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Lege Artis Medicinae

DECEMBER 20, 2007

[MYELODYSPLASTIC SYNDROMES - NEW THERAPEUTIC OPTIONS]

GADÓ Klára

[Myelodysplastic syndrome is a heterogeneous group of acquired clonal disorders of the haematopoietic stem cell characterized by ineffective haematopoiesis, peripheral cytopenia, and a high risk of progression to acute leukaemia. It is a common malignant disease with an increased incidence in the elderly population. Classification is based on a 1999 WHO recommendation, in which morphological features as well as clinical and cytogenetic characteristics are taken into account. Combined with the International Prognostic Scoring System (1997), it is suitable to predict prognosis and response to therapy. Clinical features include symptoms caused by anaemia, infections, and bleeding. Diagnosis is based on peripheral cytopenia and dysplastic morphology, as well as normal or increased cellularity in the bone marrow, with more than 10% of dysplastic cells. The verification of cytogenetic abnormalities is important both for confirming the diagnosis and predicting the prognosis. When designing the treatment strategy, it is essential to take the risk of leukaemia into account. On the other hand, the general state of the patient and the presence of accompanying diseases should also be considered. The goal of the treatment is to increase cell count and to decrease transfusion requirement, eventually to improve quality of life. Supportive therapy is an essential part of the management. In addition, growth factors, immunosuppressive and immunomodulatory agents, low-dose chemotherapy may be applied. Today, cure can only be achieved by allogenic stem cell transplantation. Recent findings in the epigenetic intracellular regulation allowed the definition of new therapeutic targets to develop drugs such as inhibitors of DNA methyltransferase and histone deacetylase.]

Lege Artis Medicinae

FEBRUARY 20, 2003

[New molecular based methods for diagnosis, classification and prognosis of leukemias]

ZVARA Ágnes, HACKLER László ifj., PUSKÁS László G.

[Normal functions of the cell are based on the precise regulation of various genes. If this strict regulation and the hierarchy of genes becomes upset due to some flaws of the system, the result will be cellular dysfunction which may eventually lead to carcinogenic transformation. The two main challenges in the classification of cancers are the discovery of new molecular markers characteristic to defined disease groups and the classification of already diagnosed or new cases into existing groups. This precise classification may open the door to tailored treatment or project the expected outcome of the disease. Today, there is unlimited access available to the databases containing sequences and localisation of the genes within the confines of Human Genome project. It provides significant help for the discovery of chromosome abnormalities and systematic analysis of gene expression patterns. This is important not only to understand normal functions of the cells, but it also contributes to the identification of new genes that are characteristic to given disease groups as markers and that are potential drug targets. Until the second half of the twentieth century the study of the function and regulation of genes was based on step by step investigation of individual genes. The fact that the genomes of an increasing number of organisms have become identified in whole or in part, numerous new techniques have been developed facilitating the systematic analysis of gene functions. The aim of this study is to summarise the new, molecular based possibilities for classification, diagnosis and prognosis of cancers, as well as to summarise the results of these areas, primarily from the point of view of leukemias.]

Ca&Bone

SEPTEMBER 11, 2007

[Femoral head osteonecrosis in a child with acute lypmhoblastic leukemia]

GÁCS ZSÓFIA, KOVÁCS GÁBOR, HOSSZÚ ÉVA

[INTRODUCTION - The treatment of pediatric leukemia has become increasingly successful, with a survival rate over 80%. Thus interest has been increasingly focused on the long-term side-effects of the treatment. The questions of reduced fertility rate, occurance of second malignancies, cardiomyopathy, impaired renal and pulmonal function have been extensively studied. Changes of bone metabolism in connection with the disease itself and the treatment have been analysed in the past decade. CASE REPORT - We present the case of a 15-year-old boy with acute lymphoblastic leukemia, who had bone pain soon after the diagnosis. During the course of chemotherapy his complaints were fluctuating, and he developed severe osteoporosis. The level of a bone resorption marker, β-CrossLaps, was elevated. In the second year of therapy an acute pain of the left hip occured with fever and restriction of joint movement, which was diagnosed and treated as osteomyelitis. A few months later avascular necrosis of the left femoral head was revealed. Both pharmaceutic (calcium, vitamin D, calcitonin, bisphophonate) and orthopedic treatment were used, as a result bone mineral density and movement restriction improved; his leukemia is now in remission. CONCLUSIONS - The factors influencing bone metabolism in leukemic children are reviewed. Firstly the effects of malignant cells on bone mineral content are analyzed, then the chemotherapeutic drugs’ mechanisms of action are examined extensively. The direct and indirect effects of secondary factors (hospitalization, immobility, lack of sun exposure, malabsorption, immunsuppression, peripheral neuropahty) are also analyzed. The advantages and disadvantages of drugs used in preventing and treating childhood osteopenia are reviewed.]

