , Policitemia Vera/diagnóstico , Policitemia Vera/genética , Policitemia Vera/terapia , Guias de Prática Clínica como Assunto , Mielofibrose Primária/diagnóstico , Mielofibrose Primária/genética , Mielofibrose Primária/terapia , Trombocitemia Essencial/diagnóstico , Trombocitemia Essencial/genética , Trombocitemia Essencial/terapia
12.
Klin Onkol ; 23(3): 146-54, 2010.
Artigo em Tcheco | MEDLINE | ID: mdl-20608324

RESUMO

BACKGROUNDS: The Ann Arbor system is typically used for the staging of Non-Hodgkin's lymphomas. This classification was nevertheless originally developed in the 1970s for Hodgkin's lymphoma, a disease usually confined to the lymph nodes with less frequent dissemination to extralymphatic organs/tissues and extremely rare primary extranodal involvement. Non-Hodgkin's lymphomas, however, are more often associated with extralymphatic involvement and primary extranodal lymphomas are relatively common (approximately 1/3 of cases). Therefore, the value of the Ann Arbor staging system appears to be limited in these cases. An analysis of data from centres participating within the Czech Lymphoma Study Group showed that staging of Non-Hodgkin's lymphomas with extranodal involvement is not uniform. DESIGN: At the end of 2009, a draft for a Non-Hodgkin's lymphomas staging system was put forward for use by the lymphoma register of the Czech Lymphoma Study Group with special regard paid to the involvement of extralymphatic organs/tissues. This draft was further refined following comments from members of the Czech Lymphoma Study Group committee and the final form was accepted at the meeting of the Czech Lymphoma Study Group committee in January 2010. RESULTS: A consensus was reached at the meeting of the Czech Lymphoma Study Group committee regarding the staging of various combinations of nodal and extranodal involvement. For the purpose of suitable staging and appropriate treatment intensity, extranodal organs were divided into "major"--liver, lungs, bones, mesothelium (pleura, peritoneum, pericardium) and soft tissues. All other organs were defined as "minor". CONCLUSION: The Ann Arbor staging system is suitable for the staging of Non-Hodgkin's lymphomas with lymph node/lymphatic tissue involvement. As regards the extralymphatic spread of the disease or primary extranodal lymphomas, this classification should rather be adapted to practical needs. The validity of the updated classification system will be assessed in both prospective and retrospective Czech Lymphoma Study Group studies.


Assuntos
Linfoma não Hodgkin/patologia , Humanos , Linfoma não Hodgkin/classificação , Estadiamento de Neoplasias
13.
Neoplasma ; 57(5): 449-54, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20568899

RESUMO

Primary mediastinal B-cell lymphoma (PMBL) seems to be reliably distinguished from diffuse large B-cell lymphoma (DLBCL) with microarray technology. We measured expression of Fcer2, Pdl2 and Blk genes using real-time quantitative polymerase chain reaction (RTqPCR) on formalin fixed, paraffin embedded material (FFPE) and suggested a formula to discriminate PMBL from DLBCL. For 39/82 included patients the diagnosis of PMBL was expected clinico-pathologically. Diagnosis of 10/39 and 2/43 of clinically considered PMBLs and DLBCLs, respectively, was not genetically confirmed. Compared to confirmed PMBLs, unconfirmed ones showed clinical features similar to DLBCLs, e.g. spleen infiltration (p=0,028) and decreased invasiveness in pericardium (p=0,045). They tended to have more common infradiaphragmatic involvement, less often tumor sclerosis or fluidothorax. There were no immunohistochemical differences between genetically confirmed and unconfirmed PMBLs. New approach of distinguishing PMBL and DLBCL is presented. It is based on expression of three genes in routinely available FFPE material using RTqPCR.


Assuntos
Linfoma de Células B/diagnóstico , Linfoma Difuso de Grandes Células B/diagnóstico , Neoplasias do Mediastino/diagnóstico , Reação em Cadeia da Polimerase/métodos , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade
15.
Neoplasma ; 53(3): 219-25, 2006.
Artigo em Inglês | MEDLINE | ID: mdl-16652191

