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Biomedical subjects
Publications and source records attributed to A Jirásek.
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Idiopathic myelofibrosis may be accompanied by portal hypertension. The authors report a 56-year-old man with idiopathic myelofibrosis and splenomegaly complicated by hepatopathy, severe portal hypertension and recurrent variceal bleeding. A transjugular intrahepatic porto-systemic shunt (TIPS) was inserted. Variceal bleeding never recurred. A short episode of encephalopathy, which is a known complication of porto-systemic shunting, ceased promptly after conservative treatment. The patient eventually died six months later due to metabolic deterioration and hepatic failure related to his underlying hematological disease. TIPS is a promising treatment modality for alleviating symptomatic portal hypertension in hematological disorders.
We report a unique case of aleukemic granulocytic sarcoma of the neck, originally misdiagnosed as non-Hodgkin's lymphoma (NHL), though chloroma was also suspected due to a greenish macroscopic appearance and the presence of myeloid chloroacetate esterase (CAE)+ cells. The proof of clonal T cell receptor gamma chain (TcRgamma) gene rearrangements in the recurring tumor was deemed to confirm the initial diagnosis of T cell NHL. Altogether five distinct types of clonal TcRgamma gene rearrangements were found in the tumor, bone marrow and peripheral blood. Only retrospectively, using RT-PCR, did we detect the acute myeloid leukemia subset-specific fusion gene AML1/ETO in the frozen samples of the relapsed tumor, as well as in the otherwise unaffected bone marrow and peripheral blood (representing 'minimal initial disease' in the latter two samples). Simultaneous staining verified that the neoplastic CAE+ cells and CD45RO+ T cells were different populations.
The expression of cytoplasmic c-erbB2, epidermal growth factor receptor (EGFR), proliferating cell nuclear antigen (PCNA) and dipeptidylpeptidase IV (DPP IV) was significantly higher in sporadic cancer of the right than of the left colon. In addition, cytoplasmic c-erbB2 displayed the same difference in the adjacent (less than 2 cm) and distant (more than 5 cm from the tumour margin) mucosa. The findings cannot be related to Dukes staging. It is suggested that different ontogenic development of the right (from the midgut) and the left (from the hindgut) colon may be a possible explanation. Therefore, data on the expression of different molecular markers in colorectal cancer and surrounding mucosa should always be supplemented by data on tumour location.
Progressive multifocal leucoencephalopathy is caused by infection with JC virus. The disease affects patients with immunodeficiencies, hematologic diseases, and patients treated with radiotherapy. The disease is characterised by foci of demyelinisation with atypical astrocytes and oligodendrocytes. Oligodendrocytes contain typical intranuclear inclusions. Progressive multifocal leucoencephalopathy and its verification is presented in three cases. Two patients died of progression of a malignant neoplasm and the leucoencephalopathy was a complication of the malignancy. The third case was a biopsy specimen taken from the brain of a patient who received a renal transplant. The material of all patients was analysed by light and electron microscopy, and in situ hybridisation with a probe specific for JC virus. In situ hybridisation proved to be the most specific and a simple method to demonstrate the infection in all cases. It is useful in instances in which the histologically detectable lesion is not characteristic, and in cells in which the conventional histologic methods fail to reveal the intranuclear inclusions of JC virus.
We have studied tissue expression of the cytokine receptors using a high sensitivity biotin-streptavidin system on cryostat sections. We used a panel of monoclonal antibodies from the 6th International Workshop on Human Leukocyte Differentiation Antigens, namely CD25 (IL-2R alpha), CD95 (FAS antigen), CD116 (GM CSFR), CD117 (SCFR), CD120 alpha (TNFR I), CD120b (TNFR II), CD121a (IL-1R I), CDw123 (IL-3R), CD124 (IL-4R), CD126 (IL-6R), CD127 (IL-7R), CDw128 (IL-8R), CD130 (gpl130), CD131 (IL-3R), CD132 (IL-2R gamma), CD134 (OC-40), CD135 (FLT3/FLK2). Examined tissues (lymph nodes and spleens) were obtained from 12 patients with folicular non-Hodgkin's lymphoma, periferal T non-Hodgkin's lymphoma, B lymphoma, myeloma, Hodgkin's disease, two cases of T cell rich B-lymphoma, autoimmune haemolytic anemia and two cases of rudimentary trombocytopenic purpura. Our results indicate that immunohistological technology using native tissues on cryostat sections, monoclonal antibodies and the visualisation with biotin-streptavidin is a particularly suitable supplementary staining procedure for detection of the cytokine receptors in tissues.