Clinical Neuroscience

MAY 30, 2010

[Intracranial propagation of invasive aspergilloma in an immuncompromised patient]

SIMON Márta, VASTAGH Ildikó, VÁRALLYAY György, TURÁNYI Eszter, SRÉTER Lídia, CZIRJÁK Sándor, BERECZKI Dániel

[Aspergillus infection of the central nervous system is a rare disease, occasionally seen among immunocompromised patients. The most frequent pathway is hematogenic dissemination. Less known is the direct propagation from the paranasal sinuses, which is usually observed in immunocompetent patients. We report a patient who developed cavernous sinus syndrome due to an invasive intracranial aspergilloma after longlasting chemo- and steroid therapy for chronic lymphoid leukemia and immunhemolytic anemia. The characteristic features seen on radiological images - brain CT and MRI - suggested the possibility of invasive aspergilloma. Postoperative histology defined the diagnosis. Our case review highlights the importance of considering the possibility of an invasive opportunistic infection of the CNS in an immunocompromised patient presenting a new neurological sign.]

Clinical Neuroscience

MAY 20, 2007

[PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY]

PÁL Endre, ASCHERMANN Zsuzsanna, GÖMÖRI Éva, KOVÁCS Gábor Géza, SIMON Gábor, MARÓDI László, KOMOLY Sámuel, ILLÉS Zsolt

[Progressive multifocal leukoencephalopathy is a rare disease caused by the reactivation of an opportunistic agent, JC virus almost in every cases in immunodeficient conditions. The disease is characterized by multifocal demyelinating lesions of the central nervous system and causes death within a few months. The authors report two patients: a 67 year-old male treated because of chronic lymphoid leukemia, and a 19 year-old male having a hereditary immunodeficiency, X-linked hyper IgM syndrome. In both cases continuously progressive right, later both hemispheric signs were detected. Cerebrospinal fluid was not helpful. Brain MRI showed bilateral large, white matter lesion. The progression was not influenced by the treatment, finally both patient died ten and six weeks after the appearance of first complaints. The diagnosis was confirmed by brain biopsy and autopsy in both cases. Our cases demonstrate that progressive multifocal leukoencephalopathy can develop in various immunodeficiencies.]

Lege Artis Medicinae

MAY 20, 2011

[Increasing importance of invasive fungal diseases]

PRINZ Gyula, SINKÓ János

[INTRODUCTION - Invasive mycoses are life-threatening fungal infections affecting patients with severe underlying conditions or impaired immune system. According to epidemiological studies, the incidence of invasive mycosis is increasing in several patient groups. CASE REPORT - In patients who require ICU care, the majority of invasive mycoses are caused by Candida species. A shift has been observed within the spectrum of pathogenic species. In patients undergoing chemotherapy for acute leukemia and in recipients of allogenic stem cell transplantation, however, an increased morbidity and mortality of invasive aspergillosis have been observed. The authors discuss current issues regarding the diagnosis and treatment of invasive fungal diseases on the basis of case reports. CONCLUSION - Invasive mycoses are lifethreatening infections affecting patients with severe underlying conditions. While the opportunities of state-of-the-art medicine are expanding, the incidence and significance of these infections increases. If detected and treated in time with appropriate antifungal agents, these infections can be efficiently treated.]

Hungarian Radiology

JUNE 20, 2007

[Sonographic appearance of the cytostatic therapyrelated hepatic injury]

JÓKÚTI László, VARGA Edit, KARÁDI Zoltán, KOVÁCS GÁBOR

[INTRODUCTION - The correlation between biochemical parameters and sonographic appearance of the liver in children and young adults receiving cytostatic therapy was investigated. PATIENTS AND METHODS - 104 (54 male, 50 female) patients at the hemato-oncologic unit of 2nd Department of Pediatrics, Semmelweis University Budapest were enrolled into this prospective, single-blind, uncontrolled study: patients’ ages were between 2.0 and 32.7 years (mean 12.2 yrs, ± SD 5.7). 69 patients received chemotherapy for acute lymphoblastic leukemia (ALL), 35 patiens for osteogenic osteosarcoma (OSC). The time interval between the initiation of the cytostatic therapy and the examination was between 1 month and 16 years (mean 3.9 yrs). Ultrasonography was performed after 5-8 hours of fasting, without sedation. Echogenicity, distal attenuation of liver parenchyma and Doppler waveforms of the hepatic vein branches were evaluated. Alanine-aminotransferase (ALT) and gamma-glutamyltransferase (GGT) activity were measured in peripheric blood samples. Statistical correlation was analysed between sonographic appearance and biochemical parameters of the liver. RESULTS - 35 patients had at least one sonographic abnormality: of these, 9 had elevated enzyme activity. Among the 69 patients with normal ultrasound findings, only 2 had increased enzyme activity (Chi square test, p=0.001). When tested against enzyme activity, echogenicity and attenuation showed significant (p=0.002 and p=0.01, respectively), Doppler waveform in the hepatic vein branches showed marginally significant correlation (p=0.05). All three ultrasound parameters had low sensitivity regarding the elevation of enzyme levels, however attenuation and hepatic vein waveform proved to be specific (both 94%) for enzyme level elevation and all three showed high negative predictive values (96%, 93% and 92%, respectively). When combining all three sonographic parameters in a single variable, correlation was even higher (p=0.001), sensitivity became acceptable (82%) and negative predictive value increased further to 97%. CONCLUSIONS - The authors conclude that a correlation exists between three simple sonographic indicators (echogenecity, distal attenuation, Doppler waveforms of the HVs) and liver injury detected by biochemical parameters. Due to the low sensitivity of the ultrasound parameters to confirm of the presence of diffuse liver injury is not possible. However, the combined use of the ultrasound and biochemical parameters a good negative predictive power can be achieved and therefore this is a useful tool in the follow-up for hepatic status.]