RESUMO

Morphological examination is the routine first step in the diagnosis of hematological malignancies, including chronic lymphocytic leukemia (CLL). Atypical cell morphology according to the FAB criteria is known to herald disease progression. Several years ago, it was proposed that FAB morphology at diagnosis had a considerable prognostic impact. However, this proposal has not been widely adopted in practice. Thus we questioned the prognostic value of the morphological examination, which was performed retrospectively in 88 patients out of our 110 institutional registry patients (70 males and 40 females, median age 57 yrs) with CLL at diagnosis. We related the results to the more modern prognostic markers. Atypical FAB morphology was shown to correlate with IgVH gene mutation status, trisomy of chromosome 12 and deletion of 17p detected either by conventional G-banding or by fluorescence in situ hybridization (FISH) analysis. The correlation of FAB morphology with CD38 antigen expression or with the histopathological pattern of bone marrow infiltration was not significant. Overall survival (OS) data were available for 84 morphologically examined patients. The patients with atypical morphology (64 patients) had a significantly shorter OS (103 months) than the 20 patients presenting with typical CLL morphology (237 months; p=0.03). Only the mutation status of IgVH genes correlated more closely with OS (p=0.002). Of note, there was no leukemia-related death within "unmutated" cases who had typical FAB morphology (p=0.14), and vice versa, the mutation status had a significant prognostic impact within the morphologically atypical cases (p=0.01). Thus FAB morphology and the mutation status may yield complementary prognostic information. OS was affected both by the presence of cytogenetic aberrations (p=0.03) - most adversely by deletions of 17p and 11q, and by CD38 expression (p=0.003). We conclude that careful examination of peripheral blood smears according to FAB is a simple, cheap and valuable tool in the first-line assessment of prognosis of CLL patients and should not be overlooked even in 3rd millennium when more sophisticated prognostic markers are at hand. This ought to be confirmed in larger prospective studies with multivariate analysis of data.


Assuntos
Cadeias Pesadas de Imunoglobulinas/genética , Região Variável de Imunoglobulina/genética , Leucemia Linfocítica Crônica de Células B/mortalidade , Mutação , ADP-Ribosil Ciclase 1/análise , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Leucemia Linfocítica Crônica de Células B/genética , Leucemia Linfocítica Crônica de Células B/patologia , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Prognóstico
16.
J Eur Acad Dermatol Venereol ; 18(3): 347-9, 2004 May.
Artigo em Inglês | MEDLINE | ID: mdl-15096153

RESUMO

Myoblastic myoma was first described in 1926. Immunohistochemical methods have proven the neuroectodermal origin of this tumour. It most frequently affects individuals between 30 and 60 years of age, with a significant female predominance. In most cases it is a benign solitary tumour, with multiple lesions found in 25% of cases. The malignant variant of the tumour is diagnosed in less than 3% of cases. This case report of a 30-year-old woman describes the appearance of a solid resistance between her breasts following delivery of her child, with similar findings on the neck and wrists. Histopathological examination confirmed the presence of a benign variant of myoblastic myoma.


Assuntos
Tumor de Células Granulares/patologia , Neoplasias Cutâneas/patologia , Adulto , Biópsia por Agulha , Feminino , Seguimentos , Tumor de Células Granulares/cirurgia , Humanos , Imuno-Histoquímica , Medição de Risco , Neoplasias Cutâneas/cirurgia , Resultado do Tratamento
17.
Vnitr Lek ; 50(10): 786-92, 2004 Oct.
Artigo em Tcheco | MEDLINE | ID: mdl-15633936

RESUMO

Kikuchi-Fujimoto disease is a rare feverish disease characterised by lymphadenopathy, the most frequently cervical, exanthem, arthralgias and arthritis. It affects especially young women. Patients have high erythrocyte sedimentation rate (ES) and leucopenia, antibodies are missing. Course of the disease is usually very benign and can subside spontaneously. However, clinical picture is usually very dramatic and can suggest infectious, autoimmune or malign systemic disease; also association with some of autoimmune diseases was described. Its occurrence is sporadic in all the world, the most of cases were seen in Asia, in the Czech Republic it has not been yet described. Therapy consists in antibiotics administration followed with corticoid therapy and usually can restore patients to perfect health. However, exacerbations have also been described. We describe a case of a 60 year old man, a past top sportsman, who has never been seriously ill except sport traumas and prosthesis implantation for coxarthritis reasons. The last two years he has suffered from exanthem and leucopenia of an unclear origin. In May 2002 he become feverish and arthritis, lymphadenopathy, splenomegalia and exanthem progression, high ES rate and high serum level of C-reactive protein (CRP) appeared in him. His condition was first evaluated as septic condition (founded staphylococci in two blood cultures), however, cause of potential sepsis has not been identified. The patient was treated with antibiotics with improvement of his total health condition after second treatment regiment. A neck node biopsy was done because of suspicion on lymphoprolipherative disease and histiocytic necrotizing lymphadenitis of Kikuchi type was found. Autoantibodies assessment was completely negative. After antibiotic and corticoid therapies his clinical condition quite quickly standardized and ES rate and serum CPR level decreased. 4 month after lowering the dose of prednisolon a temporary exacerbation of the disease appeared and again disappeared after increasing the dose of corticoid.


