Sinus histiocytosis with massive lymphadenopathy: FDG-PET/CT documented partial remission after treatment with 2-chlorodeoxyadenosine
Authors:
Zdeněk Adam 1; Jiří Mašlaň 2; Leoš Křen 3; Roman Kodet 4; Renata Koukalová 5; Zdeněk Řehák 5; Libor Červinek 1; Luděk Pour 1; Marta Krejčí 1; Viera Sandecká 1; Zdeněk Král 1; Jiří Mayer 1
Authors‘ workplace:
Interní hematologická a onkologická klinika LF MU a FN Brno, pracoviště Bohunice
1; Oddělení patologie Nemocnice Třebíč
2; Ústav patologie LF MU a FN Brno, pracoviště Bohunice
3; Ústav patologie a molekulární medicíny 2. LF UK a FN v Motole, Praha
4; Oddělení nukleární medicíny – centrum PET a Regionální centrum aplikované molekulární onkologie (RECAMO) Masarykova onkologického ústavu Brno
5
Published in:
Vnitř Lék 2016; 62(6): 491-499
Category:
Case Reports
Overview
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a very rare disease belonging to a group of histiocytoses (more precisely non-Langerhans cell histiocytoses). Rosai-Dorfman disease is characterised by the presence of atypical histiocytic cells in the sinuses of lymph nodes or in the extranodal lymphoid tissue, absorbing lymphocytes and plasma cells. The structure and function of the absorbed cells is not impaired and they can leave histiocytes as viable cells. This effect is called emperipolesis, whereas ingestion of cells with their destruction is called phagocytosis. In our text we describe a patient with this disease located, characteristically, in supraclavicular lymph nodes, but also in mediastinal lymph nodes. Along with lymphadenopathy skin alterations appeared which were both clinically and histologically described as eczema dermatitis. At the same time as lymphadenopathy also strong headaches started which the patient had never suffered before. Within the first-line treatment prednisone was administered, but no effect was achieved. 2-chlorodeoxyadenosine in 5 mg/m2 s. c. dose was used in the second-line treatment, for 5 successive days in monthly intervals. There were four cycles of this treatment administered overall. Therapy was tolerated without any manifestations of toxicity. Already after the 1st cycle skin alterations as well as headaches entirely disappeared. To assess the effect of treatment the PET/CT examination with 18F-fluorodeoxyglucose (FDG-PET/CT) was made. After 4 cycles of treatment the mediastinal lymph nodes diminished to a physiological size and the accumulation of fluorodeoxyglucose in them was assessed as physiological. Lymphadenopathy in the neck area also significantly diminished by 50–75 % and the accumulation of fluorodeoxyglucose was reduced as well, though it did not reach the norm. Therefore we evaluate the effect of treatment as a partial remission with complete disappearance of skin alterations and headaches. The cause of the eczema and headaches has not been clarified, however considering the same time of their arising and then disappearance after the application of 2-chlorodeoxyadenosine the causal connection with Rosai-Dorfman disease is likely.
Key words:
Castleman’s disease – lenalidomide – Rosai-Dorfman disease – rituximab – sinus lymphadenopathy with massive lymphadenopathy – thalidomide – 2-chlorodeoxyadenosine
Sources
1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969; 87(1): 63–70.
2. La Barge DV, Salzman KL, Harnsberger HR et al. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): imaging manifestations in the head and neck. AJR Am J Roentgenol 2008; 191(6): W299-W306. Dostupné z DOI: http://dx.doi.org/10.2214/AJR.08.1114.
3. Mazánek J, Povýšil C. Rosai-Dorfmanova nemoc v oblasti hlavy a krku. Choroby hlavy a krku; 2006; 15(1) a 2005; 14(3–4): 39–43.
4. Lim KS. A case of cutaneous Rosai-Dorfman Disease presenting as breast nodules. Čs Derm 2006; 81(1): 44–45.
5. Jabali Y, Smrčka V, Přádná J. Rosai-Dorfman disease – successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine – a case report. Internat J Surgl Pathol 2005; 13(3): 285–289.
