Myeloid sarcoma (myelosarcoma): classification, diagnosis,biologic manifestation and prognosis
Myelosarkóm – klasifikácia, diagnostika, biologickámanifestácia a prognóza
Myelosarkóm je zriedkavý extramedulárny alebo kostný zhubný nádor, ktorý vzniká tumoriformnounádorovou proliferáciou myeloidných buniek na úrovni rôzneho štádia ich diferenciácie. Vyskytuje saobyčajne v súvislosti s iným klonálnym myeloproliferatívnym ochorením kostnej drene, a to najčastejšies procesom zo spektra akútnej myeloblastovej leukémie. Extramedulárna manifestácia procesumôže byť pritom primárna („de novo“), alebo sekundárna. Jeho diagnózu určuje komplex morfologických,imunohistochemických, cytogenetických a klinických parametrov. Tieto parametre však vykazujúznačnú individuálnu heterogenitu, čo negatívne spolu s nedokonalými princípmi jeho gradinguovplyvňuje jeho správnu diagnostiku a voľbu adekvátnej terapie. V práci sa predkladá integráciasúčasných poznatkov o myelosarkóme, vrátane jeho definície, typizácie, gradingu, biologickej manifestáciea odhadu prognózy ochorenia.
Klíčová slova:
myeloproliferatívne ochorenie, myelosarkóm, akútna myeloblastová leukémia
Authors:
J. Hrnková; P. Szépe; L. Plank
Authors‘ workplace:
Ústav patologickej anatómie a Konzultačné centrum bioptickej diagnostiky ochorení krvotvorby JLF UK a MFN v Martine, Slovenská republika
Published in:
Transfuze Hematol. dnes,, 2004, No. 3, p. 99-105.
Category:
Overview
Myeloid sarcoma (myelosarcoma) represents a rare malignant tumour arising by extramedullary orbone tumour mass forming neoplastic proliferation of myeloid cells at different stages of their differentiation.It is usually associated with some clonal myeloproliferative disease – most often with someof acute myeloblastic leukemia types – and the extramedullary manifestation might be primary („denovo“) or secondary. The diagnosis of myelosarcoma is defined by a complex of morphologic, immunohistochemical,cytogenetical, and clinical parameters. However, all these parameters are individuallyheterogenous what might negatively influence the correct diagnosis and appropriate treatment selection.The paper includes an integration of recent information about the definition, typing, grading,biologic manifestation and prognosis of myeloid sarcoma.
Key words:
myeloproliferative disease, myeloid sarcoma (myelosarcoma), acute myeloblastic leukemia
Labels
Haematology Internal medicine Clinical oncologyArticle was published in
Transfusion and Haematology Today
2004 Issue 3
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