Contributions
Abstract: PB1771
Type: Publication Only
Background
Angioimmunoblastic T-cell lymphoma (AITL) is a rare form of non-Hodgkin’s lymphoma, characterized by generalized lymphadenopathy and frequent extra nodal sites. Cutaneous lesions of AITL are observed in 38-58% of pts and often precede lymphadenopathy.
Aims
The aim of our study is to identify the genesis of cutaneous lesions of AITL.
Methods
Clinical data of 54 patients with newly diagnosed AITL we analyzed. Diagnosis was based on standard WHO criteria. The male/female ratio was 30/24; median age was 61 (29-81) years. Histological, molecular studies of skin biopsies were performed. To evaluate T-cell clonality TCRG and TCRB gene rearrangements were PCR-amplified according to BIOMED-2 standardized protocol and analyzed by capillary electrophoresis on ABI PRISM 3130 (Applied Biosystems). Sensitivity of T-cell clonality assay was limited to 10% of clonal Т-cells of the total T lymphocytes in the sample. Gly17Val RHOA mutation was analyzed by quantitative allele-specific (qAS) TaqMan Real-Time PCR assay. The detection level of this method was 1% of mutated cells in the total cell population.
Results
Cutaneous lesions were observed in 24 (44.4%) of 54 pts, they were more common in males (75%) than females (25%). Maculopapular rash was noted in 22 (91.7%) of 24 cases. Morphological and molecular studies of skin biopsies with maculopapular rash demonstrated reactive changes. The level of polyclonal IgE was elevated in patients with maculopapular rash. Tumor cutaneous lesions were detected in 8 (14.8%) of 54 pts and were represented by "livedo reticularis" in 3 cases, focal hyperpigmentation – in 2, erythroderma – in 1, tumor node – in 1 and plaques – in 1. Tumor and reactive cutaneous lesions were presented in 6 pts, in 4 (7.4%) of 54 cases they were noted at the same time. The type of cutaneous elements changed during of the disease in 3 (5.6%) pts.
Conclusion
Maculopapular rash is the most frequent cutaneous manifestation of AITL and it has a reactive genesis. Tumor lesion of the skin is rarely and can manifest by various cutaneous elements. In some cases AITL tumor and reactive cutaneous lesions can be observed simultaneously or sequentially.
Session topic: 21. Aggressive Non-Hodgkin lymphoma - Clinical
Keyword(s): Angioimmunoblastic T-cell lymphoma, Clonality, Immunoglobulin, TCR
Abstract: PB1771
Type: Publication Only
Background
Angioimmunoblastic T-cell lymphoma (AITL) is a rare form of non-Hodgkin’s lymphoma, characterized by generalized lymphadenopathy and frequent extra nodal sites. Cutaneous lesions of AITL are observed in 38-58% of pts and often precede lymphadenopathy.
Aims
The aim of our study is to identify the genesis of cutaneous lesions of AITL.
Methods
Clinical data of 54 patients with newly diagnosed AITL we analyzed. Diagnosis was based on standard WHO criteria. The male/female ratio was 30/24; median age was 61 (29-81) years. Histological, molecular studies of skin biopsies were performed. To evaluate T-cell clonality TCRG and TCRB gene rearrangements were PCR-amplified according to BIOMED-2 standardized protocol and analyzed by capillary electrophoresis on ABI PRISM 3130 (Applied Biosystems). Sensitivity of T-cell clonality assay was limited to 10% of clonal Т-cells of the total T lymphocytes in the sample. Gly17Val RHOA mutation was analyzed by quantitative allele-specific (qAS) TaqMan Real-Time PCR assay. The detection level of this method was 1% of mutated cells in the total cell population.
Results
Cutaneous lesions were observed in 24 (44.4%) of 54 pts, they were more common in males (75%) than females (25%). Maculopapular rash was noted in 22 (91.7%) of 24 cases. Morphological and molecular studies of skin biopsies with maculopapular rash demonstrated reactive changes. The level of polyclonal IgE was elevated in patients with maculopapular rash. Tumor cutaneous lesions were detected in 8 (14.8%) of 54 pts and were represented by "livedo reticularis" in 3 cases, focal hyperpigmentation – in 2, erythroderma – in 1, tumor node – in 1 and plaques – in 1. Tumor and reactive cutaneous lesions were presented in 6 pts, in 4 (7.4%) of 54 cases they were noted at the same time. The type of cutaneous elements changed during of the disease in 3 (5.6%) pts.
Conclusion
Maculopapular rash is the most frequent cutaneous manifestation of AITL and it has a reactive genesis. Tumor lesion of the skin is rarely and can manifest by various cutaneous elements. In some cases AITL tumor and reactive cutaneous lesions can be observed simultaneously or sequentially.
Session topic: 21. Aggressive Non-Hodgkin lymphoma - Clinical
Keyword(s): Angioimmunoblastic T-cell lymphoma, Clonality, Immunoglobulin, TCR