Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder

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Haematolymphoid Tumours (WHO Classification, 5th ed.)

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Primary Author(s)*

Ahmed Eladely, MBBCh. Andrew Siref, MD.

Creighton University, Omaha, NE.

WHO Classification of Disease

Structure Disease
Book Haematolymphoid Tumours (5th ed.)
Category T-cell and NK-cell lymphoid proliferations and lymphomas
Family Mature T-cell and NK-cell neoplasms
Type Primary cutaneous T-cell lymphoid proliferations and lymphomas
Subtype(s) Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder

Definition / Description of Disease

Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder is a rare type of lymphoproliferative disorder characterized by slow-growing papules and nodules primarily affecting acral sites, such as the ears, with a benign clinical course. [1]

Synonyms / Terminology

Indolent CD8-positive lymphoid proliferation of the ear.

Primary cutaneous acral CD8-positive T-cell lymphoma (older terminology; designation as lymphoma is no longer preferred in the 5th edition WHO Classification).

Epidemiology / Prevalence

Rare disease accounting for < 1% of all primary cutaneous lymphomas. The disease predominates in males, with an M:F ratio of 2:1. The median age is 56 years. [2]

Clinical Features

Signs and Symptoms Cutaneous, slowly progressive papule or nodule.

Solitary or multiple (rare). [1] [3] [4]

Laboratory Findings None

Sites of Involvement

Ears (most common), retroauricular area, nose, and feet.

Rare sites: leg, trunk, genitals, and eyelid.[1][4] [5]

Morphologic Features

On H&E, the tumor shows a dense, monotonous dermal proliferation of atypical medium-sized lymphocytes. The lymphocytes have irregular and frequently folded nuclei with fine chromatin and moderate nuclear pleomorphism.[6]A perivascular pattern may be seen, although it is less common.

Usually, the epidermis is spared, but focal minimal epidermotropism and focal folliculotropism may be seen. A Grenz zone separates the epidermis from the dermal infiltrate in approximately one-third of cases. The proliferation may extend into the subcutis.

Mitotic activity is absent or low. Plasma cells, histiocytes, neutrophils, and eosinophils are either absent or infrequent.[1][7]

Immunophenotype

Finding Marker
Positive CD3, TIA1, CD45RA, CD68 (Golgi pattern)
CD4/CD8 CD4(-), CD8(+)
CD2/CD5/CD7 Loss of ≥ 1: CD2, CD5, and/or CD7
TCR TCR-βF1(+), TCRγδ(-)
Ki-67/MIB1 <15%
Negative CD56, CD57, CD30, CD45RO, Perforin, Granzyme B, ICOS, PD-1, EBER (always negative)

There is one reported case with a CD4+/CD8+ phenotype. [8]

Few reported cases have shown a high Ki67 proliferation index.[9]

The Golgi dot-like staining pattern of CD68 in tumor cells is unique to this entity.[10]

Chromosomal Rearrangements (Gene Fusions)

Chromosomal rearrangements contributing to tumor formation have not yet been described.

Individual Region Genomic Gain / Loss / LOH

Individual region genomic gain, loss, or LOH contributing to tumor formation have not yet been described

Characteristic Chromosomal Patterns

Characteristic chromosomal patterns contributing to tumor formation have not yet been described

Gene Mutations (SNV / INDEL)

Gene mutations contributing to tumor formation have not yet been described

Epigenomic Alterations

Epigenomic alterations contributing to tumor formation have not yet been described

Genes and Main Pathways Involved

Gene mutations contributing to tumor formation have not yet been described

Genetic Diagnostic Testing Methods

In nearly all cases, the neoplastic T cells exhibit clonal rearrangements of T-cell receptor (TCR) gamma and/or beta genes.[11]

Familial Forms

None.

Additional Information

The tumor generally has an excellent prognosis, with no reported fatal outcomes. Complete remission following surgical excision or local radiation therapy is common. Recurrence after treatment is possible, more frequently seen in younger patients, and can occasionally occur at other cutaneous sites. Dissemination to extracutaneous sites has been reported in only one case.[11][12]

