Richter Syndrome Presenting as Subcutaneous Nodules and a Dermal Plaque.
Journal
The American Journal of dermatopathology
ISSN: 1533-0311
Titre abrégé: Am J Dermatopathol
Pays: United States
ID NLM: 7911005
Informations de publication
Date de publication:
23 Apr 2024
23 Apr 2024
Historique:
medline:
22
4
2024
pubmed:
22
4
2024
entrez:
22
4
2024
Statut:
aheadofprint
Résumé
Richter syndrome (RS) describes a phenomenon in which a patient with chronic lymphocytic leukemia (CLL) develops an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). Reports of cutaneous RS remain exceedingly rare. We report a 61-year-old woman with relapsed/refractory CLL presenting with several subcutaneous nodules on her arms and legs and a single dermal plaque on her abdomen. Skin biopsy revealed a diagnosis of DLBCL, ABC-type, and her clinical status rapidly deteriorated following diagnosis. We review the variety of clinical presentations of cutaneous RS, its association with CLL, risk factors for RS development in CLL patients, and the distinctive histopathologic and immunophenotypic features of DLBCL. We hope to highlight the importance of prompt skin biopsy in patients with CLL presenting with progressive skin lesions and increase awareness of this aggressive clinical syndrome.
Identifiants
pubmed: 38648045
doi: 10.1097/DAD.0000000000002720
pii: 00000372-990000000-00338
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.
Déclaration de conflit d'intérêts
The authors declare no conflicts of interest.
Références
Mouhssine S, Gaidano G. Richter syndrome: from molecular pathogenesis to druggable targets. Cancers (Basel). 2022;14:4644.
Rogers TS, Gardner JA, Devitt KA. High-grade B-Cell lymphoma with MYC and BCL6 rearrangements associated with Richter transformation of chronic lymphocytic leukemia. Autops Case Rep. 2019;9:e2019090.
Salem DA, Stetler-Stevenson M. Clinical flow-cytometric testing in chronic lymphocytic leukemia. Methods Mol Biol. 2019;2032:311–321.
Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127:2375–2390.
Hallek M, Cheson BD, Catovsky D, et al. Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the international workshop on chronic lymphocytic leukemia updating the National Cancer Institute–Working Group 1996 guidelines. Blood. 2008;111:5446–5456.
Inamdar KV, Bueso-Ramos CE. Pathology of chronic lymphocytic leukemia: an update. Ann Diagn Pathol. 2007;11:363–389.
Parikh SA, Kay NE, Shanafelt TD. How we treat Richter syndrome. Blood. 2014;123:1647–1657.
Duong T, Grange F, Auffret N, et al. Cutaneous Richter's syndrome, prognosis, and clinical, histological and immunohistological patterns: report of four cases and review of the literature. Dermatology (Basel, Switzerland). 2010;220:226–233.
Hans CP, Weisenburger DD, Greiner TC, et al. Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray. Blood. 2004;103:275–282.
Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia. 2022;36:1720–1748.
Wang Y, Tschautscher MA, Rabe KG, et al. Clinical characteristics and outcomes of Richter transformation: experience of 204 patients from a single center. Haematologica. 2020;105:765–773.
Kluk J, Moonim M, Duran A, et al. Cutaneous Richter syndrome: a better place to transform? Br J Dermatol. 2015;172:513–521.
Szukalski Ł, Sysakiewicz-Buda M, Męcińska-Jundziłł K, et al. Cutaneous manifestation of chronic lymphocytic leukemia: a case report and a literature review. Postepy Dermatol Alergol. 2019;36:778–780.
Kilaru S, Panda SS, Mishra S, et al. Cutaneous involvement in diffuse large B cell lymphoma at presentation: report of two rare cases and literature review. J Egypt Natl Cancer Inst. 2021;33:25.
Vitiello P, Sica A, Ronchi A, et al. Primary cutaneous B-cell lymphomas: an update. Front Oncol. 2020;10:651. Available at: https://www.frontiersin.org/articles/10.3389/fonc.2020.00651. Accessed January 6, 2023.
Castillo JJ, Winer ES, Olszewski AJ. Sites of extranodal involvement are prognostic in patients with diffuse large B-cell lymphoma in the rituximab era: an analysis of the Surveillance, Epidemiology and End Results database. Am J Hematol. 2014;89:310–314.
Lloret-Ruiz C, Molés-Poveda P, Barrado-Solís N, et al. Diffuse systemic large B-cell lymphoma with secondary skin involvement. Actas Dermosifiliogr. 2015;106:685–687.
Lee W, Won K, Won C, et al. Secondary cutaneous diffuse large B-cell lymphoma has a higher International Prognostic Index Score and worse prognosis than diffuse large B-cell lymphoma, leg type. Acta Derm Venerol. 2016;96:245–250.
Parikh SA, Shanafelt TD. Risk factors for Richter syndrome in chronic lymphocytic leukemia. Curr Hematol Malig Rep. 2014;9:294–299.
Rossi D, Cerri M, Capello D, et al. Biological and clinical risk factors of chronic lymphocytic leukaemia transformation to Richter syndrome. Br J Haematol. 2008;142:202–215.
El Hussein S, Medeiros LJ, Lyapichev KA, et al. Immunophenotypic and genomic landscape of Richter transformation diffuse large B-cell lymphoma. Pathology. 2023;55:514–524.
Riedell PA, Smith SM. Double hit and double expressors in lymphoma: definition and treatment. Cancer. 2018;124:4622–4632.
Rungwittayatiwat S, Boonsakan P, Chantrathammachart P, et al. Treatment outcomes and clinical relevance in patients with double expressor DLBCL. Mediterr J Hematol Infect Dis. 2021;13:e2021063.
Green TM, Young KH, Visco C, et al. Immunohistochemical double-hit score is a strong predictor of outcome in patients with diffuse large B-cell lymphoma treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone. J Clin Oncol. 2012;30:3460–3467.
Wang L, Llorente C, Hartmann P, et al. Methods to determine intestinal permeability and bacterial translocation during liver disease. J Immunol Methods. 2015;421:44–53.
Scandurra M, Rossi D, Deambrogi C, et al. Genomic profiling of Richter's syndrome: recurrent lesions and differences with de novo diffuse large B-cell lymphomas. Hematol Oncol. 2010;28:62–67.