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Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

MedGen UID:
1388716
•Concept ID:
C4518232
•
Neoplastic Process
Synonyms: Berti lymphoma; primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma; Primary Cutaneous Aggressive Epidermotropic CD8-Positive T-Cell Lymphoma; Primary cutaneous CD8 positive aggressive epidermotropic cytotoxic T-cell lymphoma; Primary Cutaneous CD8-Positive Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma; Primary Cutaneous CD8-Positive Aggressive Epidermotropic T-Cell Lymphoma; Primary cutaneous epidermotropic cytotoxic CD8 positive T-cell lymphoma; primary cutaneous epidermotropic cytotoxic CD8+ T-cell lymphoma; Primary cutaneous epidermotropic cytotoxic CD8+ T-cell lymphoma
SNOMED CT: Primary cutaneous CD8 positive aggressive epidermotropic cytotoxic T-cell lymphoma (733895005); Primary cutaneous epidermotropic cytotoxic CD8 positive T-cell lymphoma (765136002); Primary cutaneous CD8 positive aggressive epidermotropic cytotoxic T-cell lymphoma (765136002); Berti lymphoma (765136002)
 
Monarch Initiative: MONDO:0015811
Orphanet: ORPHA178528

Definition

A rare form of primary cutaneous T-cell lymphoma characterized by rapidly progressing localized or disseminated nodules, tumors or eczematous skin lesions. It has a particularly aggressive clinical course with a high tendency to spread, in advanced stages, to extracutaneous locations (central nervous system, lung, testes). Lymph nodes are often spared. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

Professional guidelines

PubMed

Histopathological aspects and differential diagnosis of CD8 positive lymphomatoid papulosis.
Marschalkó M, Gyöngyösi N, Noll J, Károlyi Z, Wikonkál N, Hársing J, Kuroli E, Csomor J, Matolcsy A, Sarolta K, Szepesi Á
J Cutan Pathol 2016 Nov;43(11):963-973. Epub 2016 Aug 30 doi: 10.1111/cup.12779. PMID: 27461449
Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: proposed diagnostic criteria and therapeutic evaluation.
Nofal A, Abdel-Mawla MY, Assaf M, Salah E
J Am Acad Dermatol 2012 Oct;67(4):748-59. Epub 2012 Jan 9 doi: 10.1016/j.jaad.2011.07.043. PMID: 22226429

Recent clinical studies

Etiology

Classifications of cutaneous lymphomas and lymphoproliferative disorders: An update from the EORTC cutaneous lymphoma histopathology group.
Kempf W, Mitteldorf C, Cerroni L, Willemze R, Berti E, Guenova E, Scarisbrick JJ, Battistella M
J Eur Acad Dermatol Venereol 2024 Aug;38(8):1491-1503. Epub 2024 Apr 6 doi: 10.1111/jdv.19987. PMID: 38581201
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part II: Prognosis and management.
Oh Y, Stoll JR, Moskowitz A, Pulitzer M, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1093-1106. Epub 2021 May 1 doi: 10.1016/j.jaad.2021.04.081. PMID: 33945836
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098
Rare T-Cell Subtypes.
van der Weyden C, McCormack C, Lade S, Johnstone RW, Prince HM
Cancer Treat Res 2019;176:195-224. doi: 10.1007/978-3-319-99716-2_10. PMID: 30596220
NK/T-cell lymphoma, nasal type, γδ T-cell lymphoma, and CD8-positive epidermotropic T-cell lymphoma-clinical and histopathologic features, differential diagnosis, and treatment.
Geller S, Myskowski PL, Pulitzer M
Semin Cutan Med Surg 2018 Mar;37(1):30-38. doi: 10.12788/j.sder.2018.020. PMID: 29719018Free PMC Article

Diagnosis

Classifications of cutaneous lymphomas and lymphoproliferative disorders: An update from the EORTC cutaneous lymphoma histopathology group.
Kempf W, Mitteldorf C, Cerroni L, Willemze R, Berti E, Guenova E, Scarisbrick JJ, Battistella M
J Eur Acad Dermatol Venereol 2024 Aug;38(8):1491-1503. Epub 2024 Apr 6 doi: 10.1111/jdv.19987. PMID: 38581201
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098
Rare T-Cell Subtypes.
van der Weyden C, McCormack C, Lade S, Johnstone RW, Prince HM
Cancer Treat Res 2019;176:195-224. doi: 10.1007/978-3-319-99716-2_10. PMID: 30596220
Rare Cutaneous T-Cell Lymphomas.
Damasco F, Akilov OE
Hematol Oncol Clin North Am 2019 Feb;33(1):135-148. doi: 10.1016/j.hoc.2018.08.004. PMID: 30497671
Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: proposed diagnostic criteria and therapeutic evaluation.
Nofal A, Abdel-Mawla MY, Assaf M, Salah E
J Am Acad Dermatol 2012 Oct;67(4):748-59. Epub 2012 Jan 9 doi: 10.1016/j.jaad.2011.07.043. PMID: 22226429