6. Kodetová D, Kodet R, Syrůček M et al. Sinusová histiocytóza s masivní lymfadenopatií – diseminovaná forma syndromu Rosai-Dorfman. Česk Patol 1996; 32(2): 53–59.
7. McClain KL, Natkunam Y, Swerdlow SH. Atypical cellular disorders. Hematology Am Soc Hematol Educ Program 2004; 283–296.
8. Picarsic J, Jaffe R. Nosology and Pathology of Langerhans Cell Histiocytosis. Hematol Oncol Clin North Am 2015; 29(5): 799–823.
9. Sarwal R, Tu E, Mendelblatt FI et al. Atypical ocular presentations of Rosai-Dorfman disease. Ocul Immunol Inflamm 2008; 16(1): 9–15.
10. Al-Daraji W, Anandan A, Klassen-Fischer M et al. Soft tissue Rosai-Dorfman disease: 29 new lesions in 18 patients, with detection of polyomavirus antigen in 3 abdominal cases. Ann Diagn Pathol 2010; 14(5): 309–316.
11. Kaur PP, Birbe RC, DeHoratius RJ. Rosai-Dorfman disease in a patient with systemic lupus erythematosus. J Rheumatol 2005; 32(5): 951–953.
12. Alqanatish JT, Houghton K, Bond M et al. Rituximab treatment in a child with Rosai-Dorfman disease and systemic lupus erythematosus. J Rheumatol 2010; 37(8): 1783–1784.
13. Salim A, Williamson M, Barker F et al. Steroid responsive cutaneous Rosai-Dorfman disease associated with uveitis and hypothyroidism. Clin Exp Dermatol 2002; 27(4): 277–279.
14. Grabczynska SA, Toh CT, Francis N et al. Rosai-Dorfman disease complicated by autoimmune haemolytic anaemia: case report and review of a multisystem disease with cutaneous infiltrates. Br J Dermatol 2001; 145(2): 323–326.
15. Venkataraman G, McClain KL, Pittaluga S et al. Development of disseminated histiocytic sarcoma in a patient with autoimmune lymphoproliferative syndrome and associated Rosai-Dorfman disease. Am J Surg Pathol 2010; 34(4): 589–594.
16. Drosos AA, Georgiadis AN, Metafratzi ZM et al. Sinus histocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with primary Sjogren’s syndrome. Scand J Rheumatol 2004; 33(2): 119–122.
17. Sachdev R, Shyama J. Co-existent Langerhans cell histiocytosis and Rosai-Dorfman disease: a diagnostic rarity. Cytopathology 2008; 19(1): 55–58.
18. Wang KH, Cheng CJ, Hu CH et al. Coexistence of localized Langerhans cell histiocytosis and cutaneous Rosai-Dorfman disease. Brit J Dermatol 2002; 147(4): 770–774.
19. O’Malley DP, Duong A, Barry TS et al. Co-occurrence of Langerhans cell histiocytosis and Rosai-Dorfman disease: possible relationship of two histiocytic disorders in rare cases. Mod Pathol 2010; 23(12): 1616–1623.
20. Llamas-Velasco M, Cannata J, Dominguez I al. Coexistence of Langerhans cell histiocytosis, Rosai-Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver. J Cutan Pathol 2012; 39(12): 1125–1130.
21. Pang CS, Grier DD, Beaty MW. Concomitant occurrence of sinus histiocytosis with massive lymphadenopathy and nodal marginal zone lymphoma. Arch Pathol Lab Med 2011; 135(3): 390–393.
22. Moore JC, Zhao X, Nelson EL. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and diffuse large B-cell lymphoma: a case report. J Med Case Rep 2008; 2: 70. Dostupné z DOI: <http://dx.doi.org/10.1186/1752–1947–2-70>.
23. Akria L, Sonkin V, Braester A et al. Rare coexistence of Rosai-Dorfman disease and nodal marginal zone lymphoma complicated by severe life-threatening autoimmune hemolytic anemia. Leuk Lymphoma 2013; 54(7): 1553–1556.
