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:* [[Primary cutaneous diffuse large B-cell lymphoma, leg type]] |
:* [[Primary cutaneous diffuse large B-cell lymphoma, leg type]] |
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Because recent studies had shown that primary cutaneous marginal zone lymphoma, which was formerly classified as a subtype of the [[MALT lymphoma]]s: '''a)''' has a distinct microscopic [[histology]] and [[Gene expression profiling in cancer|gene expression profile]]; '''b)''' spreads to extracutaneous tissue in only 4 to 8.5% of cases; '''c)''' has a [[Survival rate#Cause-specific survival and disease-specific survival|disease-specific survival]] of 99% even in patients not receiving aggressive therapy; and '''d)''' has pathological findings that overlap the benign cutaneous disorders termed [[cutaneous lymphoid hyperplasia]]. Consequently, EORTC, 2022, renamed primary cutaneous marginal zone lymphoma as primary cutaneous marginal zone lymphoproliferative disorder.<ref name="pmid36278991"/> Primary cutaneous follicle center lymphoma is also an indolent lymphoma.<ref name="pmid38309432">{{cite journal | vauthors = Medeiros LJ, Chadburn A, Natkunam Y, Naresh KN | title = Fifth Edition of the World Health Classification of Tumors of the Hematopoietic and Lymphoid Tissues: B-cell Neoplasms | journal = Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc | volume = 37 | issue = 4 | pages = 100441 | date = April 2024 | pmid = 38309432 | doi = 10.1016/j.modpat.2024.100441 | url = }}</ref> The majority of patients achieve complete remissions following surgery and/or radiation therapy. Its spread to extracutaneous spread is rare (10%) with 5-year overall survival and disease-specific survival of 87% and 95%, respectively.<ref name="pmid36278991"/> Primary cutaneous diffuse large B-cell lymphoma, leg type is an aggressive B-cell lymphoma that is often resistant to therapy and carries a poor prognosis,<ref name="pmid35261967">{{cite journal | vauthors = Trethewey CS, Walter HS, Alqahtani AN, Schmid R, Guttery DS, Griffin Y, Ahearne MJ, Saldanha GS, Jayne SP, Dyer MJ | title = Limitations of Monitoring Disease Progression Using Circulating Tumor DNA in Lymphoma: An Example From Primary Cutaneous DLBCL Leg-type | journal = HemaSphere | volume = 6 | issue = 3 | pages = e690 | date = March 2022 | pmid = 35261967 | pmc = 8893288 | doi = 10.1097/HS9.0000000000000690 | url = }}</ref> i.e., they have a 5-year disease-specific survival rate of 43% or 70% depending on whether their cancer cells have or do not have, respectively, inactivating [[mutation]]s in both of their ''[[CDKN2A]]'' genes.<ref name="pmid19020554">{{cite journal | vauthors = Senff NJ, Zoutman WH, Vermeer MH, Assaf C, Berti E, Cerroni L, Espinet B, de Misa Cabrera RF, Geerts ML, Kempf W, Mitchell TJ, Paulli M, Petrella T, Pimpinelli N, Santucci M, Whittaker SJ, Willemze R, Tensen CP | title = Fine-mapping chromosomal loss at 9p21: correlation with prognosis in primary cutaneous diffuse large B-cell lymphoma, leg type | journal = The Journal of Investigative Dermatology | volume = 129 | issue = 5 | pages = 1149–55 | date = May 2009 | pmid = 19020554 | doi = 10.1038/jid.2008.357 | url = }}</ref> |
Because recent studies had shown that primary cutaneous marginal zone lymphoma, which was formerly classified as a subtype of the [[MALT lymphoma]]s: '''a)''' has a distinct microscopic [[histology]] and [[Gene expression profiling in cancer|gene expression profile]]; '''b)''' spreads to extracutaneous tissue in only 4 to 8.5% of cases; '''c)''' has a [[Survival rate#Cause-specific survival and disease-specific survival|disease-specific survival]] of 99% even in patients not receiving aggressive therapy; and '''d)''' has pathological findings that overlap the benign cutaneous disorders termed [[cutaneous lymphoid hyperplasia]]. Consequently, EORTC, 2022, renamed primary cutaneous marginal zone lymphoma as primary cutaneous marginal zone lymphoproliferative disorder.<ref name="pmid36278991"/> Primary cutaneous follicle center lymphoma is also an indolent lymphoma.<ref name="pmid38309432">{{cite journal | vauthors = Medeiros LJ, Chadburn A, Natkunam Y, Naresh KN | title = Fifth Edition of the World Health Classification of Tumors of the Hematopoietic and Lymphoid Tissues: B-cell Neoplasms | journal = Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc | volume = 37 | issue = 4 | pages = 100441 | date = April 2024 | pmid = 38309432 | doi = 10.1016/j.modpat.2024.100441 | url = }}</ref> The majority of patients achieve complete remissions following surgery and/or radiation therapy. Its spread to extracutaneous spread is rare (10%) with 5-year [[Survival rate#Overal survival|overall survival]] and disease-specific survival of 87% and 95%, respectively.