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Cell markers in lymphoma syndrome leukemia in children: a pilot study
Insights
Children with lymphoma syndrome (LySLk) and acute lymphocytic leukemia (ALL) have a poor prognosis. Surface marker analysis revealed that cALLA-negative lymphoblasts are common in LySLk, suggesting a distinct ALL subgroup.
Area of Science:
- Pediatric Oncology
- Hematology
- Immunology
Background:
- Acute lymphocytic leukemia (ALL) in children can present with a "lymphoma syndrome" (LySLk).
- LySLk is associated with a poor prognosis when treated with standard ALL chemotherapy.
- Identifying distinct ALL subgroups is crucial for tailored treatment strategies.
Purpose of the Study:
- To investigate the prognostic significance of surface markers on leukemic cells in children with LySLk.
- To determine if LySLk represents a distinct subgroup of ALL based on immunophenotype.
Main Methods:
- Retrospective review of 21 pediatric patients with LySLk.
- Analysis of leukemic cell surface markers: E-rosettes (E), surface immunoglobulin (Slg), and common ALL antigen (cALLA).
- Correlation of surface marker status with patient outcomes (no evidence of disease - NED).
Main Results:
- The majority of LySLk patients had cALLA-negative lymphoblasts (15/18 evaluable cases).
- Four out of four cALLA-positive patients achieved NED, compared to 2 out of 16 cALLA-negative patients.
- E-negative, cALLA-positive patients may represent a distinct subgroup with a potentially better prognosis.
Conclusions:
- Children with LySLk and cALLA-negative lymphoblasts appear to constitute the majority of this ALL subgroup.
- The presence of E-negative, cALLA-positive surface markers in LySLk patients suggests a potentially better prognosis.
- Therapeutic strategies for LySLk patients should consider immunophenotype alongside standard prognostic factors.
Abstract:
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.