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Leukaemic intermediate lymphocytic lymphomas: analysis of twelve cases diagnosed by morphology
A Criel1, J Billiet, E Vandenberghe
1Department of Haematology, A.Z. St. Jan, Brugge, Belgium.
Insights
Leukaemic intermediate diffuse lymphocytic lymphoma (ILL) is a distinct B-cell malignancy. This aggressive lymphoma shows characteristic morphology and cytogenetics, often presenting with advanced disease and poor response to chemotherapy.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Leukaemic intermediate diffuse lymphocytic lymphoma (ILL) is a rare B-cell neoplasm.
- Morphological analysis is crucial for differentiating ILL from other B-cell disorders like chronic lymphocytic leukaemia (CLL).
Purpose of the Study:
- To characterize the morphological, immunophenotypic, and cytogenetic features of twelve ILL cases.
- To evaluate the clinical presentation and disease course of patients with ILL.
Main Methods:
- Morphological assessment of leukaemic cells.
- Immunophenotyping using flow cytometry (CD5, FMC7, CD10) and immunoglobulin staining (mu, chi, lambda).
- Cytogenetic analysis (karyotyping) and DNA analysis for ploidy.
Main Results:
- All twelve cases exhibited typical ILL morphology, distinct from CLL.
- Immunophenotyping revealed B-cell derivation with strong Ig staining, CD5+, FMC7+, and CD10-.
- Eight patients had t(11;14)(q13;q32) translocation; hypoploidy was common.
- Patients presented with advanced, widespread disease and low absolute lymphocytosis.
- The disease course was aggressive and unresponsive to conventional chemotherapy.
Conclusions:
- Leukaemic intermediate diffuse lymphocytic lymphoma is a distinct clinicopathological entity.
- The t(11;14) translocation and hypoploidy are significant findings in ILL.
- ILL follows an aggressive clinical course with limited therapeutic options.
Abstract:
Twelve cases of leukaemic intermediate diffuse lymphocytic lymphoma (ILL), diagnosed by morphology, were analysed. The morphology of the ILL cells was so typical that it allowed ready distinction from chronic lymphocytic leukaemia (CLL) and other related B cell disorders. All cases were of B derivation, had strong mu and chi or lambda immunoglobulin (Ig) staining, were CD5 and FMC7 positive and CD10 negative. Cytogenetic abnormalities were found in 8 patients all having t(11;14)(q13;q32). DNA analysis revealed a relatively high incidence of hypoploidy. At diagnosis all the patients (9 males, 5 females; median age 68) had a low degree of absolute lymphocytosis but the disease was advanced and mostly widespread. The course of the disease appears to be aggressive and incurable with conventional combination chemotherapy.
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