Acute undifferentiated leukemia with CD7+ and CD13+ immunophenotype. Lack of molecular lineage commitment and association with poor prognostic features.
The authors studied six adult patients with acute leukemia with these unusual characteristics: unclassifiable morphology and undifferentiated cytochemistry by French-American-British (FAB) criteria; concurrent expression of CD13 (and CD33) myeloid and early T-cell CD7 immune markers; no evidence of T-cell lineage commitment as determined by T-cell receptor beta (beta), gamma (gamma), and delta (delta) chain gene rearrangement study and cytoplasmic CD3 epsilon expression; and no evidence of myeloid cell lineage commitment, as shown by absent myeloid-specific c-fms proto-oncogene expression and negative myeloperoxidase ultrastructural staining (one case). Clinically, these diagnostic features matched with a poor prognosis, being associated with refractoriness to treatment, relapse and progression of disease, antecedent hematologic abnormality, and other malignancy. These cases may represent a distinct stem cell leukemia syndrome deserving immediate recognition and a nonconventional chemotherapeutic approach.
Divisione di Ematologia, Ospedali Riuniti, Bergamo, Italy.
R
R
Bassan
A
A
Biondi
S
S
Benvestito
M L
ML
Tini
M
M
Abbate
P
P
Viero
T
T
Barbui
A
A
Rambaldi
D000208:Acute Disease; D000328:Adult; D015703:Antigens, CD / Q000032:analysis*; D019011:Antigens, CD7; D000943:Antigens, Differentiation / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis*; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis*; D018826:CD13 Antigens; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D016130:Immunophenotyping; D007938:Leukemia / Q000145:classification* / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009710:Nucleotide Mapping; D011379:Prognosis; D000090063:Proto-Oncogene Mas; D012334:RNA, Neoplasm / Q000032:analysis
D015703:Antigens, CD; D019011:Antigens, CD7; D000943:Antigens, Differentiation; D015214:Antigens, Differentiation, Myelomonocytic; D000945:Antigens, Differentiation, T-Lymphocyte; D004273:DNA, Neoplasm; C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D012334:RNA, Neoplasm; D018826:CD13 Antigens