Therapy

Durable remission of a patient with primary cutaneous CD8(+) aggressive epidermotropic cytotoxic T-cell lymphoma.
Al Aoun SM, Iqbal S, AlHalouli TM, Zaidi SZ, Motabi IH
Hematol Oncol Stem Cell Ther 2021 Mar;14(1):71-75. Epub 2018 Sep 28 doi: 10.1016/j.hemonc.2018.09.004. PMID: 30291826
Primary Cutaneous Aggressive Epidermotropic CD8+ T-cell Lymphoma with Brain Involvement and Mimicking Dermatomyositis.
Terui H, Yamasaki K, Yoshida S, Aiba S
Acta Derm Venereol 2018 Jan 12;98(1):128-129. doi: 10.2340/00015555-2728. PMID: 28597026
Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma: a diagnostic and therapeutic challenge.
Nofal A, Abdel-Mawla MY, Assaf M, Salah E, Abd-Elazim H
Int J Dermatol 2014 Jan;53(1):76-81. Epub 2013 Jun 20 doi: 10.1111/j.1365-4632.2012.05479.x. PMID: 23786415
Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma with a chronic and indolent course. Is this different from peripheral T cell lymphoma?
Gomez Vazquez M, Navarra Amayuelas R
Dermatol Online J 2012 Mar 15;18(3):11. PMID: 22483522
Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma.
Gormley RH, Hess SD, Anand D, Junkins-Hopkins J, Rook AH, Kim EJ
J Am Acad Dermatol 2010 Feb;62(2):300-7. Epub 2009 Nov 26 doi: 10.1016/j.jaad.2009.02.035. PMID: 19944484

Prognosis

Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098
Rare Cutaneous T-Cell Lymphomas.
Damasco F, Akilov OE
Hematol Oncol Clin North Am 2019 Feb;33(1):135-148. doi: 10.1016/j.hoc.2018.08.004. PMID: 30497671
NK/T-cell lymphoma, nasal type, γδ T-cell lymphoma, and CD8-positive epidermotropic T-cell lymphoma-clinical and histopathologic features, differential diagnosis, and treatment.
Geller S, Myskowski PL, Pulitzer M
Semin Cutan Med Surg 2018 Mar;37(1):30-38. doi: 10.12788/j.sder.2018.020. PMID: 29719018Free PMC Article
Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: proposed diagnostic criteria and therapeutic evaluation.
Nofal A, Abdel-Mawla MY, Assaf M, Salah E
J Am Acad Dermatol 2012 Oct;67(4):748-59. Epub 2012 Jan 9 doi: 10.1016/j.jaad.2011.07.043. PMID: 22226429
Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma.
Gormley RH, Hess SD, Anand D, Junkins-Hopkins J, Rook AH, Kim EJ
J Am Acad Dermatol 2010 Feb;62(2):300-7. Epub 2009 Nov 26 doi: 10.1016/j.jaad.2009.02.035. PMID: 19944484

Clinical prediction guides

Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma With Secondary Cutaneous Involvement: A Diagnostic Challenge.
Olmos-Alpiste F, Vázquez I, Gallardo F, Sánchez-Gonzalez B, Colomo L, Pujol RM
Am J Dermatopathol 2021 Apr 1;43(4):300-304. doi: 10.1097/DAD.0000000000001855. PMID: 33264131
Cutaneous Lymphoproliferative Disorders: What's New in the Revised 4th Edition of the World Health Organization (WHO) Classification of Lymphoid Neoplasms.
Sundram U
Adv Anat Pathol 2019 Mar;26(2):93-113. doi: 10.1097/PAP.0000000000000208. PMID: 30199396
Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study.
Brüggen MC, Kerl K, Haralambieva E, Schanz U, Chang YT, Ignatova D, Dummer R, Cozzio A, Hoetzenecker W, French LE, Guenova E
Acta Derm Venereol 2018 Oct 10;98(9):835-841. doi: 10.2340/00015555-2950. PMID: 29693696
Primary Cutaneous Aggressive Epidermotropic CD8+ T-cell Lymphoma with Brain Involvement and Mimicking Dermatomyositis.
Terui H, Yamasaki K, Yoshida S, Aiba S
Acta Derm Venereol 2018 Jan 12;98(1):128-129. doi: 10.2340/00015555-2728. PMID: 28597026
Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma with KIR3DL2 and NKp46 expression in a human immunodeficiency virus carrier.
Karkouche R, Ingen-Housz-Oro S, Le Gouvello S, Charlotte F, Thomas M, Zehou O, Frenkel V, Boutboul D, Chosidow O, Caumes E, Gaulard P, Ortonne N
J Cutan Pathol 2015 Mar;42(3):199-205. Epub 2014 Dec 23 doi: 10.1111/cup.12448. PMID: 25407699

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