Large B - Cell Lymphoma of the Leg – Unfavourable Course with Rituximab/Bendamustin

  • Uwe Wollina Academic Teaching Hospital DresdenDepartment of Dermatology and Allergology
  • Nadine Schmidt Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital
  • Jacqueline Schönlebe Institute of Pathology “Georg Schmorl”, Städtisches Klinikum Dresden, Academic Teaching Hospital
  • Aleksandra Vojvodic Military Medical Academy of Belgrade, Belgrade, Serbia
  • Gesina Hansel Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital
  • André Koch Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital
  • Torello Lotti Department of Dermatology, University of Rome “G. Marconi”, Rome, Italy
Keywords: Cutaneous lymphomas, peripheral diffuse large B-cell lymphoma of the leg-type, Immunochemotherapy, Rituximab, Bedamustine, Histopathology

Abstract

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.

Downloads

Download data is not yet available.

Metrics

Metrics Loading ...

Plum Analytics Artifact Widget Block

Author Biographies

Uwe Wollina, Academic Teaching Hospital DresdenDepartment of Dermatology and Allergology

Head of Department of Dermatology and Allergology

Nadine Schmidt, Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital

Assistent

Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital

Jacqueline Schönlebe, Institute of Pathology “Georg Schmorl”, Städtisches Klinikum Dresden, Academic Teaching Hospital

Senior Advisor

Institute of Pathology “Georg Schmorlâ€, Städtisches Klinikum Dresden, Academic Teaching Hospital

Gesina Hansel, Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital

Senior Advisor

Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital

André Koch, Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital

Senior Advisor

Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital

Torello Lotti, Department of Dermatology, University of Rome “G. Marconi”, Rome, Italy

Professor of Dermatology

Department of Dermatology, University of Rome “G. Marconiâ€, Rome, Italy

References

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, Müller CSL, Sachse MM, Meiss F, Géraud 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, Dürk H, Rost A, Neise M, von Grünhagen 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

Published
2019-06-30
How to Cite
1.
Wollina U, Schmidt N, Schönlebe J, Vojvodic A, Hansel G, Koch A, Lotti T. Large B - Cell Lymphoma of the Leg – Unfavourable Course with Rituximab/Bendamustin. Open Access Maced J Med Sci [Internet]. 2019Jun.30 [cited 2020Oct.20];7(18):3006-8. Available from: https://www.id-press.eu/mjms/article/view/oamjms.2019.565

Most read articles by the same author(s)

1 2 > >>