Clujul Medical - Iuliu Haţieganu

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Clujul Medical 2006 vol. LXXX - nr. 1 4. GREER IP, KINNEY MC: Acute nonlymphocytic leukemia; in LEE RG, BITHEL FE, FOERSTER J, ATHENS JW, LUKENS JN. Wintrobe’s Clinical Hematology. Ninth Edition. Lea and Febiger. London 1993, pp 1920-1945. 5. HATTORI H, MATSUZAKI A, SUMINOE A, IHARA K, NAKAYAMA H, HARA T. High expression of platelet derived growth factor and transfoming growth factor beta-1 in blast cells cells from patients with myeloprolipherative disorder and organ fibrosis Br J Hematol, 2001; 115 (2): 472-475 6. SHEIKA A. Fatal familial infantile myelofibrosis. J. Pediatr Hematol Oncol, 2004; 26 (3): 164-168. 7. REILLY JT Cytogenetic and molecular genetic aspects of idiopathic myelofibrosis. Acta Haematol 2002; 108 (3): 113-119. 8. KAPPELMAYER J, SIMON A, KATANA F, SZANTO A, NAGY L, KISS C, MUSZBEK L. Coagulation factor XIIIA. A flow cytometric intracellular marker in the classification of acute myeloid leukemia. Thromb Haemostas 2005; 94 (2): 454- 459. 9. BECK E, DUCKERT F, ERNEST M. The influence of fibrin stabilizing factor on the growth of fibroblasts in vitro and wound healing. Thromb Diathes Haemorrh, 1961; 6: 485-491. 10. TOIDA M, OKUMURA T, TAKAMI T. Cells containing factor XIII a and pulmonary fibrosis induced by bleomycin. J Clin Pathol 1991; 44 (3): 255-256. 11. MUSZBEK L, YEE VC, HEVESSY Z. Blood coagulation factor XIII: structure and function. Thromb Res 1999; 94: 271-305. 12. POLGAR J, HIDASI V, MUSZBEK L. Nonproteolytic activation of cellular protransglutaminase (placenta macrophage factor XIII). Biochem J, 1990; 267: 557- 560. 13. SAKATA Y, AOKI N. Crosslinking of α2 antiplasmin to fibrin by fibrin stabilizing factor. J Clin Invest 1980; 65: 290-297. 14. LEE KN, JACKSON KW, CHRISTIANSEN VJ, LEE CS, CHUN JG, MC KEE PA. Antiplasmin cleaving enzyme is a soluble form of fibroblast activation protein. Blood 2006; 107: 1397-1404. Leucemie acută megakariocotară şi mielofibroză. Consideraţii bazate pe un studio de caz ALIZ HORÁK, R. BUIGA, OLGA ŞORIŢĂU, ŞTEFANIA NEAMŢU, M. CUCUIANU Rezumat Se prezintă cazul unei fetiţe de 2,4 ani cu stenoză pulmonară şi defect de sept atrial care a dezvoltat o leucemie acuta megakariocitară evoluând cu mielofibroză, complicaţii oculare de natură trombohemoragică şi evoluţie letală. Se discută patogeneza mielofibrozei şi a rolului jucat de subunitatea A a factorului XIII al coagulării. 136
POLYSEROSITIS AND MULTISYSTEMIC DYSFUNCTION IN A CASE OF UNCLASSIFICABLE CONNECTIVE TISSUE DISEASE. UNDIFFERENTIATED VERSUS OVELAPPING (MIXED) SYNDROME ? (A CASE REPORT AND REVIEW OF THE LITERATURE) D. RĂDULESCU 1 , F. CIOVICESCU 1 , S. PRIPON 3 , C. DUNCEA 1 , ELENA BUZDUGAN 1 , ANCA CRISTEA 2 1Clinica Medicală V, UMF “Iuliu Hatieganu”, Cluj-Napoca. 2 Clinica Medicală I, Laboratorul de Imunologie, Cluj-Napoca. 3 student anul V , UMF “Iuliu Hatieganu”, Cluj-Napoca. Summary The existence of patients with signs, symptoms and certain laboratory test results suggestive of a systemic autoimmune disease but not fulfilling the classification criteria for well-defined connective-tissue diseases (CTDs) is a more and more common experience in clinical practice. The first description of these clinical entities dates back to 1980, when LeRoy proposed the term “Undifferentiated Connective Tissue Syndromes” (UCTS) to define the early stages of connective-tissue diseases that are undefined, unclassifiable or perhaps incomplete, as opposed to mixed or overlapping syndromes in which patients exhibit enough features of more than one connective tissue disease to meet the diagnosis for several at the same time. In this article we present the case of a 53 year old female admitted to Medicala V Hospital with a diagnosis of undifferentiated connective tissue disease, documented on clinical and laboratory data. Key words: undifferentiated connective tissue disease, connective tissue disease Introduction The existence of patients with signs, symptoms and certain lab test results suggestive of a systemic autoimmune disease but not fulfilling the classification criteria for well-defined connective-tissue diseases (CTDs) is a more and more common experience in clinical practice. The first description on these clinical entities dates back to 1980, when LeRoy proposed the term “Undifferentiated Connective Tissue Syndromes” (UCTS) to define the early stages of connective-tissue diseases that are undefined, unclassifiable or perhaps incomplete, as opposed to mixed or overlapping syndromes in which patients exhibit enough features of more than one connective tissue disease to meet the diagnosis for several at the same time. Thus, they “overlap” two or more diseases. That’s because many CTDs share common signs and symptoms which frequently makes the diagnosis difficult. The definition of UCTS and overlap syndromes is still under debate. 137
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Clujul Medical Revistă de Medicin
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Articole de orientare Clujul Medica
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VOICHITA SĂBĂDUŞ, D. BORZEA, DIA
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CAMELIA GEORGETA RĂCĂREANU, GABRI
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ACTUALITĂŢI ÎN PATOGENIA BOLII V
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