<ref name="pmid36278991"/> Primary cutaneous diffuse large B-cell lymphoma, leg type is an aggressive B-cell lymphoma that is often resistant to therapy and carries a poor prognosis,<ref name="pmid35261967">{{cite journal | vauthors = Trethewey CS, Walter HS, Alqahtani AN, Schmid R, Guttery DS, Griffin Y, Ahearne MJ, Saldanha GS, Jayne SP, Dyer MJ | title = Limitations of Monitoring Disease Progression Using Circulating Tumor DNA in Lymphoma: An Example From Primary Cutaneous DLBCL Leg-type | journal = HemaSphere | volume = 6 | issue = 3 | pages = e690 | date = March 2022 | pmid = 35261967 | pmc = 8893288 | doi = 10.1097/HS9.0000000000000690 | url = }}</ref> i.e., they have a 5-year disease-specific survival rate of 43% or 70% depending on whether their cancer cells have or do not have, respectively, inactivating [[mutation]]s in both of their ''[[CDKN2A]]'' genes.<ref name="pmid19020554">{{cite journal | vauthors = Senff NJ, Zoutman WH, Vermeer MH, Assaf C, Berti E, Cerroni L, Espinet B, de Misa Cabrera RF, Geerts ML, Kempf W, Mitchell TJ, Paulli M, Petrella T, Pimpinelli N, Santucci M, Whittaker SJ, Willemze R, Tensen CP | title = Fine-mapping chromosomal loss at 9p21: correlation with prognosis in primary cutaneous diffuse large B-cell lymphoma, leg type | journal = The Journal of Investigative Dermatology | volume = 129 | issue = 5 | pages = 1149–55 | date = May 2009 | pmid = 19020554 | doi = 10.1038/jid.2008.357 | url = }}</ref> |
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:* [[Intravascular large B-cell lymphoma]] |
:* [[Intravascular large B-cell lymphoma]] |
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Revision as of 14:38, 28 July 2024
| Joflaher/sandbox | |
|---|---|
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| Cutaneous diffuse large B-cell lymphoma | |
| Specialty | Dermatology/Oncology |
Cutaneous B-cell lymphomas (CBCL), also termed primary cutaneous B-cell lymphomas (PCBCL), are a group of disorders that typically present as skin lesions consisting of proliferating B-cells. B-cells are a type of lymphocyte involved in regulating immune responses. Since its original definition in 1997, PCBCL has been considered to have a varying number of subtypes by the European Organisation for Research and Treatment of Cancer, i.e., EORTC, and World Health Organization, i.e., WHO.[1] The latest revised classification of PCBCL, which was published by EORTC in 2022, lists the folloqing three main subtypes of PCBCL:[2]
- Primary cutaneous marginal zone lymphoproliferative disorder (formerly termed primary cutaneous marginal zone lymphoma)
- Primary cutaneous follicular lymphoma (also termed primary cutaneous follicular center lymphoma)
- Primary cutaneous diffuse large B-cell lymphoma, leg type
Because recent studies had shown that primary cutaneous marginal zone lymphoma, which was formerly classified as a subtype of the MALT lymphomas: a) has a distinct microscopic histology and gene expression profile; b) spreads to extracutaneous tissue in only 4 to 8.5% of cases; c) has a disease-specific survival of 99% even in patients not receiving aggressive therapy; and d) has pathological findings that overlap the benign cutaneous disorders termed cutaneous lymphoid hyperplasia. Consequently, EORTC, 2022, renamed primary cutaneous marginal zone lymphoma as primary cutaneous marginal zone lymphoproliferative disorder.[2] Primary cutaneous follicle center lymphoma is also an indolent lymphoma.[3] The majority of patients achieve complete remissions following surgery and/or radiation therapy. Its spread to extracutaneous spread is rare (10%) with 5-year overall survival and disease-specific survival of 87% and 95%, respectively.[2] Primary cutaneous diffuse large B-cell lymphoma, leg type is an aggressive B-cell lymphoma that is often resistant to therapy and carries a poor prognosis,[4] i.e., they have a 5-year disease-specific survival rate of 43% or 70% depending on whether their cancer cells have or do not have, respectively, inactivating mutations in both of their CDKN2A genes.[5]
constitute a group of diseases that occur less commonly than cutaneous T-cell lymphoma, and are characterized histologically by B-cells that appear similar to those normally found in germinal centers of lymph nodes.