Hungarian Immunology

OCTOBER 10, 2005

[Extensive flow cytometric characterization of plasmocytoid dendritic cell leukemia cells]

GOPCSA László, KORMOS Luca, BÁNYAI Anikó, TAMÁSKA Júlia, MATOLCSY András, GOGOLÁK Péter, RAJNAVÖLGYI Éva, PÁLÓCZI Katalin

[INTRODUCTION - Accumulating evidences suggest that non-T, non-B cell CD4+/CD56+ neoplasms with lymphoblastic morphology include clinically and immunophenotypically diverse entities. Although their cells of origin or classification are still controversial several entities clearly represent a distinct type of neoplasms that are clinically aggressive. CASE REPORT - In this work we present the immunophenotypic and genotypic features of bone marrow, peripheral blood, lymph node and skin lymphocytes from a patient diagnosed as plasmacytoid dendritic cell leukemia involving the skin, bone marrow, peripheral blood, lymph nodes, liver and spleen. For determination of immunophenotypic characteristics of malignant plasmacytoid dendritic cells 73 monoclonal antibodies detecting lineage markers, chemokine receptors, cytokine receptors, activation and co-stimulatory molecules were used. The malignant cells proved to express CD4+, CD56+ lineage negative leukemia phenotype characteristically positive for CD36, CD38, CD40, CD45, CD45RA, CD68, CD123, CD184, HLA-DR, BDCA2 and granzyme-B corresponding to the preplasmacitoid dendritic cell developmental stage. CONCLUSION - The presence of CD11a/CD18, CD84, CD91, CD95, αvβ5, CDw197 and the absence of CD52 and CD133 in this case can be regarded as additional features of malignant cells.]

Lege Artis Medicinae

JUNE 22, 2004

[ONCOHEMATOLOGIC MALIGNANCIES WITH SKIN SYMPTOMS]

BENE Ibolya, ERŐS Nóra, KÁROLYI Zsuzsánna, TAKÁCS István, RADVÁNYI Gáspár

[INTRODUCTION - Haematologic malignancies can originate from the skin (cutaneous lymphomas, rarely acute myelomonocytic leukemia) or can infiltrate the skin secondarily during the progression of the disease (nodal and systemic non-Hodgkin’s lymphomas, Hodgkin'’s disease, chronic lymphocytic leukemia). PATIENTS AND METHODS - The clinical history of seven patients treated by the authors between 1997-2003, is reviewed. CONCLUSIONS - The clinical and histopathologic features of each entity are discussed, emphasizing differences in the clinical course between cutaneous and nodal lymphomas, considering diagnostic difficulties, conventional and recent therapeutic approaches.]

Hungarian Immunology

APRIL 20, 2003

[Immunophenotyping of mature cell non-Hodgkin’s lymphomas with leukemic clinical manifestation - newer approaches]

PÁLÓCZI Katalin, NÉMETH Julianna, BÁNYAI Anikó, GOPCSA László

[Immunophenotyping is commonly used in evaluating malignancies of the lympho-hemopoietic system and its use in various disease states of mature lymphoid leukemias and related non-Hodgkin’s lymphomas is reviewed here. The major goals of immunophenotyping in mature lymphoid neoplasias are the assignment of abnormal cells to the B or T/NK linkage, their maturational analysis, and the characterization of specific phenotypes which might be helpful for the subclassification of disease. There is not known, however, any lymphoma (leukemia) -specific antigen and the individual type of lymphoid leukemias and lymphomas does not follow the antigen expression profile of normal differentiation. Therefore, the approach to analysis of lymphoid neoplasias requires thoughtful utilization of laboratory testing, in order to meet both medical and economic goals of the laboratory and caregivers. The interpreter should expect to see a pattern of both positive and negative immunoreactivities that is appropriate to the final interpretation. The value and type of information provided by immunophenotyping in these malignancies varies and this paper outlines approaches for clinicians and laboratorians to follow when reviewing clinical data. The future for this technology is outstanding because it is the only one available today that can both rapidly and accurately measure multiple correlated cell properties. However, combined clinical-laboratory approach to diagnosis and prognostication seems to be important including traditional and newer (molecular genetic, molecular biology) methodologies.]