Recent advances in immunology have clarified the cellular origin of he
matopoietic neoplasms. Blast cells with a CD7+ CD4- CD8- phenotype are
demonstrated to originate from malignant pluripotent hematopoietic st
em cells. In this article, the authors describe three rare cases, desi
gnated as a lymphoma type of CD7+ stem cell leukemia/lymphoma, with cl
inical features described below. All three patients were admitted with
non-Hodgkin lymphoma with a 2-month to 4-month history of lymphadenop
athy. Histologic examination of lymph nodes showed lymphoblastic lymph
oma (LBL) in all patients. Bone marrow blast cells had an immunophenot
ype consistent with CD7+ CD4- CD8- acute leukemia, although abnormal c
ells were not observed in the peripheral blood during the course of th
e disease. One patient had a recurrence in the bone marrow, with myelo
peroxidase-positive blast cells expressing myeloid differentiation ant
igens. Chromosomal analysis detected a common abnormal karyotype initi
ally and at relapse. Furthermore, the same T-cell receptor gene rearra
ngement was found initially and at relapse, suggesting that these blas
t cells originated from the same pluripotent leukemic clone. Additiona
l studies on more patients are required to determine the clinical sign
ificance of this group, including the difference from CD7+ stem cell l
eukemia/lymphoma with circulating blast cells (leukemic type) or LBL.