International Journal of Hematology

, Volume 108, Issue 3, pp 329–334 | Cite as

EBV-positive PEL-like lymphoma that developed in the course of antisynthetase syndrome treated with tacrolimus

  • Kimi OkadaEmail author
  • Shoji Asakura
  • Tomofumi Yano
  • Takumi Kishimoto
Case Report


Primary effusion lymphoma (PEL) is a rare type of extranodal lymphoma, typically of a B-cell origin, which presents as lymphomatous effusion with no nodal enlargement or tumor masses. The development PEL is universally associated with human herpes virus-8 (HHV-8) infection. Cases of HHV-8-negative primary lymphomatous effusion have recently been reported and referred to as HHV-8-unrelated PEL-like lymphoma. Some cases of this disease have been reported in iatrogenic immunocompromised patients. The mechanisms responsible for the inhibitory effects of the discontinuation of immunosuppressants other than methotrexate (MTX) against the disease, which have been demonstrated for MTX-associated lymphoproliferative disorders, have not yet been elucidated. We describe a case of PEL-like lymphoma that developed in the course of antisynthetase syndrome and was treated with tacrolimus. A single dose of systemic chemotherapy did not improve lymphomatous effusion, whereas the discontinuation of tacrolimus resulted in the long-term remission of this disease.


EBV PEL-like lymphoma Tacrolimus 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Said J, Cesarman E. Primary effusion lymphoma. In: Swerdblow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: International Agency for Research on Cancer; 2008. p. 260–1.Google Scholar
  2. 2.
    Kobayashi Y, Kamitsuji Y, Kuroda J, Tsunoda S, Uoshima N, Kimura S, et al. Comparison of human herpes virus 8 related primary effusion lymphoma with human herpes virus 8 unrelated primary effusion lymphoma-like lymphoma on the basis of HIV: report of 2 cases and review of 212 cases in the literature. Acta Haematol. 2007;117:132–44.CrossRefPubMedGoogle Scholar
  3. 3.
    Carbone A, Gloghini A. PEL and HHV8-unrelated effusion lymphomas: classification and diagnosis. Cancer. 2008;114:225–7.CrossRefPubMedGoogle Scholar
  4. 4.
    Wu W, Youm W, Rezk AS, Zhao X. Human herpesvirus 8-unrelated primary effusion lymphoma-like lymphoma. report of a rare case and review of 54 cases in the literature. Am J Clin Pathol. 2013;140:258–73.CrossRefPubMedGoogle Scholar
  5. 5.
    Terasaki Y, Yamamoto H, Kiyokawa H, Okumura H, Saito K, Ichinohasama R, et al. Disappearance of malignant cells by effusion drainage alone in two patients with HHV-8-unrelated HIV negative primary effusion lymphoma-like lymphoma. Int J Hematol. 2011;94:279–84.CrossRefPubMedGoogle Scholar
  6. 6.
    Fonacier L, Charlesworth NE, Spergel MJ, Leung MYD. The black box warning for topical calcineurin inhibitors: looking outside the box. Ann Allergy Asthma Immunol. 2006;97:117–20.CrossRefPubMedGoogle Scholar
  7. 7.
    Sekiguchi Y, Shimada A, Imai H, Wakabayashi M, Sugimoto K, Nakamura N, et al. Epstein-Barr virus-negative, CD5-positive diffuse large B-cell lymphoma developing after treatment with oral tacrolimus for mixed connective tissue disease: a case report and review of the literature. J Clin Exp Hematol. 2012;52:211–8.CrossRefGoogle Scholar
  8. 8.
    Fukasawa M, Akazawa Y, Kasugai S, Mikami K, Saito Y, Akutsu M, et al. Four cases of other iatrogenic immunodeficiency-associated lymphoproliferative disorders in the head and neck region. Nippon Jibiinkoka Gakkai Kaiho (Tokyo). 2016;119:741–9.CrossRefGoogle Scholar
  9. 9.
    Inui Y, Matsuoka H, Yakushijin K, Okumura A, Shimada T, Yano S. Methotrexate-associated lymphoproliferative disorders: management by watchful waiting and observation of early lymphocyte recovery after methotrexate withdrawal. Leuk Lymphoma. 2015;56:3045–51.CrossRefPubMedGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2018

Authors and Affiliations

  • Kimi Okada
    • 1
    Email author
  • Shoji Asakura
    • 1
  • Tomofumi Yano
    • 1
  • Takumi Kishimoto
    • 1
  1. 1.Internal Medicine DepartmentOkayama Rousai HospitalOkayama cityJapan

Personalised recommendations