Links

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References

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  1. 1.0 1.1 1.2 1.3 Greenblatt, Danielle; et al. (2013-02). "Indolent CD8(+) lymphoid proliferation of acral sites: a clinicopathologic study of six patients with some atypical features". Journal of Cutaneous Pathology. 40 (2): 248–258. doi:10.1111/cup.12045. ISSN 1600-0560. PMID 23189944. Check date values in: |date= (help)
  2. Tjahjono, Leonardo A.; et al. (2019-09). "Primary Cutaneous Acral CD8+ T-Cell Lymphoma-A Single Center Review of 3 Cases and Recent Literature Review". The American Journal of Dermatopathology. 41 (9): 644–648. doi:10.1097/DAD.0000000000001366. ISSN 1533-0311. PMID 31433793. Check date values in: |date= (help)
  3. Beltraminelli, Helmut; et al. (2010-01). "Indolent CD8+ lymphoid proliferation of the ear: a phenotypic variant of the small-medium pleomorphic cutaneous T-cell lymphoma?". Journal of Cutaneous Pathology. 37 (1): 81–84. doi:10.1111/j.1600-0560.2009.01278.x. ISSN 1600-0560. PMID 19602068. Check date values in: |date= (help)
  4. 4.0 4.1 Kempf, Werner; et al. (2013-04). "Primary cutaneous CD8(+) small- to medium-sized lymphoproliferative disorder in extrafacial sites: clinicopathologic features and concept on their classification". The American Journal of Dermatopathology. 35 (2): 159–166. doi:10.1097/DAD.0b013e31825c3a33. ISSN 1533-0311. PMID 22885550. Check date values in: |date= (help)
  5. Hagen, Joshua W.; et al. (2014-02). "Indolent CD8+ lymphoid proliferation of the face with eyelid involvement". The American Journal of Dermatopathology. 36 (2): 137–141. doi:10.1097/DAD.0b013e318297f7fd. ISSN 1533-0311. PMID 24556898. Check date values in: |date= (help)
  6. Petrella, Tony; et al. (2007-12). "Indolent CD8-positive lymphoid proliferation of the ear: a distinct primary cutaneous T-cell lymphoma?". The American Journal of Surgical Pathology. 31 (12): 1887–1892. doi:10.1097/PAS.0b013e318068b527. ISSN 0147-5185. PMID 18043044. Check date values in: |date= (help)
  7. Butsch, Florian; et al. (2012-03). "Bilateral indolent epidermotropic CD8-positive lymphoid proliferations of the ear". Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology: JDDG. 10 (3): 195–196. doi:10.1111/j.1610-0387.2011.07859.x. ISSN 1610-0387. PMID 22142195. Check date values in: |date= (help)
  8. Toberer, Ferdinand; et al. (2019-03). "Double-positive CD8/CD4 primary cutaneous acral T-cell lymphoma". Journal of Cutaneous Pathology. 46 (3): 231–233. doi:10.1111/cup.13403. ISSN 1600-0560. PMID 30552698. Check date values in: |date= (help)
  9. Swick, Brian L.; et al. (2011-02). "Indolent CD8+ lymphoid proliferation of the ear: report of two cases and review of the literature". Journal of Cutaneous Pathology. 38 (2): 209–215. doi:10.1111/j.1600-0560.2010.01647.x. ISSN 1600-0560. PMID 21083681. Check date values in: |date= (help)
  10. Wobser, M.; et al. (2015-06). "CD68 expression is a discriminative feature of indolent cutaneous CD8-positive lymphoid proliferation and distinguishes this lymphoma subtype from other CD8-positive cutaneous lymphomas". The British Journal of Dermatology. 172 (6): 1573–1580. doi:10.1111/bjd.13628. ISSN 1365-2133. PMID 25524664. Check date values in: |date= (help)
  11. 11.0 11.1 Kempf, Werner; et al. (2022-05). "Clinical, histopathological and prognostic features of primary cutaneous acral CD8+ T-cell lymphoma and other dermal CD8+ cutaneous lymphoproliferations: results of an EORTC Cutaneous Lymphoma Group workshop". The British Journal of Dermatology. 186 (5): 887–897. doi:10.1111/bjd.20973. ISSN 1365-2133. PMID 34988968 Check |pmid= value (help). Check date values in: |date= (help)
  12. Alberti-Violetti, Silvia; et al. (2017-11). "Primary cutaneous acral CD8 positive T-cell lymphoma with extra-cutaneous involvement: A long-standing case with an unexpected progression". Journal of Cutaneous Pathology. 44 (11): 964–968. doi:10.1111/cup.13020. ISSN 1600-0560. PMID 28796362. Check date values in: |date= (help)

EXAMPLE Book

  1. Arber DA, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p129-171.

Notes

*Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage).  Additional global feedback or concerns are also welcome. *Citation of this Page: “Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated 09/6/2024, https://ccga.io/index.php/HAEM5:Primary_cutaneous_acral_CD8-positive_T-cell_lymphoproliferative_disorder.