Cell markers in lymphoma syndrome leukemia in children: a pilot study

Children with acute lymphocytic leukemia (ALL) who have a "lymphoma syndrome" (LySLk) defined by the presence of at least three of the following criteria: a) Hg greater than 10 g/dl, b( lymph nodes greater than 3 cm, c) spleen below umbilicus, d) liver below umbilicus, and e) mediastinal mass, appear to represent a subgroup of ALL. These children have a poor prognosis for survival when treated with standard chemotherapy for ALL. We performed a retrospective review of 21 patients at Children's Memorial Hospital with LySLk diagnosed from Jan 24, 1977 to July 8, 1981 and of the surface markers on their leukemic cells at diagnosis. Surface markers identified included E-rosettes (E), surface immunoglobulin (Slg), and common ALL antigen (cALLA). Four patients were cALLA positive and E-rosette negative; nine patients were E-negative, cALLA negative; six patients were E positive, cALLA negative. In two patients E-rosettes could not be accurately determined because of a low percentage of lymphoblasts in the samples studied. Follow-up data on cALLA-positive and cALLA-negative patients revealed 4/4 cALLA-positive patients with no evidence of disease (NED) at 22 + to 45 + months from diagnosis and 2/16 cALLA-negative patients NED at 19 + and 57 + months. Thus it appears that the majority of children with LySLk have lymphoblasts which are cALLA negative. Patients who meet clinical criteria for LySLk but whose surface markers are E negative, cALLA positive may have a better prognosis and may represent a separate subgroup of patients with ALL and, therefore, should be given therapy appropriate for their prognostic classification by more standard criteria, such as white blood count, age, and sex.