Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder
Haematolymphoid Tumours (WHO Classification, 5th ed.)
 | This page is under construction |
(General Instructions – The main focus of these pages is the clinically significant genetic alterations in each disease type. Use HUGO-approved gene names and symbols (italicized when appropriate), HGVS-based nomenclature for variants, as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples); to add (or move) a row or column to a table, click within the table and select the > symbol that appears to be given options. Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see Author_Instructions and FAQs as well as contact your Associate Editor or Technical Support)
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. |
| 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
Put your text placeholder here (or anywhere appropriate on the page) and use the "Link" icon at the top of the page (Instructions: Highlight text to which you want to add a link in this section or elsewhere, select the "Link" icon at the top of the page, and search the name of the internal page to which you want to link this text, or enter an external internet address by including the "http://www." portion.)
References
(use the "Cite" icon at the top of the page) (Instructions: Add each reference into the text above by clicking on where you want to insert the reference, selecting the “Cite” icon at the top of the page, and using the “Automatic” tab option to search such as by PMID to select the reference to insert. The reference list in this section will be automatically generated and sorted. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference.)
- ↑ 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) - ↑ 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) - ↑ 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) - ↑ 4.0 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) - ↑ 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) - ↑ 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) - ↑ 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) - ↑ 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) - ↑ 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) - ↑ 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) - ↑ 11.0 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) - ↑ 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
- 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.