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Extranodal multifocal Rosai–Dorfman disease: response to 2-chlorodeoxyadenosine treatment

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Abstract

Rosai–Dorfman disease (RDD) or “sinus histiocytosis with massive lymphadenopathy” is a rare lymphoproliferative disorder of unknown etiology. The disease usually presents with painless lymphadenopathy with occasional extranodal involvement in various organs. We report a case of a 36-year-old man with a history of non-Hodgkin lymphoma (NHL), who recently presented with inguinal lymphadenopathy. Following the diagnosis of RDD on lymph node biopsy, he developed symptoms of spinal cord compression due to a mass lesion discovered at T6-7 vertebral level. 18F-Fluorodeoxyglucose (18FDG) positron emission tomography (PET-CT) revealed extensive disease with lung, renal and bone involvement. The patient received a short course of steroid therapy for cord compression findings and 2-chlorodeoxyadenosine (2-CdA) treatment was initiated for long-term disease control. He had a dramatic sustained response to treatment with six courses of 2-CdA. These results suggest that 2-CdA can be an effective treatment of choice and positron emission tomography with 18FDG can be used for determining the extent of disease and for follow-up in RDD.

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References

  1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1):63–70.

    CAS  PubMed  Google Scholar 

  2. Becroft DM, et al. Benign sinus histiocytosis with massive lymphadenopathy: transient immunological defects in a child with mediastinal involvement. J Clin Pathol. 1973;26(7):463–9. doi:10.1136/jcp.26.7.463.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a pseudolymphomatous benign disorder. Analysis of 34 cases. Cancer. 1972;30(5):1174–88. 10.1002/1097-0142(197211)30:5<1174::AID-CNCR2820300507>3.0.CO;2-S.

    Article  CAS  PubMed  Google Scholar 

  4. Kugler A, et al. Unusual bilateral renal histiocytosis: extranodal variant of Rosai–Dorfman disease. J Urol. 1997;157(3):942. doi:10.1016/S0022-5347(01)65089-4.

    Article  CAS  PubMed  Google Scholar 

  5. Krishnan A, Nassar A, Nieh PT. Rosai–Dorfman disease presenting as extranodal renal mass. Urology. 2005;66(6):1319. doi:10.1016/j.urology.2005.06.103.

    Article  PubMed  Google Scholar 

  6. Al-Saad K, et al. Extranodal Rosai–Dorfman disease with multifocal bone and epidural involvement causing recurrent spinal cord compression. Pediatr Dev Pathol. 2005;8(5):593–8. doi:10.1007/s10024-005-8102-6.

    Article  PubMed  Google Scholar 

  7. Pulsoni A, et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease): report of a case and literature review. Am J Hematol. 2002;69(1):67–71. doi:10.1002/ajh.10008.

    Article  PubMed  Google Scholar 

  8. Hargett C, Bassett T. Atypical presentation of sinus histiocytosis with massive lymphadenopathy as an epidural spinal cord tumor: a case presentation and literature review. J Spinal Disord Tech. 2005;18(2):193–6. doi:10.1097/01.bsd.0000137156.44689.c0.

    Article  PubMed  Google Scholar 

  9. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7(1):19–73.

    CAS  PubMed  Google Scholar 

  10. Lossos IS, et al. Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease): report of a patient with isolated renotesticular involvement after cure of non-Hodgkin’s lymphoma. Ann Hematol. 1997;74(1):41–4. doi:10.1007/s002770050254.

    Article  CAS  PubMed  Google Scholar 

  11. Shoda H, et al. Sinus histiocytosis with massive lymphadenopathy associated with malignant lymphoma. Intern Med. 2004;43(8):741–5. doi:10.2169/internalmedicine.43.741.

    Article  PubMed  Google Scholar 

  12. Lu D, et al. Sinus histiocytosis with massive lymphadenopathy and malignant lymphoma involving the same lymph node: a report of four cases and review of the literature. Mod Pathol. 2000;13(4):414–9. doi:10.1038/modpathol.3880071.

    Article  CAS  PubMed  Google Scholar 

  13. Kubota R, et al. Intratumoral distribution of fluorine-18-fluorodeoxyglucose in vivo: high accumulation in macrophages and granulation tissues studied by microautoradiography. J Nucl Med. 1992;33(11):1972–80.

    CAS  PubMed  Google Scholar 

  14. Menzel C, et al. PET imaging of Rosai–Dorfman disease: correlation with histopathology and ex-vivo beta-imaging. Arch Dermatol Res. 2003;295(7):280–3. doi:10.1007/s00403-003-0431-6.

    Article  PubMed  Google Scholar 

  15. Yu JQ, et al. Demonstration of increased FDG activity in Rosai–Dorfman disease on positron emission tomography. Clin Nucl Med. 2004;29(3):209–10. doi:10.1097/01.rlu.0000114018.07676.15.

    Article  PubMed  Google Scholar 

  16. Lim R, et al. FDG PET of Rosai–Dorfman disease of the thymus. AJR Am J Roentgenol. 2004;182(2):514.

    Article  PubMed  Google Scholar 

  17. Baildam EM, et al. Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease): response to acyclovir. J R Soc Med. 1992;85(3):179–80.

    CAS  PubMed  PubMed Central  Google Scholar 

  18. Chan CC, Chu CY. Dapsone as a potential treatment for cutaneous Rosai–Dorfman disease with neutrophilic predominance. Arch Dermatol. 2006;142(4):428–30. doi:10.1001/archderm.142.4.428.

