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Mature B-cell acute leukemia: a clinical, morphological, immunological, and cytogenetic study of nine cases
A Hammami1, W C Chan, S D Michels
1Emory University School of Medicine, Department of Pathology, Atlanta, Georgia 30322.
Insights
This study examines mature B-cell acute leukemias without FAB-L3 morphology, revealing a very aggressive clinical course. These aggressive leukemias show a poor prognosis, with limited patient remission and short survival rates.
Area of Science:
- Hematology
- Oncology
- Cell Biology
Background:
- Mature B-cell acute leukemias (B-ALL) encompass diverse subtypes.
- Non-FAB-L3 B-ALL presents unique clinical and biological characteristics.
- Understanding these specific leukemias is crucial for treatment strategies.
Purpose of the Study:
- To characterize the clinical presentation and outcomes of acute lymphoid leukemia with a mature B-cell phenotype, excluding FAB-L3 morphology.
- To identify key biological features associated with this leukemia subtype.
- To evaluate the efficacy of current treatment regimens.
Main Methods:
- Retrospective analysis of nine male patients with mature B-cell acute lymphoid leukemia (non-FAB-L3).
- Evaluation of bone marrow involvement, peripheral blood counts, extramedullary disease, and meningeal involvement.
- Immunophenotyping for terminal deoxynucleotidyl transferase (TdT) and surface immunoglobulin (monotypic).
- Cytogenetic analysis for clonal chromosomal abnormalities.
Main Results:
- All nine patients were male, median age 69, with extensive bone marrow and peripheral blood involvement.
- Six patients had extramedullary disease, and three developed meningeal involvement.
- Leukemic cells were TdT-negative; monoclonal surface immunoglobulin (lambda predominance) was observed.
- Clonal chromosomal abnormalities, often involving chromosome 8, were detected in 4/6 cases.
- Only two patients achieved complete remission with aggressive chemotherapy, one lasting only one month.
- Eight patients died within 1 week to 20 months, with a median survival of 5.5 months.
Conclusions:
- Mature B-cell acute leukemias, not of the FAB-L3 type, exhibit an aggressive clinical course.
- These leukemias are associated with poor prognosis and short survival.
- Further research into targeted therapies is warranted for this challenging subtype.
Abstract:
Nine patients who presented with acute lymphoid leukemia of mature B-cell phenotype without FAB-L3 morphology are discussed. All patients were male with a median age of 69 years. All patients had extensive bone marrow involvement at presentation with lymphoid leukemic cells in the peripheral blood. Six patients had extramedullary disease and 3 developed meningeal involvement sometime during the course of their illness. The leukemic cells were negative for terminal deoxynucleotidyl transferase in all 9 cases, and monoclonal surface immunoglobulin was demonstrated in all 8 cases evaluated with a lambda light chain predominance. Clonal chromosomal abnormalities were detected in 4 of 6 cases studied with no specific pattern identified, although abnormalities involving chromosome 8 were present in all 4 cases. Despite aggressive chemotherapy, only 2 patients achieved complete remission which was of short duration (1 month) in 1 patient. Eight patients died of their disease 1 week to 20 months after diagnosis with a median survival of 5.5 months. Mature B-cell acute leukemias that are not of the FAB-L3 type have a very aggressive clinical course and poor prognosis.
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