24. Di Tommaso L, Rahal D, Bossi P et al. Hepatic Rosai-Dorfman disease with coincidental lymphoma: report of a case. Int J Surg Pathol 2010; 18(6): 540–543.
25. Noguchi S Yatera K, Shimajiri S et al. Intrathoracic Rosai Dorfman disease with spontaneous remission: a clinical report and review of the literature. Tohoku J Exp Med 2012; 227(3): 231–325.
26. Foucar E, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymfadenopathy. An analysis of 14 death occuring in patient registry. Cancer 1984: 54(9): 1834–1840.
27. Noguchi S, Yatera K, Shimajiri S et al. Intrathoracic Rosai-Dorfman disease with spontaneous remission: a clinical report and a review of the literature. Tohoku J Exp Med 2012; 227(3): 231–325.
28. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Patho 1990; 7(1): 19–73.
29. Wang KH, Chen WY, Liu HN et al. Cutaneous Rosai-Dorfman disease: clinicopathological profiles, spectrum and evolution of 21 lesions in six patients. Br J Dermatol 2006; 154(2): 277–286.
30. Beros V, Houra K, Rotim K et al. Isolated cerebellar intraparenchymal Rosai-Dorfman disease – case report and review of literature. Br J Neurosurg 2011; 25(2): 292–296.
31. Catalucci A, Lanni G, Ventura L et al. A rare case intracranial Rosai Dorfman disease mimicking multiple meningiomas. Neuroradiol 2012; 25(5): 569–574.
32. Green I, Dorfman RF, Rosai J. Breast involvement by extranodal Rosai-Dorfman disease: report of seven cases. Am J Surg Pathol 1997; 21(6): 664–668.
33. Abdollahi A, Ardalan FA, Ayati M. Extranodal Rosai-Dorfman disease of the kidney. Ann Saudi Med 2009; 29(1): 55–57.
34. Chhabra S, Agarwal R, Garg S et al. Extra-nodal Rosai-Dorfman disease: a case report with thyroid involvement. Diagn Cytopathol 2012; 40(5): 447–449.
35. Del Gobbo A, Moltrasio F, Young RH et al. Involvement of the testis and related structures by Rosai-Dorfman disease: report of 2 new cases and review of the literature. Am J Surg Pathol 2013; 37(12): 1871–1875.
36. Zhu F, Zhang JT, Xing XW et al. Rosai-Dorfman disease: a retrospective analysis of 13 cases. Am J Med Sci 2013; 345(3): 200–210.
37. Demico EG, Rosenberg AE, Björnsson J et al. Primary Rosai-Dorfman disease of bone: a clinicopathologic study of 15 cases. Am J Surg Pathol 2010; 34(9): 1324–1333.
38. Shiran MS, Tan CG, Kinali MS et al. Multifocal nodal and extanodal Rosai Dorfman disease initially diagnosed as histiocytic lymphoma. Malays J Pathol 2008; 30(1): 63–65.
39. Albano D, Bosio G, Bertagna F. 18F-FDG PET/CT Follow-up of Rosai-Dorfman Disease. Clin Nucl Med 2015; 40(8): e420-e422. Dostupné z DOI: http://dx.doi.org/10.1097/RLU.0000000000000853.
40. Karunanithi S, Singh H, Sharma P et al. 18F-FDG PET/CT imaging features of Rosai Dorfman disease: a rare cause of massive generalized lymphadenopathy. Clin Nucl Med 2014; 39(3): 268–289.
41. Deshayes E, Le Berre JP, Jouanneau E et al. 18F-FDG PET/CT findings in a patient with isolated intracranial Rosai-Dorfman disease. Clin Nucl Med 2013; 38(1): e50-e52. Dostupné z DOI: http://dx.doi.org/10.1097/RLU.0b013e31825ae73b.
