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Vol.48, No.6, 2014
Current Issue
Archive (2009~
Archive-the Korean Journal of Pathology(1967~2008)
Archive-the Korean Journal of Cytopathology(1990~2008)
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  Vol.43, No.5:482-488, October 2009
Case reports
Extramedullary B Lymphoblastic Crisis of CML, Presenting as a Leptomeningeal Tumor - A Case Report

Hoiseon Jeong, Bongkyung Shin, Sook Young Bae, Sangho Lee. Youngjune Ryu, Hankyeom Kim, Insun Kim

Department of Pathology, Korea University Guro Hospita

We report here on a rare case of a patient who presented with an extramedullary B lymphoblastic
crisis as an initial manifestation of chronic myelogenous leukemia (CML). A 71-year-old
man visited the emergency room due to suddenly developed dysarthria and right side weakness.
Emergency craniotomy was done under the presumptive diagnosis of subdural hemorrhage.
During the operation, a poorly demarcated firm mass was identified in the leptomeningeal
space. Microscopically, the majority of the tumor was composed of monotonous immature
cells with blast morphology, and these cells were immunoreactive for TdT, CD34, CD10 and
CD20, indicating the precursor B-cell phenotype. The peripheral area of the tumor consisted
of myeloid cells in various stages of maturation, and these cells were reactive for myeloperoxidase,
chloroacetate esterase, CD43 and CD15. FISH analysis using the LSI bcr-abl dual
color probe showed gene fusion signals in both the B-lymphoblasts and myeloid cells. The
peripheral blood and bone marrow findings were consistent with CML with no evidence of a
blast crisis. Cytogenetic study of the bone marrow demonstrated the 46, XY, t(9;22)(q34;q11)
chromosome. A diagnosis of extramedullary B lymphoblastic blast crisis in a patient with Philadelphia
chromosome-positive CML was made. Despite treatment, the patient died 3 months
after he was diagnosed.
Key Words : Blast crisis; Chronic myelogenous leukemia; Fluorescent in situ hybridization; Philadelphia chromosome; Leptomeninges

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