Unmasking a T cell lymphoma: Folliculotropic mycosis fungoides with a gamma-delta phenotype

Tran, Jessica; Vega, Francisco; Duvic, Madeleine

doi:10.1016/j.jdcr.2020.09.012

Cited by 4 publications

(4 citation statements)

References 3 publications

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“…16 In their landmark paper, Rodriguez-Pinilla et al 17 noted that there is no apparent detriment in survival in patients with gamma/delta-positive mycosis fungoides. 13,[17][18][19][20][21][22][23][24][25] Our report is one of few studies to make use of rich case report literature in dermatology and dermatopathology as a means to collect a wide range of cases, particularly when collation of slide materials for such a broad array of patients over time and space would not be possible. Two major limitations of this study are the impact of publication bias and lack of central review.…”

Section: Discussionmentioning

confidence: 99%

The significance of epidermal involvement in primary cutaneous gamma/delta (γδ) T‐cell lymphoma: A systematic review and meta‐analysis

2021

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Background: Primary cutaneous gamma/delta (γδ) T-cell lymphoma (PCGDTCL) is a rare, aggressive peripheral T-cell lymphoma. There is evidence that patients with epidermotropic PCGDTCL may have an improved prognosis compared with those with only dermal and/or subcutaneous involvement.Methods: Systematic review of the literature and application of inclusion criteria yielded 48 manuscripts detailing the cases of 104 patients.Results: Of the 104 patients, 57 were male (51.4%) and 47 were female (48.5%) Based on provided histopathologic descriptions, 57 cases (54.8%) had no epidermotropism, 47 cases (45.2%) patients demonstrated any degree of epidermotropism, and 25 cases were predominantly epidermotropic (25/104, 24%).Five-year overall survivals for patients with no epidermotropism, any epidermotropism, and predominantly epidermotropic presentation were 32.8%, 28.9%, and 40.0%, respectively (p = 0.40). The most commonly performed immunohistochemical markers were CD3, CD4, CD8, CD5, CD7, CD30, CD56, TCR beta, TCR γ, and TCR δ. There was no statistically significant difference in immunophenotype between groups. Lesion morphology was described in the majority of cases (85/104, 80.9%); most cases presented as a combination of nodules, plaques, and tumors (77.4%). Several cases had more atypical presentations, including "mycosisfungoides-like" and ulcerated. Conclusion:In PCGDTCL, neither epidermotropism nor predominantly epidermotropic phenotype predict a better prognosis. In addition, the case report literature in dermatology and dermatopathology is rich and highly valuable. K E Y W O R D S case report, case series, cutaneous T-cell lymphoma, primary cutaneous gamma/delta T-cell lymphoma, review article 1 | INTRODUCTION Primary cutaneous gamma/delta (γδ) T-cell lymphoma (PCGDTCL) is a rare, aggressive peripheral T-cell lymphoma characterized as a subcutaneous lymphoma composed of neoplastic gamma/delta T-cells. PCGDTCL typically presents with ulcerated nodules or tumors on the extremities. Mortality from this lymphoma is relatively high, approaching 89% at 5 years. 1 Patients are at high risk of complications including hemophagocytic lymphohistiocytosis (HLH, also known as hemophagocytic syndrome, or HPS) and sepsis. At this time, IRB statement: This study was IRB exempt.

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Section: Discussionmentioning

confidence: 99%

The significance of epidermal involvement in primary cutaneous gamma/delta (γδ) T‐cell lymphoma: A systematic review and meta‐analysis

2021

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“…In our review of the literature, we found 17 previous cases of CD20 MF and three previous cases of TCR-delta MF (Table 1). [2][3][4][5][6][7][8][9][10][11][12] In contrast with the rarity of the reported cases, 2 of the 28 (7%) of the incident cases of MF at our institution exhibited unexpected CD20 or TCR-delta expression. We suspect anomalous expression of CD20 or TCR delta may be more common in MF than its rarity in the literature suggests.…”

Section: Discussionmentioning

confidence: 99%

Gamma–Delta and CD20 Mycosis Fungoides: Two Cases Uncovered by Broad-Spectrum Immunostaining

Patel

Fraga

2021

The American Journal of Dermatopathology

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Mycosis fungoides (MF) expresses T-cell markers and the alpha-beta T-cell receptor (TCR) complex. Here, we describe a case of MF with dual expression of TCR delta and TCR beta and a case of MF expressing the B-cell marker CD20. Both anomalies were detected after we instituted a broad-spectrum immunostaining panel for cutaneous T-cell lymphomas. These findings suggest anomalous immunophenotypes may be more common in MF than previously appreciated. Histopathologists should be aware of unexpected malleability in the immunophenotype of MF to avoid confusion with other subtypes of cutaneous lymphoma. Further research into the prevalence and significance of CD20 and TCR-delta expression in MF is encouraged.

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“…42 Although rare, a handful of cases of gd TCR expressing cases of MF have been documented, and the significance of this finding may come into question in clinical practice. 6,[84][85][86][87][88][89] Given that the number of cases is very limited, evidence does not clearly demonstrate a significant difference in prognosis for patients with gd phenotype. For example, Pulitzer et al 84 identified 9 patients with gd-positive MF.…”

Section: Mycosis Fungoidesmentioning

confidence: 99%

“…42 Although rare, a handful of cases of γδ TCR expressing cases of MF have been documented, and the significance of this finding may come into question in clinical practice. 6,84–89…”

Section: Leukemias and Lymphomas With A Rare γδ Tcr Phenotypementioning

confidence: 99%

Gamma/Delta (γδ) T Cells: The Role of the T-Cell Receptor in Diagnosis and Prognosis of Hematologic Malignancies

Goyal

Nardi

2021

The American Journal of Dermatopathology

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There are 2 types of T cells: ab and gd T cells, named based on the composition of the T-cell receptor. gd T cells are rare, making up 0.5%-10% of T cells. Although most leukemias, lymphomas, and immune-mediated conditions derive from ab T cells, a handful of rare but important diseases are generally derived from gd T cells, particularly primary cutaneous gd T-cell lymphoma, hepatosplenic T-cell lymphoma, and monomorphic epitheliotropic intestinal T-cell lymphoma. There are also malignancies that may evince a gd TCR phenotype, including large granulocytic lymphocyte leukemia, T-cell acute lymphobplastic leukemia (T-ALL), and mycosis fungoides, although such cases are rare. In this article, we will review the genesis of the T-cell receptor, the role of gd T cells, and the importance of TCR type and methods of detection and outline the evidence for prognostic significance (or lack thereof) in lymphomas of gd T cells. We will also highlight conditions that rarely may present with a gd TCR phenotype and assess the utility of testing for TCR type in these diseases.

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Unmasking a T cell lymphoma: Folliculotropic mycosis fungoides with a gamma-delta phenotype

Cited by 4 publications

References 3 publications

The significance of epidermal involvement in primary cutaneous gamma/delta (γδ) T‐cell lymphoma: A systematic review and meta‐analysis

The significance of epidermal involvement in primary cutaneous gamma/delta (γδ) T‐cell lymphoma: A systematic review and meta‐analysis

Gamma–Delta and CD20 Mycosis Fungoides: Two Cases Uncovered by Broad-Spectrum Immunostaining

Gamma/Delta (γδ) T Cells: The Role of the T-Cell Receptor in Diagnosis and Prognosis of Hematologic Malignancies

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