Signs and symptoms
Cutaneous B-cell lymphoma appears as single or many, often strongly consistent tumors, patches, plaques, and non-ulcerated nodules. While extracutaneous spread is a possibility, the disease usually stays restricted to the skin.[6]
Diagnosis
Using a biopsy of the skin lesions, histological and cytological analyses, as well as phenotypic and genotypic studies, the diagnosis is made.[6]
See also
References
- ^ 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.
- ^ 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.
- ^ Medeiros LJ, Chadburn A, Natkunam Y, Naresh KN (April 2024). "Fifth Edition of the World Health Classification of Tumors of the Hematopoietic and Lymphoid Tissues: B-cell Neoplasms". Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc. 37 (4): 100441. doi:10.1016/j.modpat.2024.100441. PMID 38309432.
- ^ Trethewey CS, Walter HS, Alqahtani AN, Schmid R, Guttery DS, Griffin Y, Ahearne MJ, Saldanha GS, Jayne SP, Dyer MJ (March 2022). "Limitations of Monitoring Disease Progression Using Circulating Tumor DNA in Lymphoma: An Example From Primary Cutaneous DLBCL Leg-type". HemaSphere. 6 (3): e690. doi:10.1097/HS9.0000000000000690. PMC 8893288. PMID 35261967.
- ^ Senff NJ, Zoutman WH, Vermeer MH, Assaf C, Berti E, Cerroni L, Espinet B, de Misa Cabrera RF, Geerts ML, Kempf W, Mitchell TJ, Paulli M, Petrella T, Pimpinelli N, Santucci M, Whittaker SJ, Willemze R, Tensen CP (May 2009). "Fine-mapping chromosomal loss at 9p21: correlation with prognosis in primary cutaneous diffuse large B-cell lymphoma, leg type". The Journal of Investigative Dermatology. 129 (5): 1149–55. doi:10.1038/jid.2008.357. PMID 19020554.
- ^ a b Lima, Margarida (2015). "Cutaneous primary B-cell lymphomas: from diagnosis to treatment". Anais Brasileiros de Dermatologia. 90 (5). FapUNIFESP (SciELO): 687–706. doi:10.1590/abd1806-4841.20153638. hdl:10400.16/1960. ISSN 0365-0596.
Further reading
- Vitiello, Paola; Sica, Antonello; Ronchi, Andrea; Caccavale, Stefano; Franco, Renato; Argenziano, Giuseppe (2020-05-27). "Primary Cutaneous B-Cell Lymphomas: An Update". Frontiers in Oncology. 10. Frontiers Media SA. doi:10.3389/fonc.2020.00651. ISSN 2234-943X. PMC 7266949.
- Gurumurthy, Ravichandran; Mohapatra, RanjanKumar; Easow, JoseM; Mohan, Subhashini (2015). "Cutaneous B cell lymphomas: Report of two interesting cases". Indian Journal of Dermatology. 60 (2). Medknow: 176. doi:10.4103/0019-5154.152521. ISSN 0019-5154. PMC 4372911.