42. Tsang JS, Anthony MP, Wong MP et al. The use of FDG-PET/CT in extranodal Rosai-Dorfman disease of bone. Skeletal Radiol 2012; 41(6): 715–717.
43. Raslan OA, Schellingerhout D, Fuller GN et al. Rosai-Dorfman disease in neuroradiology: imaging findings in a series of 10 patients. AJR Am J Roentgenol 2011; 196(2): W187-W193.
44. Hock AT, Long MT, Sittampalam K et al. Rosai-Dorfman disease: FDG PET/CT findings in a patient presenting with pyrexia and cervical adenopathy. Clin Nucl Med 2010; 35(8): 576–578.
45. Maia RC, Meis E, Romano S et al. Rosai Dorfman disease: a report of 8 cases in a tertiary care center and review of the literature. Brazil J Med Biolog Research 2015; 48(1): 6–12.
46. Ocheni S, Ibegbulam OG, Okafor OC et al. Usefulness of oral corticosteroid in Rosai-Dorfman disease. Eur J Cancer Care (Engl) 2007; 16(3): 286–288.
47. Oka M, Kamo T, Goto N et al. Successful treatment of Rosai-Dorfman disease with low-dose oral corticosteroid. J Dermatol 2009; 36(4): 237–240.
48. Maklad AM, Bayoumi Y, Tunio M et al. Steroid-resistant extranodal rosai-dorfman disease of cheek mass and ptosis treated with radiation therapy. Case Rep Hematol 2013; 2013: 428297. Dostupné z DOI: http://dx.doi.org/10.1155/2013/428297.
49. Horneff G, Jurgens H, Hort W et al. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to methotrexate and mercaptopurine. Med Pediatr Oncol 1996; 27(3): 187–192.
50. Guchelaar H, Richel DS, Schaafsma MR. Clinical and toxicological aspect of the antineoplastic drug cladribine: a review. Ann Hematom 1994; 69(5): 223–230.
51. Carrera CJ, Terai C, Lotz M et al. Potent toxicity of 2-chlorodeoxyadenosine toward human monocytes in vitro and in vivo. A novel approach to immunosupressive therapy. J Clin. Incest 1990; 86(5): 1480–1488.
52. Konca C, Ozkurt ZN, Deger M et al. Extranodal multifocal Rosai-Dorfman disease: response to 2-chlorodeoxyadenosine treatment. Int J Hematol 2009; 89(1): 58–62.
53. Aouba A, Terrier B, Vasiliu V 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.
54. Tasso M, Esquembre C, Blanco E et al. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine. Pediatr Blood Cancer 2006; 47(5): 612–615.
55. Sasaki K, Pemmaraju N, Westin JR et al. A single case of Rosai-dorfman disease marked by pathologic fractures, kidney failure, and liver cirrhosis treated with single-agent cladribine. Front Oncol 2014; 4: 297. Dostupné z DOI: http://dx.doi.org/10.3389/fonc.2014.00297.
56. Simko SJ, Tran HD, Jones J et al. Clofarabine salvage therapy in refractory multifocal histiocytic disorders, including Langerhans cell histiocytosis, juvenile xanthogranuloma and Rosai-Dorfman disease. Pediatr Blood Cancer 2014; 61(3): 479–487.
57. Nadal M, Kervarrec T, Machet MC et a. Cutaneous Rosai-Dorfman disease located on the breast: Rapid effectiveness of methotrexate after failure of topical Corticosteroids, acitretin and thalidomide. Acta Derm Venereol 2015; 95(6): 758–759.
58. Wang F, Zhou H, Luo DQ et al. Dermatoscopic findings in cutaneous Rosai-Dorfman disease and response to low-dose thalidomide. J Dtsch Dermatol Ges 2014; 12(4): 350–352.
59. Li X, Hong Y, An Q et al. Successful treatment of Rosai-Dorfman disease with low-dose oral thalidomide. JAMA Dermatol 2013; 149(8): 992–993.
60. Du J, Ding X, Cai L et al. Rosai-Dorfman disease: three difficult-to-diagnose cases. Eur J Dermatol 2010; 20(5): 650–651.
