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Annals of Hematology

, Volume 98, Issue 10, pp 2433–2436 | Cite as

Addictive response of primary cutaneous diffuse large B cell lymphoma leg type to low-dose ibrutinib

  • Annie Pang
  • Rex Au-Yeung
  • Rock Y.Y. Leung
  • Yok-Lam KwongEmail author
Letter to the Editor

Primary cutaneous diffuse large B cell lymphoma leg type (PCLBCL-LT) is a rare malignancy occurring predominantly below the knee, but may involve other cutaneous and extra-cutaneous sites [1]. Lymphoma cells typically express CD20, CD79A, BCL2, BCL6, MUM-1, and FOXP1, but not CD10 [1, 2]. Gene expression of PCLBCL-LT resembled activated B cell diffuse large B cell lymphoma (ABC-DLBCL). Consequently, genes involved in NF-κB activation were frequently mutated [3], with the MYD88 L265P mutation most prevalent, found in 60–80% of cases [3].

Outcome of PCLBCL-LT is unfavorable. Pre-rituximab, the 5-year overall survival (OS) was about 50%, with few survivals beyond 10 years [2, 4]. With rituximab-containing regimens, 5-year survivals were improved to about 75% [4]. Poor risks affecting 5-year OS included disseminated disease (solitary versus disseminated: 70% versus 0%) [5], BCL2 expression (positive versus negative: 41% versus 89%) [6], and MYD88status (L265P versus wildtype: 41% versus...

Notes

Author contribution

A. Pang: performed the investigations, wrote and approved the manuscript.

R. Au-Yeung: performed the pathologic analysis, wrote and approved the manuscript.

R. Leung: performed the pathologic analysis, wrote and approved the manuscript.

Y.L. Kwong: treated the patient, wrote and approved the manuscript.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

Not applicable.

Informed consent

Patient gave informed consent to treatment.

References

  1. 1.
    Senff NJ, Noordijk EM, Kim YH, Bagot M, Berti E, Cerroni L, Dummer R, Duvic M, Hoppe RT, Pimpinelli N, Rosen ST, Vermeer MH, Whittaker S, Willemze R, European Organization for Research and Treatment of Cancer; International Society for Cutaneous Lymphoma (2008) European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood 112(5):1600–1609CrossRefGoogle Scholar
  2. 2.
    Kodama K, Massone C, Chott A, Metze D, Kerl H, Cerroni L (2005) Primary cutaneous large B-cell lymphomas: clinicopathologic features, classification, and prognostic factors in a large series of patients. Blood 106(7):2491–2497CrossRefGoogle Scholar
  3. 3.
    Mareschal S, Pham-Ledard A, Viailly PJ, Dubois S, Bertrand P, Maingonnat C, Fontanilles M, Bohers E, Ruminy P, Tournier I, Courville P, Lenormand B, Duval AB, Andrieu E, Verneuil L, Vergier B, Tilly H, Joly P, Frebourg T, Beylot-Barry M, Merlio JP, Jardin F (2017) Identification of somatic mutations in primary cutaneous diffuse large B-cell lymphoma, leg type by massive parallel sequencing. J Invest Dermatol 137(9):1984–1994CrossRefGoogle Scholar
  4. 4.
    Grange F, Joly P, Barbe C, Bagot M, Dalle S, Ingen-Housz-Oro S, Maubec E, D'Incan M, Ram-Wolff C, Dalac S, Templier I, Esteve E, Quereux G, Machet L, Leduc M, Dereure O, Laroche L, Saiag P, Vergier B, Beylot-Barry M (2014) Improvement of survival in patients with primary cutaneous diffuse large B-cell lymphoma, leg type, in France. JAMA Dermatol 2014 150(5):535–541Google Scholar
  5. 5.
    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 (2007) 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 25(12):1581–1587CrossRefGoogle Scholar
  6. 6.
    Grange F, Petrella T, Beylot-Barry M, Joly P, D'Incan M, Delaunay M, Machet L, Avril MF, Dalac S, Bernard P, Carlotti A, Esteve E, Vergier B, Dechelotte P, Cassagnau E, Courville P, Saiag P, Laroche L, Bagot M, Wechsler J (2004) Bcl-2 protein expression is the strongest independent prognostic factor of survival in primary cutaneous large B-cell lymphomas. Blood 103(10):3662–3668CrossRefGoogle Scholar
  7. 7.
    Pham-Ledard A, Beylot-Barry M, Barbe C, Leduc M, Petrella T, Vergier B, Martinez F, Cappellen D, Merlio JP, Grange F (2014) High frequency and clinical prognostic value of MYD88 L265P mutation in primary cutaneous diffuse large B-cell lymphoma, leg-type. JAMA Dermatol 150(11):1173–1179CrossRefGoogle Scholar
  8. 8.
    Savini P, Lanzi A, Foschi FG, Marano G, Stefanini GF (2014) Lenalidomide monotherapy in relapsed primary cutaneous diffuse large B cell lymphoma-leg type. Ann Hematol 93(2):333–334CrossRefGoogle Scholar
  9. 9.
    Wilson WH, Young RM, Schmitz R, Yang Y, Pittaluga S, Wright G, Lih CJ, Williams PM, Shaffer AL, Gerecitano J, de Vos S, Goy A, Kenkre VP, Barr PM, Blum KA, Shustov A, Advani R, Fowler NH, Vose JM, Elstrom RL, Habermann TM, Barrientos JC, McGreivy J, Fardis M, Chang BY, Clow F, Munneke B, Moussa D, Beaupre DM, Staudt LM (2015) Targeting B cell receptor signaling with ibrutinib in diffuse large B cell lymphoma. Nat Med 21(8):922–926CrossRefGoogle Scholar
  10. 10.
    Barr PM, Brown JR, Hillmen P, O'Brien S, Barrientos JC, Reddy NM, Coutre S, Mulligan SP, Jaeger U, Furman RR, Cymbalista F, Montillo M, Dearden C, Robak T, Moreno C, Pagel JM, Burger JA, Suzuki S, Sukbuntherng J, Cole G, James DF, Byrd JC (2017) Impact of ibrutinib dose adherence on therapeutic efficacy in patients with previously treated CLL/SLL. Blood 129(19):2612–2615CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of MedicineQueen Mary HospitalHong KongChina
  2. 2.Department of PathologyQueen Mary HospitalHong KongChina

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