Large B - Cell Lymphoma of the Leg â€“ Unfavourable Course with Rituximab/Bendamustin
BACKGROUND: Cutaneous B-cell lymphomas represent about 25% of all cutaneous lymphomas. Peripheral diffuse large B-cell lymphoma of the leg type is the most aggressive subtype seen mainly in elderly patients. Treatment is not standardised.
CASE REPORT: An 87-year-old female patient was presented in May 2018 because of the development of painless subcutaneous nodules on the legs since late 2017. On examination, we observed up to 5 cm large erythematous nodules on the legs and a smaller plaque in the left submammary fold. The histology of a skin demonstrated tumour infiltrate that was separated from the overlying epidermis by a grenz zone. It consisted of densely packed, blastoid lymphocytic cells with numerous, and some atypical mitoses. The cells were positive for CD20, CD79A and CD5. Almost 100% of the cells were labelled with Ki67. The diagnosis of a diffuse large B-cell lymphoma (PCLBCL-LT) of the leg was confirmed. Histologic analysis of a bone marrow biopsy demonstrated a hypercellular bone marrow without malignant lymphatic infiltrates. Diagnostic ultrasound of cervical nodes and computerised tomography (CT) scans (native and with contrast medium) of head, neck and trunk excluded an extracutaneous manifestation of the PCLBCL-LT. Treatment with rituximab plus bendamustibe was initiated, but tumour progress was noted after the second course. Suggested palliative therapy with radiation and rituximab was refused. The patient died 7 months after diagnosis.
CONCLUSIONS: Although some trials suggested a beneficial effect of immuno-chemotherapy, the prognosis of (PCLBCL-LT) remains poor. Standardised treatment is missing due to the relative rarity of this malignancy.
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Goyal A, LeBlanc RE, Carter JB. Cutaneous B-Cell Lymphoma. Hematol Oncol Clin North Am. 2019; 33(1):149-161. https://doi.org/10.1016/j.hoc.2018.08.006 PMid:30497672
Menguy S, Beylot-Barry M, Marie P, Pham Ledard A, Frison E, Comoz F, Battistella M, Szablewski V, Balme B, Croue A, Franck F, Ortonne N, Tournier E, Lamant L, Carlotti A, De Muret A, Le Gall F, Lorton MH, Merlio JP, Vergier B. Primary Cutaneous Large B-Cell Lymphomas: relevance of the 2017 WHO Classification. Histopathology. 2019;
Felcht M, Klemke CD, Nicolay JP, Weiss C, Assaf C, Wobser M, Schlaak M, Hillen U, Moritz R, Tantcheva-Poor I, Nashan D, Beyer M, Dippel E, MÃ¼ller CSL, Sachse MM, Meiss F, GÃ©raud C, Marx A, Goerdt S, Geissinger E, Kempf W. Primary cutaneous diffuse large B-cell lymphoma, NOS and leg type: Clinical, morphologic and prognostic differences. J Dtsch Dermatol Ges. 2019; 17(3):275-285. https://doi.org/10.1111/ddg.13773
Schrader AMR, Jansen PM, Vermeer MH, Kleiverda JK, Vermaat JSP, Willemze R. High Incidence and Clinical Significance of MYC Rearrangements in Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type. Am J Surg Pathol. 2018; 42(11):1488-1494. https://doi.org/10.1097/PAS.0000000000001132 PMid:30113335
Paulli M, Lucioni M, Maffi A, Croci GA, Nicola M, Berti E. Primary cutaneous diffuse large B-cell lymphoma (PCDLBCL), leg-type and other: an update on morphology and treatment. G Ital Dermatol Venereol. 2012; 147(6):589-602.
Wilcox RA. Cutaneous B-cell lymphomas: 2015 update on diagnosis, risk-stratification, and management. Am J Hematol. 2015; 90(1):73-6. https://doi.org/10.1002/ajh.23863 PMid:25535037
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. Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases. Arch Dermatol. 2007; 143(9):1144-50. https://doi.org/10.1001/archderm.143.9.1144 PMid:17875875
Senff NJ, Hoefnagel JJ, Jansen PM, Vermeer MH, van Baarlen J, Blokx WA, Canninga-van Dijk MR, Geerts ML, Hebeda KM, Kluin PM, Lam KH, Meijer CJ, Willemze R. 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):1581-7. https://doi.org/10.1200/JCO.2006.09.6396 PMid:17353548
Hamilton SN, Wai ES, Tan K, Alexander C, Gascoyne RD, Connors JM. Treatment and outcomes in patients with primary cutaneous B-cell lymphoma: the BC Cancer Agency experience. Int J Radiat Oncol Biol Phys. 2013; 87(4):719-25. https://doi.org/10.1016/j.ijrobp.2013.07.019 PMid:24001373
Rummel MJ, Al-Batran SE, Kim SZ, Welslau M, Hecker R, Kofahl-Krause D, Josten KM, DÃ¼rk H, Rost A, Neise M, von GrÃ¼nhagen U, Chow KU, Hansmann ML, Hoelzer D, Mitrou PS. Bendamustine plus rituximab is effective and has a favorable toxicity profile in the treatment of mantle cell and low-grade non-Hodgkin's lymphoma. J Clin Oncol. 2005; 23(15):3383-9. https://doi.org/10.1200/JCO.2005.08.100 PMid:15908650
Vacirca JL, Acs PI, Tabbara IA, Rosen PJ, Lee P, Lynam E. Bendamustine combined with rituximab for patients with relapsed or refractory diffuse large B cell lymphoma. Ann Hematol. 2014; 93(3):403-9. https://doi.org/10.1007/s00277-013-1879-x PMid:23955074 PMCid:PMC3918114
Rigacci L, Puccini B, Cortelazzo S, Gaidano G, Piccin A, D'Arco A, Freilone R, Storti S, Orciuolo E, Zinzani PL, Zaja F, Bongarzoni V, Balzarotti M, Rota-Scalabrini D, Patti C, Gobbi M, Carpaneto A, Liberati AM, Bosi A, Iannitto E. Bendamustine with or without rituximab for the treatment of heavily pretreated non-Hodgkin's lymphoma patients: A multicenter retrospective study on behalf of the Italian Lymphoma Foundation (FIL). Ann Hematol. 2012; 91(7):1013-22. https://doi.org/10.1007/s00277-012-1422-5 PMid:22349722
Deng AL, Kim YR, Lichtenstein EA, O'Connor OA, Deng C. Combination of ibrutinib and chemotherapy produced a durable remission in multiply relapsed diffuse large B-cell lymphoma leg type with mutant MYD88 and wildtype CD79. Haematologica. 2017; 102(7):e275-e277. https://doi.org/10.3324/haematol.2016.161893 PMid:28341730 PMCid:PMC5566054
Beylot-Barry M, Mermin D, Maillard A, Bouabdallah R, Bonnet N, Duval-Modeste AB, Mortier L, Ingen-Housz-Oro S, Ram-Wolff C, Barete S, Dalle S, Maubec E, Quereux G, Templier I, Bagot M, Grange F, Joly P, Vergier B, Vially PJ, Gros A, Pham-Ledard A, Frison E, Merlio JP. A Single-Arm Phase II Trial of Lenalidomide in Relapsing or Refractory Primary Cutaneous Large B-Cell Lymphoma, Leg Type. J Invest Dermatol. 2018; 138(9):1982-1989. https://doi.org/10.1016/j.jid.2018.03.1516 PMid:29596904
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