English Polski
Vol 29, No 1 (2023)
Case report
Published online: 2023-02-28

open access

Page views 1283
Article views/downloads 312
Get Citation

Connect on Social Media

Connect on Social Media

Unilateral lower extremity lymphedema as a first symptom of primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT)

Aleksander Truszyński1, Jakub Brodowski1, Michał Jeleń2, Mateusz Ollik2, Justyna Putek1, Monika Sowicz1, Tomasz Wróbel3, Andrzej Szuba1, Angelika Chachaj1
Acta Angiologica 2023;29(1):30-36.

Abstract

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), is a rare and aggressive variant of extranodal lymphoma. We report the case of a 64-year-old woman with unilateral lymphedema of the lower limb as the first and only symptom of PCDLBCL-LT for six months. Violaceous nodules were the second symptom and they progressively developed on the edematous calf. Initially, they were diagnosed as warty overgrowths, which are common skin changes in the course of chronic lymphedema. The lack of improvement in the violaceous nodules after compression therapy prompted to perform a skin biopsy. Histopathological evaluation revealed the presence of PCDLBCL-LT. In this article, we want to highlight the challenges of making a diagnosis of PCDLBCL- -LT. To our knowledge, no other study has reported on lymphedema as an initial symptom of PCDLBCL-LT.

Article available in PDF format

View PDF Download PDF file

References

  1. Willemze R, Cerroni L, Kempf W, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood. 2019; 133(16): 1703–1714.
  2. Senff NJ, Hoefnagel JJ, Jansen PM, et al. Reclassification of 300 primary cutaneous B-Cell lymphomas according to the new WHO-EORTC classification for cutaneous lymphomas: comparison with previous classifications and identification of prognostic markers. J Clin Oncol. 2007; 25(12): 1581–1587.
  3. Grange F, Beylot-Barry M, Courville P, et al. Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases. Arch Dermatol. 2007; 143(9): 1144–1150.
  4. Grange F, Bekkenk MW, Wechsler J, et al. Prognostic factors in primary cutaneous large B-cell lymphomas: a European multicenter study. J Clin Oncol. 2001; 19(16): 3602–3610.
  5. Hallermann C, Kaune KM, Gesk S, et al. Molecular cytogenetic analysis of chromosomal breakpoints in the IGH, MYC, BCL6, and MALT1 gene loci in primary cutaneous B-cell lymphomas. J Invest Dermatol. 2004; 123(1): 213–219.
  6. Kodama K, Massone C, Chott A, et al. Primary cutaneous large B-cell lymphomas: clinicopathologic features, classification, and prognostic factors in a large series of patients. Blood. 2005; 106(7): 2491–2497.
  7. Gokdemir G, Ari S, Altunay I, et al. Primary cutaneous diffuse large B-cell lymphoma of the leg, with an atypical clinical picture of verrucous plaques associated with stasis dermatitis. Clin Exp Dermatol. 2010; 35(3): e87–e89.
  8. Belousova IE, Vanecek T, Skreg SV, et al. Unusual clinicopathological presentation of primary cutaneous diffuse large B-cell lymphoma, leg type, with multiple nodules and widespread garland-like lesions. Am J Dermatopathol. 2009; 31(4): 370–374.
  9. Huang CT, Yang WC, Liu YC, et al. Primary cutaneous diffuse large B-cell lymphoma, leg type, with unusual clinical presentation of bluish-reddish multicolored rainbow pattern. J Clin Oncol. 2011; 29(17): e497–e498.
  10. Massone C, Fink-Puches R, Wolf I, et al. Atypical clinicopathologic presentation of primary cutaneous diffuse large B-cell lymphoma, leg type. J Am Acad Dermatol. 2015; 72(6): 1016–1020.
  11. Sisto K, Khachemoune A. Elephantiasis nostras verrucosa: a review. Am J Clin Dermatol. 2008; 9(3): 141–146.
  12. Executive Committee. The diagnosis and treatment of peripheral lymphedema: 2016 Consensus Document of the International Society of Lymphology. Lymphology. 2016; 49(4): 170–184.
  13. Long V, Liang MW, Lee JS, et al. Two instructive cases of primary cutaneous diffuse large B-cell lymphoma (leg type) mimicking cellulitis and sporotrichosis. JAAD Case Rep. 2020; 6(9): 815–818.
  14. Tiwari A, Cheng KS, Button M, et al. Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surg. 2003; 138(2): 152–161.
  15. Szuba A, Rockson SG. Lymphedema: classification, diagnosis and therapy. Vasc Med. 1998; 3(2): 145–156.
  16. Hao K, Xin J, Sun Y, et al. Lymphedema As The Initial Symptom of Lymphoma. 01 December 2021, PREPRINT (Version 1) available at Research Square. .
  17. Hawkins KA, Amorosi EL, Silber R. Unilateral leg edema. A symptom of lymphoma. JAMA. 1980; 244(23): 2640–2641.
  18. Ruocco V, Schwartz RA, Ruocco E. Lymphedema: an immunologically vulnerable site for development of neoplasms. J Am Acad Dermatol. 2002; 47(1): 124–127.
  19. Sun L, Sun Y, Xin W, et al. A case of CD5-positive primary cutaneous diffuse large B-cell lymphoma, leg type secondary to chronic lymphedema. Am J Dermatopathol. 2022; 44(3): 179–182.
  20. Marasca C, Fabbrocini G, Cinelli E, et al. A case of a primary cutaneous diffuse large B-cell lymphoma, leg type. Int Wound J. 2020; 17(2): 514–515.
  21. Hoshida Y, Xu JX, Fujita S, et al. Lymphoproliferative disorders in rheumatoid arthritis: clinicopathological analysis of 76 cases in relation to methotrexate medication. J Rheumatol. 2007; 34(2): 322–331.
  22. Ichikawa A, Arakawa F, Kiyasu J, et al. Methotrexate/iatrogenic lymphoproliferative disorders in rheumatoid arthritis: histology, Epstein-Barr virus, and clonality are important predictors of disease progression and regression. Eur J Haematol. 2013; 91(1): 20–28.
  23. Delaleu J, Maubec E, Rodrigues F, et al. Methotrexate-induced primary cutaneous diffuse large B-cell lymphoma in patients with erythrodermic cutaneous T-cell lymphoma. Acta Derm Venereol. 2020; 100(15): adv00226.
  24. Georgescu L, Paget SA. Lymphoma in patients with rheumatoid arthritis: what is the evidence of a link with methotrexate? Drug Saf. 1999; 20(6): 475–487.
  25. Miyazaki T, Fujimaki K, Shirasugi Y, et al. Remission of lymphoma after withdrawal of methotrexate in rheumatoid arthritis: relationship with type of latent Epstein-Barr virus infection. Am J Hematol. 2007; 82(12): 1106–1109.
  26. Rizzi R, Curci P, Delia M, et al. Spontaneous remission of "methotrexate-associated lymphoproliferative disorders" after discontinuation of immunosuppressive treatment for autoimmune disease. Review of the literature. Med Oncol. 2009; 26(1): 1–9.
  27. Grange F, Joly P, Barbe C, et al. Improvement of survival in patients with primary cutaneous diffuse large B-cell lymphoma, leg type, in France. JAMA Dermatol. 2014; 150(5): 535–541.