    Article  PubMed  Google Scholar 

  19. Giudicelli J, et al. Destombes-Rosai–Dorfman syndrome: 2 uncommon clinical forms. Rev Med Interne. 1994;15(12):834–7. doi:10.1016/S0248-8663(05)82841-3.

    Article  CAS  PubMed  Google Scholar 

  20. Foucar E, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. Arch Otolaryngol. 1978;104(12):687–93.

    Article  CAS  PubMed  Google Scholar 

  21. Ocheni S, et al. Usefulness of oral corticosteroid in Rosai–Dorfman disease. Eur J Cancer Care Engl. 2007;16(3):286–8. doi:10.1111/j.1365-2354.2006.00746.x.

    Article  CAS  PubMed  Google Scholar 

  22. Sita G, et al. Rosai–Dorfman syndrome with extranodal localizations and response to glucocorticoids: a case report. Haematologica. 1996;81(2):165–7.

    CAS  PubMed  Google Scholar 

  23. Raveenthiran V, et al. Rosai–Dorfman disease: report of a 3-year-old girl with critical review of treatment options. Eur J Pediatr Surg. 2003;13(5):350–4. doi:10.1055/s-2003-43578.

    Article  CAS  PubMed  Google Scholar 

  24. Palomera L, et al. Sinus histiocytosis with massive lymphadenopathy: complete response to low-dose interferon-alpha. J Clin Oncol. 1997;15(5):2176.

    Article  CAS  PubMed  Google Scholar 

  25. Silvers AR, Som PM, Meyer RJ. Egg shell nodal calcification in a patient with sinus histiocytosis with massive lymphadenopathy treated with interferon. AJNR Am J Neuroradiol. 1996;17(2):361–3.

    CAS  PubMed  Google Scholar 

  26. Mebazaa A, et al. Extensive purely cutaneous Rosai–Dorfman disease responsive to acitretin. Int J Dermatol. 2007;46(11):1208–10. doi:10.1111/j.1365-4632.2007.03234.x.

    Article  PubMed  Google Scholar 

  27. Chang LY, Kuo TT, Chan HL. Extranodal Rosai–Dorfman disease with cutaneous, ophthalmic and laryngeal involvement: report of a case treated with isotretinoin. Int J Dermatol. 2002;41(12):888–91. doi:10.1046/j.1365-4362.2002.01675.x.

    Article  PubMed  Google Scholar 

  28. Tjiu JW, Hsiao CH, Tsai TF. Cutaneous Rosai–Dorfman disease: remission with thalidomide treatment. Br J Dermatol. 2003;148(5):1060–1. doi:10.1046/j.1365-2133.2003.05311.x.

    Article  PubMed  Google Scholar 

  29. Utikal J, et al. Imatinib as a treatment option for systemic non-Langerhans cell histiocytoses. Arch Dermatol. 2007;143(6):736–40. doi:10.1001/archderm.143.6.736.

    Article  CAS  PubMed  Google Scholar 

  30. Pagel JM, et al. Therapeutic use of Rituximab for sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease). Am J Hematol. 2007;82(12):1121–2. doi:10.1002/ajh.21024.

    Article  PubMed  Google Scholar 

  31. Horneff G, et al. Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease): response to methotrexate and mercaptopurine. Med Pediatr Oncol. 1996;27(3):187–92. 10.1002/(SICI)1096-911X(199609)27:3<;187::AID-MPO10>;3.0.CO;2-D.

    Article  CAS  PubMed  Google Scholar 

  32. Jabali Y, Smrcka V, Pradna J. Rosai–Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report. Int J Surg Pathol. 2005;13(3):285–9. doi:10.1177/106689690501300311.

    Article  CAS  PubMed  Google Scholar 

  33. Inoue S, Onwuzurike N. Venorelbine and methotrexate for the treatment of Rosai–Dorfman disease. Pediatr Blood Cancer. 2005;45(1):84–5. doi:10.1002/pbc.20361.

    Article  PubMed  Google Scholar 

  34. Tasso M, et al. Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) treated with 2-chlorodeoxyadenosine. Pediatr Blood Cancer. 2006;47(5):612–5. doi:10.1002/pbc.20668.

    Article  CAS  PubMed  Google Scholar 

  35. Rodriguez-Galindo C, et al. Treatment of children with Langerhans cell histiocytosis with 2-chlorodeoxyadenosine. Am J Hematol. 2002;69(3):179–84. doi:10.1002/ajh.10053.

    Article  CAS  PubMed  Google Scholar 

  36. Rodriguez-Galindo C, et al. Extranodal Rosai–Dorfman disease in children. J Pediatr Hematol Oncol. 2004;26(1):19–24. doi:10.1097/00043426-200401000-00007.

    Article  PubMed  Google Scholar 

  37. Aouba A, et al. Dramatic clinical efficacy of cladribine in Rosai–Dorfman disease and evolution of the cytokine profile: towards a new therapeutic approach. Haematologica. 2006;91(12 Suppl.):ECR52.

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey

    Ceyla Konca & Müge Deger

  2. Division of Hematology, Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey

    Zübeyde N. Özkurt, Zeynep Akı & Münci Yağcı

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  1. Ceyla Konca
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  2. Zübeyde N. Özkurt
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  3. Müge Deger
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  4. Zeynep Akı
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  5. Münci Yağcı
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Correspondence to Ceyla Konca.

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Konca, C., Özkurt, Z.N., Deger, M. et al. Extranodal multifocal Rosai–Dorfman disease: response to 2-chlorodeoxyadenosine treatment. Int J Hematol 89, 58–62 (2009). https://doi.org/10.1007/s12185-008-0192-2

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  • DOI: https://doi.org/10.1007/s12185-008-0192-2

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