Extreme Peripheral Blood Plasmacytosis Mimicking Plasma Cell Leukemia as a Presenting Feature of Angioimmunoblastic T-Cell Lymphoma (AITL)

Abstract Peripheral T-cell lymphoma (PTCL) is often challenging to diagnose and classify. Gene expression profiling was performed on 144 cases of PTCL and natural killer cell lymphoma and robust molecular classifiers were constructed for angioimmunoblastic T-cell lymphoma (AITL), anaplastic lymphoma kinase-positive (ALK+) anaplastic large-cell lymphoma (ALCL), and adult T-cell leukemia/lymphoma. PTCL-unclassifiable was molecularly heterogeneous, but we were able to identify a molecular subgroup with features of cytotoxic T lymphocytes and a poor survival compared with the remaining PTCL–not otherwise specified cases. Many of the pathologic features and substantial components of the molecular signature of AITL are contributed by the follicular dendritic cells, B-cell, and other stromal components. The expression of Th17-associated molecules in ALK+ ALCL was noted and may represent aberrant activation of Th17-cell differentiation by abnormal cytokine secretion. Adult T-cell leukemia/lymphoma has a homogeneous molecular signature demonstrating high expression of human T-lymphotropic virus type 1–induced genes. These classifiers reflect the biology of the tumor cells as well as their microenvironment. We also constructed a molecular prognosticator for AITL that appears to be largely related to the microenvironmental signature, and the high expression of 2 immunosuppressive signatures are associated with poor outcome. Oncogenic pathways and tumor-host interactions also were identified, and these findings may lead to better therapies and outcome in the future.

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Molecular diagnosis angioimmunoblastic T-cell lymphoma

Terapevticheskii arkhiv ◽

10.26442/00403660.2019.07.000330 ◽

2019 ◽

Vol 91 (7) ◽

pp. 63-69

Author(s):

N G Chernova ◽

Y V Sidorova ◽

S Y Smirnova ◽

N V Ryzhikova ◽

E E Nikulina ◽

...

Keyword(s):

Bone Marrow ◽

Lymph Node ◽

T Cell ◽

Peripheral Blood ◽

Cell Lymphoma ◽

Cell Receptor ◽

T Cell Lymphoma ◽

Tissue Samples ◽

Angioimmunoblastic T Cell Lymphoma ◽

Allele Specific

Aim: to determine molecular diagnostics routine for different tissue samples in angioimmunoblastic T-cell lymphoma. Materials and methods. Molecular studies were performed for 84 primary AITL patients. The median age was 61 year (29-81); the male to female ratio was 48/36. T-cell and B-cell clonality was assessed by GeneScan analysis of rearranged T-cell receptor (TCRG, TCRB) and immunoglobulin heavy chain genes. For the quantitative determination of cells with RHOA G17V mutation real - time polymerase chain reaction (PCR) with allele - specific LNA modified primers was used. Results. In lymph nodes rearrangements of T-cell receptor genes were determined in 76 (90.5%) of 84 patients and were absent in 8 (9.5%) cases. Identification of the same clonal products of the TCRG and TCRB genes in the lymph node and in peripheral blood and/or bone marrow indicated the prevalence of the tumor process and was observed in 64.7% of patients. Clonal products in peripheral blood and/or bone marrow different from those in the lymph node indicated reactive cytotoxic lymphocyte population and were noted in 58.8% of AITL cases. Simultaneous detection of T- and B-cell clonality in the lymph node was observed in 20 (24.7%) of 81 patients. Cells with RHOA G17V mutation were detected in lymph node in 45 (54.9%) of 82 patients. The use of allele - specific PCR with LNA modified primers revealed presence of the tumor cells in peripheral blood in 100% and in bone marrow in 93.9% of patients with G17V RHOA mutation in the lymph nodes. Conclusion. The validity of different molecular assays performed on certain tissue samples for the diagnosis of angioimmunoblastic T-cell lymphoma has been evaluated. Quantitative allele - specific PCR assay for RHOA G17V mutation based on LNA modified primers possesses sufficient sensitivity for tumor process prevalence evaluation and minimal residual disease monitoring.

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Angioimmunoblastic T-Cell Lymphoma Partially Obscured by an Epstein-Barr Virus–Negative Clonal Plasma Cell Proliferation

Journal of Clinical Oncology ◽

10.1200/jco.2012.43.3797 ◽

2013 ◽

Vol 31 (2) ◽

pp. e28-e30 ◽

Cited By ~ 17

Author(s):

Alison R. Huppmann ◽

Michele R. Roullet ◽

Mark Raffeld ◽

Elaine S. Jaffe

Keyword(s):

Cell Proliferation ◽

T Cell ◽

Plasma Cell ◽

Epstein Barr Virus ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Angioimmunoblastic T Cell Lymphoma ◽

Barr Virus ◽

Epstein Barr

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Expression of multiple cytokines and chemokine receptor 4 in angioimmunoblastic T-cell lymphoma with abundant plasma cell infiltration involving the skin

British Journal of Haematology ◽

10.1111/bjh.14092 ◽

2016 ◽

Vol 177 (4) ◽

pp. 656-659

Author(s):

Shoko Nakayama ◽

Taiji Yokote ◽

Nobuya Hiraoka ◽

Uta Nishiwaki ◽

Toshiaki Hanafusa ◽

...

Keyword(s):

T Cell ◽

Plasma Cell ◽

Chemokine Receptor ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Cell Infiltration ◽

Angioimmunoblastic T Cell Lymphoma ◽

Plasma Cell Infiltration ◽

Chemokine Receptor 4

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The Early Diagnostic Dilemma in Angioimmunoblastic T Cell Lymphoma with Excessive Plasma Cells Proliferation

Case Reports in Medicine ◽

10.1155/2021/9951122 ◽

2021 ◽

Vol 2021 ◽

pp. 1-8

Author(s):

Chunyan Wang ◽

Xia Mao ◽

Songya Liu ◽

Cheng He ◽

Ying Wang ◽

...

Keyword(s):

Cell Proliferation ◽

T Cell ◽

Plasma Cell ◽

Plasma Cells ◽

Cell Lymphoma ◽

Clinical Manifestations ◽

Underlying Disease ◽

T Cell Lymphoma ◽

Diagnostic Dilemma ◽

Angioimmunoblastic T Cell Lymphoma

Background. Angioimmunoblastic T cell lymphoma (AITL) is an aggressive Epstein–Barr virus-associated T cell lymphoma. Clinical syndromes of AITL are not confined to fever and lymphadenopathy, and patients may initially present with polyclonal plasma cell proliferation, which may obscure the underlying disease of AITL, delaying diagnosis. Case Presentation. Here, we report two AITL patients with excessive plasma cell proliferation in the bone marrow, peripheral blood, and ascites even mimicking plasma cell leukemia. Both of them had poor endings. Conclusions. Our report emphasizes the complexity of the clinical manifestations of AITL, which aims to increase the alertness of physicians and improve the rate of early diagnosis. Integrated diagnostic approaches such as histopathology, flow cytometry, cytogenetics, and molecular biology are essential for accurate diagnosis and precise therapy.

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