A Case with Disseminated Macrophage-related Histiocytic Sarcoma Diagnosed by Positive Histiocytic Markers / 대한혈액학회지

ABSTRACT

Malignant histiocytosis (MH) is characterized by an invasive, progressive proliferation of neoplastic histiocytes associated with jaundice, lymphadenopathy, refractory anemia, leukopenia, and often hepatic and splenic enlargement. As lymphoblastic leukemia and lymphoma are regarded as neoplasms of lymphoid cells, MH is thought to represent a malignant transformation of the macrophage and dendritic cells. A classification of malignant histiocytic disorders was oriented by cell lineage in the Histiocyte Society's 1987 version. So dendritic cell-related histiocytic sarcoma (localized or disseminated) and macrophage-related histiocytic sarcoma (localized or disseminated) are the recommended nosology. To establish the correct diagnosis, the major challenge seems to distinguish lymphoid from histiocytic cells. M-CSF receptor, lysozyme, Ki-M8, and S-100 protein, etc are useful markers for histiocytes and T-cell and B-cell lineage markers, such as CD3, CD20, and CD79, etc, for lymphocytes. We have experienced a patient with disseminated histiocytic sarcoma diagnosed by positive istiocytic markers, such as lysozyme, S-100 protein, and CD68.