Cutaneous lesions of angioimmunoblastic T‐cell lymphoma: Clinical, pathological, and immunophenotypic features

Cutaneous T cell lymphomas represent a heterogenous group of lymphoproliferative disorders defined by clonal proliferation of T cells present in the skin. The latest WHO classification in 2016 and WHO-EORTC classification in 2018 has updated the classification of these entities based on the molecular profile. Research in the field of molecular genetics of CTCL has allowed a better understanding of the biology of these tumors and has helped to identify potential targets for therapy that can be tailored to individual patients. In this review, we discuss the latest developments in the molecular profile of CTCLs including biomarkers for diagnosis, prognosis, and potential therapeutic targets. We have also touched upon the utility of various molecular diagnostic modalities. For the purpose of this review, we researched papers in PubMed indexed journals in English literature published in the past 20 years using keywords CTCL, mycosis fungoides, molecular profile, molecular diagnosis, whole genome profile, genomic landscape, TCR clonality.

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“…Amongst these, AITL frequently involves skin in about 50% cases (88). TCR rearrangement was found in 82% cases (89).…”

Section: Cuatneous Lymphomas With T Follicular Helper (Tfh) Phenotypementioning

confidence: 99%

An Update on Molecular Biology of Cutaneous T Cell Lymphoma

Walia

Yeung

2020

Front. Oncol.

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“…1 ). Other typical clinical manifestation of AITL are cutaneous lesions also called skin rash in 20–50% of the patients and accumulation of abdominal ascites 5 , 10 – 13 . These patients often test positive for autoimmunity including detection of rheumatoid factor, anti-smooth muscle, and nuclear autoantibodies 14 .…”

Section: Angioimmunoblastic T-cell Lymphoma: a Challenging Complex Mamentioning

confidence: 99%

New preclinical models for angioimmunoblastic T-cell lymphoma: filling the GAP

et al. 2020

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Mouse models are essential to study and comprehend normal and malignant hematopoiesis. The ideal preclinical model should mimic closely the human malignancy. This means that these mice should recapitulate the clinical behavior of the human diseases such as cancer and therapeutic responses with high reproducibility. In addition, the genetic mutational status, the cell phenotype, the microenvironment of the tumor and the time until tumor development occurs, should be mimicked in a preclinical model. This has been particularly challenging for human angioimmunoblastic lymphoma (AITL), one of the most prominent forms of peripheral T-cell lymphomas. A complex network of interactions between AITL tumor cells and the various cells of the tumor microenvironment has impeded the study of AITL pathogenesis in vitro. Very recently, new mouse models that recapitulate faithfully the major features of human AITL disease have been developed. Here, we provide a summary of the pathology, the transcriptional profile and genetic and immune-phenotypic features of human AITL. In addition, we give an overview of preclinical models that recapitulate more or less faithfully human AITL characteristics and pathology. These recently engineered mouse models were essential in the evaluation of novel therapeutic agents for possible treatment of AITL, a malignancy in urgent need of new treatment options. Angioimmunoblastic T-cell lymphoma: a challenging complex malignancy Clinical aspects Angioimmunoblastic T-cell lymphoma (AITL) belongs since 2016 after revision of the world health organization (WHO) classification to the nodal T-cell lymphomas with follicular helper T-cell (Tfh) phenotype 1. AITL is a rare, aggressive lymphoma with little treatment options. Overall survival is generally poor with a five-year median survival rate of 32% 2,3. It represents only 1-2% of the non-Hodgkin's lymphomas. However, AITL appears to be the most prevalent PTCL, representing up to 35% of the noncutaneous peripheral T-cell lymphomas 2,4-6. AITL pathology develops late in life with a median age at onset of 59-65 years and patients display a generalized lymphoadenopathy 7,8. Seventy percent of the patients have bone marrow involvement and unfortunately early stage detection of AITL is very uncommon (10%) 9. Disease symptoms resemble closely an infection inducing immunologic hyper-activation (fever, rash, loss of weight, hemolytic anemia 2,5) (Fig. 1). AITL has particular clinical and pathological features, associated with splenomegaly, hepatomegaly and enlarged lymph nodes showing effacement of normal architecture and appearance of endothelial venules 3 (Fig. 1). Other typical clinical manifestation of AITL are cutaneous lesions also called skin rash in 20-50% of the patients and accumulation of abdominal ascites 5,10-13. These patients often test positive for autoimmunity including detection of rheumatoid factor, anti-smooth muscle, and nuclear autoantibodies 14. They also present a general increase in immunoglobulins called hypergammaglobulemia (30%) 7,12 .

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“…The skin involvement by AITL is polymorphic and often non-specific, commonly consisting of maculopapular eruption that may mimic drug reaction or viral exanthema [6,16,17]. In general, there are three main categories of skin findings in AITL macular, papular, plaque-like-nodular lesions [15], or mixed features of those above [18]. In a recent retrospective analysis, 48.8% of AITL patients had skin involvement, most frequently in the form of a non-specific rash (57.1%), followed by papular (23.8%), erythrodermic (16.7%), nodular (9.5%) and petechial/ purpuric (7.1%) efflorescents [17].…”

Section: Discussionmentioning

confidence: 99%

Fever, rash, and eosinophilia – early signs of angioimmunoblastic T-cell lymphoma

Wawrzycki

Prystupa

Szumiło

et al. 2021

Ann Agric Environ Med.

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Angioimmunoblastic T-cell lymphoma (AITL) is an uncommon lymphoma of elderly adults with a poor prognosis. AITL patients show systemic symptoms, lymphadenopathy, and not infrequently, skin rash with various dysimmune phenomena rashes. The case is presented of a 68-year-old male with skin rash, lymphadenopathy and hypereosinophilia who, after investigations, was diagnosed with AITL. Despite the treatment used, the patient's condition gradually deteriorated and died due to heart and kidney failure. The diagnosis of AITL is often established only after several weeks or months because of transient physical findings, non-specific symptoms, and a broad range of serologic or radiologic abnormalities. Some patients with AITL experience non-specific dermatitis and eosinophilia. The presented case should raise awareness of the presentations of AITL which is important for physicians to reach an accurate diagnosis.

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Cutaneous lesions of angioimmunoblastic T‐cell lymphoma: Clinical, pathological, and immunophenotypic features

Cited by 22 publications

References 37 publications

An Update on Molecular Biology of Cutaneous T Cell Lymphoma

An Update on Molecular Biology of Cutaneous T Cell Lymphoma

New preclinical models for angioimmunoblastic T-cell lymphoma: filling the GAP

Fever, rash, and eosinophilia – early signs of angioimmunoblastic T-cell lymphoma

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