U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Primary cutaneous diffuse large B-cell lymphoma, Leg type

MedGen UID:
311155
Concept ID:
C1709656
Neoplastic Process
Synonyms: PCDLBCL,LT; Primary cutaneous diffuse large B-cell lymphoma leg type; Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type; primary cutaneous diffuse large B-cell lymphoma, Leg type; Primary cutaneous diffuse large B-cell lymphoma, leg type; Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg-Type
SNOMED CT: Primary cutaneous diffuse large B-cell lymphoma leg type (1187190002)
 
Monarch Initiative: MONDO:0006383
Orphanet: ORPHA178544

Definition

A rare, aggressive, primary cutaneous B-cell lymphoma characterized by rapidly progressive, red to bluish, often ulcerating, nodular tumors predominantly involving the lower legs. Histology shows sheets of centroblasts and immunoblasts that spare the epidermis, but infiltrate the dermis and subcutaneous tissues, and often disseminate extracutaneously. The neoplastic cells typically express CD20, CD79a, Bcl-2, MUM-1, and FOXP1, but are negative for CD10. [from ORDO]

Term Hierarchy

Professional guidelines

PubMed

Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Aug;98(8):1326-1332. Epub 2023 May 22 doi: 10.1002/ajh.26968. PMID: 37434388
Krenitsky A, Klager S, Hatch L, Sarriera-Lazaro C, Chen PL, Seminario-Vidal L
Am J Clin Dermatol 2022 Sep;23(5):689-706. Epub 2022 Jul 19 doi: 10.1007/s40257-022-00704-0. PMID: 35854102
Fava P, Roccuzzo G, Alberti-Violetti S, Grandi V, Pileri A, Pimpinelli N, Berti E, Quaglino P
Presse Med 2022 Mar;51(1):104109. Epub 2022 Jan 11 doi: 10.1016/j.lpm.2022.104109. PMID: 35026390

Recent clinical studies

Etiology

Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Aug;98(8):1326-1332. Epub 2023 May 22 doi: 10.1002/ajh.26968. PMID: 37434388
Blanchard M, Hoetzenecker W, Blanchard G, Guenova E
Ital J Dermatol Venerol 2023 Jun;158(3):205-216. doi: 10.23736/S2784-8671.23.07572-2. PMID: 37278499
Fava P, Roccuzzo G, Alberti-Violetti S, Grandi V, Pileri A, Pimpinelli N, Berti E, Quaglino P
Presse Med 2022 Mar;51(1):104109. Epub 2022 Jan 11 doi: 10.1016/j.lpm.2022.104109. PMID: 35026390
Kraft RM, Ansell SM, Villasboas JC, Bennani NN, Wang Y, Habermann TM, Thanarajasingam G, Lester SC, Macon W, Inwards DJ, Porrata LF, Micallef IN, Witzig TE, Thompson CA, Johnston PB, Nowakowski GS, Lin Y, Paludo J
Hematol Oncol 2021 Dec;39(5):658-663. Epub 2021 Aug 28 doi: 10.1002/hon.2919. PMID: 34453851
Hope CB, Pincus LB
Clin Lab Med 2017 Sep;37(3):547-574. doi: 10.1016/j.cll.2017.05.009. PMID: 28802500

Diagnosis

Villasenor-Park J, Chung J, Kim EJ
Hematol Oncol Clin North Am 2024 Oct;38(5):1111-1131. Epub 2024 Jul 23 doi: 10.1016/j.hoc.2024.05.017. PMID: 39048407
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Aug;98(8):1326-1332. Epub 2023 May 22 doi: 10.1002/ajh.26968. PMID: 37434388
Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ
Pathology 2020 Jan;52(1):53-67. Epub 2019 Nov 15 doi: 10.1016/j.pathol.2019.08.013. PMID: 31735345
Goyal A, LeBlanc RE, Carter JB
Hematol Oncol Clin North Am 2019 Feb;33(1):149-161. doi: 10.1016/j.hoc.2018.08.006. PMID: 30497672
Hope CB, Pincus LB
Clin Lab Med 2017 Sep;37(3):547-574. doi: 10.1016/j.cll.2017.05.009. PMID: 28802500