Assuntos
Linfadenite Histiocítica Necrosante/diagnóstico , Linfadenite Histiocítica Necrosante/terapia , Humanos , Masculino , Pessoa de Meia-Idade
18.
Cas Lek Cesk ; 142(11): 651-5, 2003.
Artigo em Tcheco | MEDLINE | ID: mdl-14689823

RESUMO

BACKGROUND: Immunophenotyping of malignant lymphomas becomes necessary for the correct classification, for the design of therapy and for prognosis projection (WHO). Although the spectrum of classic immunohistochemical (IHC) examinations in paraffin embedded or frozen sections has recently considerably extended, IHC should preferably be combined with flow cytometry. The main advantage of flow cytometry is a synchronous application of two or more antibodies marked with various fluorochromes in one sample. The method is limited by utilizing native material only. METHODS AND RESULTS: The flow cytometry combined with histological and IHC investigations were used in diagnosis of primary non-Hodgkin's lymphomas of B-cell origin (B-NHL) and for bone marrow staging or restaging. We studied 90 patients with confirmed or suspected B-NHL and we found a good correlation in 89% of samples of primary lymphomas and in 85% of bone marrow samples when IHC and flow cytometry results were compared. The overall efficacy of the flow cytometry determination in lymphoma infiltration of the samples was 89%. CONCLUSIONS: Immunophenotyping utilizing flow cytometry contributes to diagnosis and classification of B-cell lymphomas in the significant proportion of investigated patients. In some cases it is even unnecessary to employ IHC examination of tissue sections. The method is especially suitable for determination of monoclonal populations of B-cells by detection of cell surface markers because it is more specific and sensitive than IHC. The immunophenotyping by flow cytometry as an auxiliary method and in correlation with morphological findings it can make the diagnosis of B-cell lymphomas faster and more specific.


Assuntos
Medula Óssea/patologia , Citometria de Fluxo , Imunofenotipagem , Linfoma de Células B/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia por Agulha , Citodiagnóstico , Feminino , Humanos , Linfoma de Células B/patologia , Masculino , Pessoa de Meia-Idade
19.
Cesk Patol ; 39(3): 120-5, 2003 Jul.
Artigo em Tcheco | MEDLINE | ID: mdl-14631809

RESUMO

The knowledge about MALT lymphomas has dramatically changed in the last few years. The genesis of MALT lymphomas has become clearer, and new tools in modern diagnostics are available. This article, as a concise review, summarises the latest data and the clinical and morphological characteristics with the aspect of routine diagnostics. The characteristic translocation t(11;18) and its chimeric transcript API2-MALT1 with antiapoptotic function is emphasised.


Assuntos
Linfoma de Zona Marginal Tipo Células B , Neoplasias Gástricas , Humanos , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/genética , Linfoma de Zona Marginal Tipo Células B/patologia , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/genética , Neoplasias Gástricas/patologia
20.
Cas Lek Cesk ; 142(7): 417-22, 2003.
Artigo em Tcheco | MEDLINE | ID: mdl-14515445

RESUMO

BACKGROUND: Diffuse large B-cell lymphomas represent a heterogeneous group of tumors with a different origin, morphological findings and a variable clinical prognosis. These tumors have been recently classified into two prognostically relevant subgroups differing in the gene expression. The key genes suitable for routine diagnostics of DLBCL have not been yet identified. The aim of this work was to study changes and expression of several genes and proteins participating in the genesis of DLBCL. METHODS AND RESULTS: We analysed a group of 31 patients with diffuse large B-cell lymphomas. Basic clinical data including follow-up of the patients were available. Tumors were examined by a panel of immunohistochemical reactions with antibodies against CD20, CD79a, BCL-2, BCL-6, CD10, Ki-67 and TP53. FISH was used to detect a translocation t(14;18)(q32;q21) and/or a break in BCL6 region (3q27) suggestive of a translocation with a variable translocation partner t(3;?). PCR was utilized to detect the translocation t(14;18) and a clonal rearrangement of heavy and/or kappa chain of the immunoglobin genes. CONCLUSIONS: The expression of BCL-2 protein appeared to correlate with a higher mortality rate. The expression of other proteins examined in the study did not correspond significantly with the clinical development of the disease. Tumors with follicular lymphoma as a component had significantly higher mortality rate than the tumors developing de novo. Moreover, higher mortality was evident in cases with higher values of the International Prognostic Index (IPI).


Assuntos
Linfoma de Células B/diagnóstico , Linfoma Difuso de Grandes Células B/diagnóstico , Proteínas de Ligação a DNA/análise , Proteínas de Ligação a DNA/genética , Humanos , Imuno-Histoquímica , Linfoma de Células B/genética , Linfoma Difuso de Grandes Células B/química , Linfoma Difuso de Grandes Células B/genética , Linfoma Difuso de Grandes Células B/mortalidade , Neprilisina/análise , Prognóstico , Proteínas Proto-Oncogênicas/análise , Proteínas Proto-Oncogênicas/genética , Proteínas Proto-Oncogênicas c-bcl-6 , Taxa de Sobrevida , Fatores de Transcrição/análise , Fatores de Transcrição/genética , Translocação Genética
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