In the submitted case-history the authors wished to draw attention to serious complications after transplantation. Posttransplantation lymphoproliferation (PTLP) is a rare complication of organ transplantation, its incidence amounts to some 2% of organ recipients, in combined heart-lung transplantations the incidence is as high as 10%. The prerequisite of lymphproliferations is infection with the Epstein-Barr virus. The virus causes transformation of B lymphocytes and subsequent lymphoproliferation. Immunosuppressive preparations, due to their effect on Tlymphocytes promote this transformation. The decisive imaging method in tumoriform occurrence is high resolution computed tomography. Based on CT examination surgical biopsy is performed with subsequent in situ hybridization which confirms unequivocally the diagnosis of posttransplantation lymphoproliferation. Only on the basis of results of in situ hybridization treatment may be started which involves restriction or discontinuation of immunosuppressive treatment and administration of antiviral preparations. Frequently this treatment fails and must be discontinued on account of a rejection reaction of the organism. The prognosis in untreated forms is adverse.
Immunologic activation of the splenic white pulp seemed to be an important feature of the structural changes in the 8 splenectomized patients with ITP. However, destruction of the platelets with CD 16+ monocytes was the most prominent change in the Billroth's cords of the splenic red pulp. Classical NK cells did not play an important role in platelets destruction.
Recombinant human hematopoietic growth factors are widely used in the treatment of multiple myeloma (MM) especially due to the increasing role of autologous blood stem cell transplantation (ABSCT). We report a patient with MM in whom rapid extramedullary progression of disease was observed during stem cell mobilization with G-CSF. In 56-year-old man with relapsing IgG lambda MM myeloablative therapy with ABSCT was planned 2 years after diagnosis. G-CSF is increasing doses was used for mobilization. Ten days after the start of G-CSF therapy 2 extramedullary (subcutaneous) myeloma infiltrates appeared. For the second mobilization high dose cyclophosphamide and VP-16 with subsequent G-CSF was used. During the time of chemotherapy tumour infiltrates disappeared, however, after one week of G-CSF treatment rapid progression of disease with the formation of multiple extramedullary infiltrates occurred and the patient died in June 1996. Small pieces of subcutaneous tumour infiltrates were removed at autopsy and immediately frozen in liquid nitrogen. Using the panel of specific antibodies the expression of cytokine receptors (IL-1, 2, 3, 6, 7, 8, 10, SCF, gp130, G-CSF, GM-CSF, EPO) and Fas, Pgp and 24-34 kD multidrug resistance-associated protein were examined. However, no expression of cytokine receptors on tumor cells was found. On the contrary, high positivity of surface MDR associated proteins was observed.
A short review on the interaction of hematopoietic growth factors with their receptors in the regulation of hematopoiesis. The introductory notes on the nature of cytokines and cytokine receptors, their biological and physicochemical properties as well as clinical and diagnostic consequences are mentioned.
The authors describe the case of a 66-year-old female patient after transplantation of the kidney where a rare complication of immunosuppressive treatment was diagnosed--progressive multifocal leukoencephalopathy. The disease was manifested by gradually developing hemiparesis of the lower extremity five months after transplantation. Examination of the brain by computed tomography and examination of cerebrospinal fluid were normal. Subsequently the neurological finding developed into quadruparesis. Imaging of the brain by magnetic resonance revealed multiple demyelinization foci. In brain biopsy there were typical structures of progressive multifocal leukoencephalopathy and positive hybridization in situ for JC-virus in cell nuclei. Immunosuppressive treatment was restricted and ganciclovirus administration was started. Further progression of the clinical symptomatology ceased, the function of the transplanted kidney remained satisfactory. The patient died 14 months after the transplantation from sepsis. Examination of the post-mortem material revealed positivity of the JC-virus only in the cytoplasm of the affected cells. As effective treatment of infection with JC-virus is not known so far, the possible action of ganciclovirus is only speculative.