Therapy

Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Aug;98(8):1326-1332. Epub 2023 May 22 doi: 10.1002/ajh.26968. PMID: 37434388
Laurent C, Ram-Wolff C, Ingen-Housz-Oro S, Beylot-Barry M, Barete S, Saillard C, Dupuy A, Bagot M, Adamski H
Clin Exp Dermatol 2023 Feb 2;48(2):116-120. doi: 10.1093/ced/llac044. PMID: 36730524
Zehnder M, Amarov B, Abrunhosa-Branquinho AN, Maiwald-Urosevic M, Mühleisen B, Saulite I, Anzengruber F, Imhof L, Navarini AA, Cozzio A, Dummer R, Dimitriou F, Guenova E
Dermatology 2022;238(5):967-976. Epub 2022 Feb 14 doi: 10.1159/000522053. PMID: 35158362
Kraft RM, Ansell SM, Villasboas JC, Bennani NN, Wang Y, Habermann TM, Thanarajasingam G, Lester SC, Macon W, Inwards DJ, Porrata LF, Micallef IN, Witzig TE, Thompson CA, Johnston PB, Nowakowski GS, Lin Y, Paludo J
Hematol Oncol 2021 Dec;39(5):658-663. Epub 2021 Aug 28 doi: 10.1002/hon.2919. PMID: 34453851
Suárez AL, Querfeld C, Horwitz S, Pulitzer M, Moskowitz A, Myskowski PL
J Am Acad Dermatol 2013 Sep;69(3):343.e1-11; quiz 355-6. doi: 10.1016/j.jaad.2013.06.011. PMID: 23957985

Prognosis

Villasenor-Park J, Chung J, Kim EJ
Hematol Oncol Clin North Am 2024 Oct;38(5):1111-1131. Epub 2024 Jul 23 doi: 10.1016/j.hoc.2024.05.017. PMID: 39048407
Hristov AC, Tejasvi T, Wilcox RA
Am J Hematol 2023 Aug;98(8):1326-1332. Epub 2023 May 22 doi: 10.1002/ajh.26968. PMID: 37434388
Kraft RM, Ansell SM, Villasboas JC, Bennani NN, Wang Y, Habermann TM, Thanarajasingam G, Lester SC, Macon W, Inwards DJ, Porrata LF, Micallef IN, Witzig TE, Thompson CA, Johnston PB, Nowakowski GS, Lin Y, Paludo J
Hematol Oncol 2021 Dec;39(5):658-663. Epub 2021 Aug 28 doi: 10.1002/hon.2919. PMID: 34453851
Goyal A, LeBlanc RE, Carter JB
Hematol Oncol Clin North Am 2019 Feb;33(1):149-161. doi: 10.1016/j.hoc.2018.08.006. PMID: 30497672
Skala SL, Hristov B, Hristov AC
Arch Pathol Lab Med 2018 Nov;142(11):1313-1321. doi: 10.5858/arpa.2018-0215-RA. PMID: 30407851

Clinical prediction guides

Kraft RM, Ansell SM, Villasboas JC, Bennani NN, Wang Y, Habermann TM, Thanarajasingam G, Lester SC, Macon W, Inwards DJ, Porrata LF, Micallef IN, Witzig TE, Thompson CA, Johnston PB, Nowakowski GS, Lin Y, Paludo J
Hematol Oncol 2021 Dec;39(5):658-663. Epub 2021 Aug 28 doi: 10.1002/hon.2919. PMID: 34453851
Menguy S, Prochazkova-Carlotti M, Beylot-Barry M, Saltel F, Vergier B, Merlio JP, Pham-Ledard A
Am J Surg Pathol 2018 Mar;42(3):326-334. doi: 10.1097/PAS.0000000000000983. PMID: 29112015
Kim MJ, Hong ME, Maeng CH, Jung HA, Hong JY, Choi MK, Kim SJ, Ko YH, Kim WS
Int J Hematol 2015 Mar;101(3):273-8. Epub 2015 Jan 1 doi: 10.1007/s12185-014-1728-2. PMID: 25552248
Suárez AL, Querfeld C, Horwitz S, Pulitzer M, Moskowitz A, Myskowski PL
J Am Acad Dermatol 2013 Sep;69(3):343.e1-11; quiz 355-6. doi: 10.1016/j.jaad.2013.06.011. PMID: 23957985
Grange F, Beylot-Barry M, Courville P, Maubec E, Bagot M, Vergier B, Souteyrand P, Machet L, Dalac S, Esteve E, Templier I, Delaporte E, Avril MF, Robert C, Dalle S, Laroche L, Delaunay M, Joly P, Wechsler J, Petrella T
Arch Dermatol 2007 Sep;143(9):1144-50. doi: 10.1001/archderm.143.9.1144. PMID: 17875875

Recent systematic reviews

Sokol L, Naghashpour M, Glass LF
Cancer Control 2012 Jul;19(3):236-44. doi: 10.1177/107327481201900308. PMID: 22710899

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...