T-cell blast crisis in chronic myelogenous leukemia. Immunophenotypic and molecular biologic findings.
T-cell blast crisis in chronic myelogenous leukemia is rare. We examined three patients (ages 35 to 72 years) in whom T-cell blast crisis developed 11 to 36 months (mean, 25 months) after diagnosis of chronic myelogenous leukemia and who died 4 to 12 months (mean, 7 months) thereafter. Two patients had diffuse lymphadenopathy, and the third had marked lymphocytosis (white blood cell count 217,000/microL, with 90% circulating blasts). In all three patients, neoplastic cells had the appearance of lymphoblasts and were immunoreactive for T-cell markers by immunohistochemical or flow cytometric analysis or both. Molecular diagnostic studies revealed the presence of a bcr-abl oncogene rearrangement in all three cases, but none exhibited a clonal T-cell receptor delta, beta, or gamma chain gene rearrangement. One case exhibited deletion of the J delta 1 region of both delta chain genes. The significance of these findings is discussed, and they are compared with those of other reported cases of T-cell blast crisis in chronic myelogenous leukemia.
Dorfman
D M
DM
Department of Pathology, Brigham and Women's Hospital, Boston, MA 02115, USA.
Longtine
J A
JA
Fox
E A
EA
Weinberg
D S
DS
Pinkus
G S
GS
D000328:Adult; D000368:Aged; D001706:Biopsy; D001752:Blast Crisis / Q000209:etiology / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D005434:Flow Cytometry; D016044:Fusion Proteins, bcr-abl / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000209:etiology / Q000473:pathology*; D008198:Lymph Nodes / Q000737:chemistry / Q000473:pathology; D008213:Lymphocyte Activation; D008297:Male; D013601:T-Lymphocytes / Q000473:pathology*
D016044:Fusion Proteins, bcr-abl