61. Merola JF, Pulitzer M, Rosenman K et al. Cutaneous Rosai-Dorfman disease. Dermatol Online J 2008; 14(5): 8.
62. Lu CI, Kuo TT, Wong WR et al. Clinical and histopathologic spectrum of cutaneous Rosai-Dorfman disease in Taiwan. J Amer Acad Dermatol 2004; 51(6): 931–939.
63. Tjiu JW, Hsiao CH, Tsai TF. Cutaneous Rosai-Dorfman disease: remission with thalidomide treatment. Brit J Dermatol 2003; 148(5): 1060–1061.
64. Rubinstein M, Assal A, Scherba M et al. Lenalidomide in the treatment of Rosai Dorfman Disease – a first in use report. Am J Hematol 2016; 91(2): E1. Dostupné z DOI: http://dx.doi.org/10.1002/ajh.24225.
65. Alliot C, Capiod JC, Claisse JF et al. Sinus histiocytosis of the Rosai-Dorfman type following Hodgkin disease: efficacy of interferon-alpha. Presse Med 1996; 25(4): 171.
66. Le Guenno G, Galicier L, Fieschi C et al. Dramatic efficiency of pegylated interferon in sinus histiocytosis with massive lymphadenopathy. Brit J Dermatol 2011; 164(1): 213–215.
67. Löhr HF, Gödderz W, Wölfe T et al. Long-term survival in a patient with Rosai-Dorfman disease treated with interferon-alpha. Eur J Cancer 1995; 31A(13–14): 2427–2428.
68. Palomera L, Domingo JM, Olave T et al. Sinus histiocytosis with massive lymphadenopathy: complete response to low-dose interferon-alpha. J Clin Oncol 1997; 15(5): 2176.
69. Palomera L, M Domingo J, Soria J et al. Long term survival in a patient with aggressive Rosai-Dorfman disease treated with interferon alpha. Med Clin (Barc) 2001; 116(20): 797–798.
70. Pulsoni A, Anghel G, Falcucci P et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Amer J Hematom 2002; 69(1): 67–71.
71. Silvers AR, Som PM, Meyer RJ. Egg shell nodal calcification in a patient with sinus histiocytosis with massive lymphadenopathy treated with interferon. AJNR Amer J Neuroradiol 1996; 17(2): 361–363.
72. Xia JX, Jin XH, Mou Y et al. Combined treatment for cutaneous Rosai-Dorfman disease: a report of 2 cases. Int J Clin Exp Med 2013; 6(9): 822–827.
73. Alqanatish JT, Houghton K, Bond M et al. Rituximab treatment in a child with rosai-dorfman disease and systemic lupus erythematosus. J Rheumatol 2010; 37(8): 1783–1784.
74. Pagel JM, Lionberger J, Gopal AK et al. Therapeutic use of Rituximab for sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Am J Hematol 2007; 82(12): 1121–1122.
75. Petschner F, Walker UA, Schmitt-Gräff A et al. “Catastrophic systemic lupus erythematosus” with Rosai-Dorfman sinus histiocytosis. Successful treatment with anti-CD20/rituximab. Dtsch Med Wochenschr 2001; 126(37): 998–1001.
76. Utikal J, Ugurel S, Kurzen H et al. Imatinib as a treatment option for systemic non-Langerhans cell histiocytoses. Arch Dermatol 2007; 143(6): 736–740.
77. Gebhardt C, Averbeck M, Paasch U et al. A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy. Arch Dermatol 2009; 145(5): 571–574.
Labels
Diabetology Endocrinology Internal medicineArticle was published in
Internal Medicine
2016 Issue 6
Most read in this issue
- Sinus histiocytosis with massive lymphadenopathy: FDG-PET/CT documented partial remission after treatment with 2-chlorodeoxyadenosine
- Insulin application techniques in adult patients with diabetes
- Cardiomyopathy in MR image
- Acute causes of sudden deaths in patients with severe hypoglycemia