The authors describe the case of a 20-year-old patient where the first leading symptom was hypercalcaemia. A similar case was not published so far in the Czech literature. The disease took a fulminant course and proved fatal nine days after the first symptoms of the disease. The correct diagnosis was established only by necropsy. The adverse course of the disease could not be influenced by repeated haemodialysis nor by the administration of disodium pamidronate (Aredia) and calcitonin. The authors discuss differential diagnostic problems of hypercalcaemias and the pathogenesis of hypercalcaemia in malignant diseases of the haematopoietic system.
During 1991-94 we treated 51 patients with acute myeloid leukaemias and 3 patients with a myelodysplastic syndrome of refractory anaemia with excess of blasts in transformation. The patients received trimethoprim-sulphamethoxazole (TMP-SMX) 1,920 mg daily as a prophylaxis of Pneumocystis carinii infections and selective decontamination of gastrointestinal tract. The majority of patients received TMP-SMX in their first course of chemotherapy with daunorubicin and cytosine arabinoside. Only one of the 18 patients without TMP-SMX prophylaxis during the first course of chemotherapy developed Pneumocystis carinii pneumonia. That pneumonia was successfully treated by intravenous administration of TMP-SMX 1920 mg four times a day. No other Pneumocystis carinii infection was encountered in all other patients during their clinical follow up or in autopsy material of expired patients. TMP-SMX prophylaxis had to be interrupted in 11 patients due to their suspicious allergic skin reactions, however, TMP-SMX was readministered in all without any skin changes attributable to TMP-SMX during next cycles of chemotherapy. TMP-SMX in a given daily dose of 1,920 mg seems to be a successful prophylaxis of Pneumocystis carinii infections in patients with malignant diseases of hematopoiesis.
Leukemic blasts of two patients with acute leukemia exhibited similar characteristics. They were heterogeneous in size with a diameter of 14-30 microns in smears and unclassifiable by morphological, cytochemical, immunophenotypic and ultrastructural examinations. Cytogenetic examinations of both revealed a near-tetraploid karyotype. Blasts from both patients differentiated into macrophages in cultures with 10 ng/ml 12-O-tetradecanoylphorbol-13-acetate (TPA) which is a feature specific for myeloid blasts and the cases were thus classified as poorly differentiated acute myeloid leukemias (AML M0). Near-tetraploid poorly differentiated acute myeloid leukemias M0 seem to be a special category of AML in the morphologic, immunologic and cytogenetic (MIC) classification. The presence of very large blasts in the heterogeneous blast population in acute unclassified leukemias could be a morphological sign of near-tetraploid leukemias AML M0.
The authors describe a case of extrapulmonary tuberculosis with affection of the reticuloendothelial system in a 59-year-old female patient. After successful treatment the findings in the spleen, bone marrow and the laboratory results had normal levels. The authors draw attention to the diagnostic difficulties. They were able to confirm the diagnosis only by histological examination of biopsy from the pelvic bone. An interesting finding was eruption of lupus vulgaris on the legs in the course of treatment. The authors discuss some aspects of the disease and its differential diagnosis.
The authors present a report dealing with their experience with reoperations for occlusion of vascular prostheses implanted in aorto-femoral region in the late postoperative period. The most frequent cause of this occlusions was the obstruction of the outflow track at the distal anastomosis. In small arteries (under 5 mm) this obstruction was mainly caused by myointimal proliferation which originated from the host artery. In the early period after occlusion it is possible to remove the thrombus from the prostheses mostly in all patients by means of Fogarty catheter and to ensure the long lasting passage of the prostheses by plastic operations of the origin of profunda femoral artery. In long lasting occlusions of the prostheses can thrombolytic therapy 1-2 weeks before reoperation facilitate removing of the thrombus. In 113 patients with the different type of reoperations for occlusion of the prostheses in the aorto-femoral region we succeeded in 102 patients (90.3%). In 8 patients the reoperation was not successful (7%) and we had to amputate the distal extremity and three patients (2.3%) died from the heart complications in the early postoperative period.
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