| Joflaher/sandbox | |
|---|---|
| Other names | Primary cutaneous acral CD8 positive T cell lymphoma, Indolent CD8+ lymphoid proliferation of the ear |
| Specialty | Dermatology, Oncology |
| Usual onset | Adults |
| Prognosis | Good to excellent |
| Frequency | Rare |
Primary cutaneous acral CD8 positive T cell lymphoproliferative disorder (CD8+ TLPD) is a disease in which a slow-growing papule or nodule develops on the ear or, less commonly, other acral sites. Acral sites are peripheral parts of the body and include the hands, arms, feet, legs, ears, nose, fingernails, and toenails.[1] Petrella et al., 2007[2] reported four patients with these lesions on their ears and termed the disorder indolent CD8+ lymphoid proliferation of the ear. In 2018, the World Health Organization and European Organisation for Research and Treatment of Cancer provisionally classified this disorder as a lymphoma and termed it primary cutaneous acral CD8 positive T cell lymphoma.[3] (The "primary" used to designate cutaneous lymphomas indicates that the lymphoma was first diagnosed as limited to the skin and there was no evidence of spread to extracutaneous tissues for 6 months after the diagnosis was first made.[4]) In 2022, however, the International Consensus Classification (ICC) group of experts in various medical fields maintained that this disorder was not malignant and therefore termed it primary cutaneous acral CD8 positive T cell lymphoproliferative disorder.[1][5] This article follows the ICC classification and renaming of primary cutaneous acral CD8 positive T cell lymphoma as primary cutaneous acral CD8 positive T cell lymphoproliferative disorder.
Presentation
editAt presentation, 31 patients with CD8+ TLPD were 29–89 years old (average 52.1 years); 23 were male, 8 were female; 26 had nodules, 5 had plaques; 28 had a single lesion, 2 had bilateral lesions, and 1 had multiple lesions; 18 had a single lesion on the ear, 3 on the nose, 1 on the leg, 4 on the foot, 2 on the arm, 1 on the hand, 1 on a non-acral site behind the ear, and 1 had disseminated lesions on the head and neck .[6] These lesions had been present for several months, were slow-growing, and reddish/purple nodules or plaques that measured up to several centimeters in maximal dimension. Among the 26 patients for which this data was available, their lesions began and slow growed for 3 to 420 months (median duration 12 months) prior to their diagnosis.[1] Various analyses to define the patients stage, i.e., extent, of disease using computed tomography scans and/or Bone marrow examinations almost uniformly found that the lesions did not spread to non-cutaneous sites.[6][7]
References
edit- ^ a b c Goodlad JR, Cerroni L, Swerdlow SH (January 2023). "Recent advances in cutaneous lymphoma-implications for current and future classifications". Virchows Archiv : an International Journal of Pathology. 482 (1): 281–298. doi:10.1007/s00428-022-03421-5. PMC 9852132. PMID 36278991.
- ^ Petrella T, Maubec E, Cornillet-Lefebvre P, Willemze R, Pluot M, Durlach A, Marinho E, Benhamou JL, Jansen P, Robson A, Grange F (December 2007). "Indolent CD8-positive lymphoid proliferation of the ear: a distinct primary cutaneous T-cell lymphoma?". The American Journal of Surgical Pathology. 31 (12): 1887–92. doi:10.1097/PAS.0b013e318068b527. PMID 18043044.
- ^ Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, Jaffe ES (April 2019). "The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas". Blood. 133 (16): 1703–1714. doi:10.1182/blood-2018-11-881268. PMC 6473500. PMID 30635287.
- ^ Magro CM, Porcu P, Ahmad N, Klinger D, Crowson AN, Nuovo G (September 2004). "Cutaneous immunocytoma: a clinical, histologic, and phenotypic study of 11 cases". Applied Immunohistochemistry & Molecular Morphology : AIMM. 12 (3): 216–24. doi:10.1097/00129039-200409000-00006. PMID 15551734.
- ^ Campo E, Jaffe ES, Cook JR, Quintanilla-Martinez L, Swerdlow SH, Anderson KC, Brousset P, Cerroni L, de Leval L, Dirnhofer S, Dogan A, Feldman AL, Fend F, Friedberg JW, Gaulard P, Ghia P, Horwitz SM, King RL, Salles G, San-Miguel J, Seymour JF, Treon SP, Vose JM, Zucca E, Advani R, Ansell S, Au WY, Barrionuevo C, Bergsagel L, Chan WC, Cohen JI, d'Amore F, Davies A, Falini B, Ghobrial IM, Goodlad JR, Gribben JG, Hsi ED, Kahl BS, Kim WS, Kumar S, LaCasce AS, Laurent C, Lenz G, Leonard JP, Link MP, Lopez-Guillermo A, Mateos MV, Macintyre E, Melnick AM, Morschhauser F, Nakamura S, Narbaitz M, Pavlovsky A, Pileri SA, Piris M, Pro B, Rajkumar V, Rosen ST, Sander B, Sehn L, Shipp MA, Smith SM, Staudt LM, Thieblemont C, Tousseyn T, Wilson WH, Yoshino T, Zinzani PL, Dreyling M, Scott DW, Winter JN, Zelenetz AD (September 2022). "The International Consensus Classification of Mature Lymphoid Neoplasms: a report from the Clinical Advisory Committee". Blood. 140 (11): 1229–1253. doi:10.1182/blood.2022015851. PMC 9479027. PMID 35653592.
- ^ a b Kempf W, Petrella T, Willemze R, Jansen P, Berti E, Santucci M, Geissinger E, Cerroni L, Maubec E, Battistella M, Goodlad J, Guenova E, Lappalainen K, Ranki A, Craig P, Calonje E, Martin B, Whittaker S, Oschlies I, Wehkamp U, Nicolay JP, Wobser M, Scarisbruck J, Pimpinelli N, Stadler R, Kerl French K, Quaglino P, Lin J, Chen L, Beer M, Emanuel P, Dalle S, Robson A (May 2022). "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. PMID 34988968.
- ^ Tjahjono LA, Davis MD, Witzig TE, Comfere NI (September 2019). "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. PMID 31433793.