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Angiocentric immunoproliferative lesion (lymphomatoid granulomatosis). A cytogenetic, immunophenotypic, and genotypic
L R Donner1, S Dobin, D Harrington
1Department of Pathology, Scott and White Memorial Hospital, Temple, TX 76508.
Insights
A cytogenetically abnormal clone was found in a patient with angiocentric immunoproliferative lesion (AIL). This discovery supports the idea that AIL is a neoplastic (cancerous) process.
Area of Science:
- Hematology
- Oncology
- Genetics
Background:
- Angiocentric immunoproliferative lesion (AIL), also known as lymphomatoid granulomatosis, is a rare condition.
- Understanding the underlying biological mechanisms of AIL is crucial for diagnosis and treatment.
Observation:
- A 23-year-old woman with a three-year history of AIL presented with involvement of skin, lungs, spleen, liver, and bone marrow.
- Atypical lymphohistiocytic infiltrates were observed in these organs.
Findings:
- Cytogenetic analysis of the spleen revealed an abnormal clone: 46,XX,t(1;6)(p35;q23),t(1;9;19)(q23;p24;q13).
- Immunophenotypic study showed 46% of splenic cells had a helper/inducer T-cell phenotype.
- Despite the T-cell phenotype, DNA analysis did not reveal clonal rearrangements in T-cell receptor or immunoglobulin genes.
Implications:
- The identification of a cytogenetically abnormal clone provides strong evidence that AIL is a neoplastic process.
- This finding may lead to new diagnostic criteria and therapeutic strategies for AIL.
- Further research into the genetic basis of AIL is warranted.
Abstract:
We report the occurrence of a cytogenetically abnormal clone 46,XX,t(1;6)(p35;q23),t(1;9;19)(q23;p24;q13) in the spleen of a 23-year-old woman with a three-year history of angiocentric immunoproliferative lesion (AIL) (lymphomatoid granulomatosis). The skin, lungs, spleen, liver and, focally, bone marrow were involved by atypical lymphohistiocytic infiltrates. Immunophenotypic study of the spleen showed that 46% of the cells displayed a helper/inducer T-cell phenotype. However, analysis of DNA isolated from the spleen failed to show clonal T-cell receptor beta-chain gene, T-cell receptor gamma-chain gene, or immunoglobulin heavy chain gene and light chain gene rearrangements. The finding of a cytogenetically abnormal clone supports the concept that angiocentric immunoproliferative lesion is a neoplastic process.