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Rosai-Dorfman disease with a concurrent mantle cell lymphoma

2018; Elsevier BV; Volume: 5; Issue: 1 Linguagem: Inglês

10.1016/j.jdcr.2018.09.017

2352-5126

Alexandra Edelman, Brittany Patterson, Kristen Donovan, Janine C. Malone, Jeffrey P. Callen,

Viral-associated cancers and disorders

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare, benign histiocytic disorder characterized by generalized lymphadenopathy and constitutional symptoms.1Foucar E. Rosai J. Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity.Semin Diagn Pathol. 1990; 7: 19-73PubMed Google Scholar The classic presentation of RDD is painless cervical lymphadenopathy with associated fever, night sweats, and weight loss.2Moore J.C. Zhao X. Nelson E.L. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and diffuse large B-cell lymphoma: a case report.J Med Case Rep. 2008; 2: 70Crossref PubMed Scopus (25) Google Scholar Extranodal involvement is present in up to 40% of cases of RDD, with the skin being the most common site.1Foucar E. Rosai J. Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity.Semin Diagn Pathol. 1990; 7: 19-73PubMed Google Scholar Although considered benign, 10% of patients with RDD have coexisting immunologic abnormalities, such as postinfectious conditions and hematologic malignancies.1Foucar E. Rosai J. Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity.Semin Diagn Pathol. 1990; 7: 19-73PubMed Google Scholar, 2Moore J.C. Zhao X. Nelson E.L. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and diffuse large B-cell lymphoma: a case report.J Med Case Rep. 2008; 2: 70Crossref PubMed Scopus (25) Google Scholar There have been only 25 reported cases of RDD in association with Hodgkin or non-Hodgkin lymphomas.3Akria L. Sonkin V. Braester A. Cohen H.I. Suriu C. Polliack A. Rare coexistence of Rosai-Dorfman disease and nodal marginal zone lymphoma complicated by severe life-threatening autoimmune hemolytic anemia.Leuk Lymphoma. 2013; 54: 1553-1556Crossref PubMed Scopus (12) Google Scholar, 4Ferandez-Vega I. Santos-Juanes J. Ramsay A. Cutaneous Rosai-Dorfman disease following a classical hodgkin lymphoma, nodular sclerosis subtype.Am J Dermatopathol. 2014; 36: 280-281Crossref PubMed Scopus (7) Google Scholar, 5Garg K. Singh H. Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) and anaplastic large cell lymphoma.EJCRIM. 2017; 4: 70Google Scholar We present a case of RDD in association with mantle cell lymphoma with a primary presentation of extranodal skin involvement without constitutional symptoms. In August of 2014, a 54-year-old man presented with a several-month history of nontender, nonpruritic well-circumscribed erythematous plaques on his temples. The patient was otherwise feeling well without constitutional symptoms. A biopsy at that time found a benign lymphocytic infiltrate consistent with pseudolymphoma. Additional evaluation with blood work and imaging was advised but not completed by the patient as he stated the lesions self-resolved. In June 2017, the patient returned to the clinic with similar lesions on the temples with additional widespread, asymptomatic erythematous papules on the trunk and lower extremities (Fig 1, A). The patient was otherwise feeling well and denied any constitutional symptoms. Three skin biopsy specimens were taken from the left central temple, the left lower back, and right medial distal pretibial region (Fig 2). Histopathologic examination found intact lymphocytes and plasma cells within the cytoplasm of histiocytes (emperipolesis). Immunohistochemically, the histiocytes were positive for CD68 and CD163 with coexpression of S100. Based on the clinical and histopathologic findings, a diagnosis of Rosai-Dorfman was made. Laboratory evaluation found a normocytic anemia (hemoglobin, 12.4 g/dL), eosinophilia (11%), and peripheral blood flow cytometry showing excess polyclonal IgG and IgA. A total-body competed tomography scan found extensive lymphadenopathy within the chest, abdomen, retroperitoneum, and pelvis; moderately severe splenomegaly; a mural mass in the sigmoid colon; and mild pulmonary nodularity in the left lower lobe. Bone marrow and lymph node biopsies were performed and were consistent with mantle cell non-Hodgkin lymphoma stage IVa. The patient was enrolled in a clinical trial at MD Anderson Hospital for further treatment, which involved ibrutinib/rituximab and hyperCVAD for consolidation (fractionated cyclophosphamide, vincristine, Adriamycin, and dexamethasone). Two cycles of therapy resulted in a marked decrease in his number of skin lesions (Fig 1, B) and resolution of his widespread lymphadenopathy. The patient has remained free of constitutional symptoms. RDD or SHML was first described in 1969, since then more than 400 cases have been reported in the RDD registry.1Foucar E. Rosai J. Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity.Semin Diagn Pathol. 1990; 7: 19-73PubMed Google Scholar, 6Rosai J. Dorfman R.F. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinico- pathologicalentity.Arch Pathol. 1969; 87: 63-70PubMed Google Scholar This disease is usually seen in children and young adults with a predilection for white males and those of African descent.6Rosai J. Dorfman R.F. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinico- pathologicalentity.Arch Pathol. 1969; 87: 63-70PubMed Google Scholar RDD is a rare entity; even rarer are reports of concomitant lymphoma. This report is the first, to our knowledge, to demonstrate RDD with concomitant mantle cell lymphoma. After reviewing the relevant literature, we found 25 cases of RDD in association with Hodgkin and non-Hodgkin lymphoma; of these, most of these cases (70%) had simultaneous diagnosis of RDD and lymphoma (Table I).3Akria L. Sonkin V. Braester A. Cohen H.I. Suriu C. Polliack A. Rare coexistence of Rosai-Dorfman disease and nodal marginal zone lymphoma complicated by severe life-threatening autoimmune hemolytic anemia.Leuk Lymphoma. 2013; 54: 1553-1556Crossref PubMed Scopus (12) Google Scholar, 4Ferandez-Vega I. Santos-Juanes J. Ramsay A. Cutaneous Rosai-Dorfman disease following a classical hodgkin lymphoma, nodular sclerosis subtype.Am J Dermatopathol. 2014; 36: 280-281Crossref PubMed Scopus (7) Google Scholar, 5Garg K. Singh H. Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) and anaplastic large cell lymphoma.EJCRIM. 2017; 4: 70Google Scholar The pathogenesis of RDD is unclear. Suggested possibilities include a macrophage colony stimulating factor resulting in immune-suppressive abnormal histiocytes (an immune-related phenomenon), an exaggerated infectious response to an agent (both viral and bacterial), and/or a genetic predisposition.2Moore J.C. Zhao X. Nelson E.L. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and diffuse large B-cell lymphoma: a case report.J Med Case Rep. 2008; 2: 70Crossref PubMed Scopus (25) Google Scholar, 21Pang C.S. Grier D.D. Beaty M.W. Concomitant occurrence of sinus histiocytosis with massive lymphadenopathy and nodal marginal zone lymphoma.Arch Pathol Lab Med. 2011; 135: 390-393PubMed Google Scholar Our patient's concurrent diagnosis of mantle cell lymphoma after his diagnosis of RDD, and the observation that consolidation therapy of his lymphoma resulted in improvement of his RDD, lends support for the possible immune-mediated etiology of RDD.Table ICases of RDD and malignant lymphomaNote. Table was created/adapted by Akria et al3Akria L. Sonkin V. Braester A. Cohen H.I. Suriu C. Polliack A. Rare coexistence of Rosai-Dorfman disease and nodal marginal zone lymphoma complicated by severe life-threatening autoimmune hemolytic anemia.Leuk Lymphoma. 2013; 54: 1553-1556Crossref PubMed Scopus (12) Google Scholar with additional cases added since their publication in 2013.Case no.ReferenceAge/sexLymphoma typeTime interval1Foucar et al,7Foucar E. Rosai J. Dorfman R.F. Sinus histiocytosis with massive lymphadenopathy. An analysis of 14 deaths occurring in a patient registry.Cancer. 1984; 53: 1834-1840Crossref Scopus (170) Google Scholar 19836/MLarge cell immunoblasticNHL 8 mo after RDD2Rangwala et al,8Rangwala A.F. Zinterhofer L.J. Nyi K.M. Ferreira P.P. Sinus histiocytosis with massive lymphadenopathy. An unreported association.Cancer. 1990; 65: 999-1002Crossref PubMed Scopus (33) Google Scholar 199062/MSmall noncleavedNHL 4 y after RDD3Falk et al,9Falk S. Stutte H.J. Frizzera G. Hodgkin's disease and sinus histiocytosis with massive lymphadenopathy-like changes.Histopathology. 1991; 19: 221-224Crossref PubMed Scopus (31) Google Scholar 199149/MHD, MCConcurrent424/MHD, NOSConcurrent5Maia et al,10Maia D.M. Dorfman R.F. Focal changes of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) associated with nodular lymphocyte predominant Hodgkin's disease.Hum Pathol. 1995; 26: 1378-1382Crossref PubMed Scopus (37) Google Scholar 199539/MHD, LPConcurrent611/MHD, LPConcurrent7Koduru et al,11Koduru P.R. Susin M. Kolitz J.E. et al.Morphological, ultrastructural, and genetic characterization of an unusual T-cell lymphoma in a patient with sinus histiocytosis with massive lymphadenopathy.Am J Hematol. 1995; 48: 192-200Crossref PubMed Scopus (22) Google Scholar 199552/MT cellNHL 8 y after RDD8Alliot et al,12Alliot C. Capiod J.C. Claisse J.F. Goutier M.F. Desablens B. Sinus histiocytosis of the Rosai-Dorfman type following Hodgkin disease:efficiency of interferon-alpha.Press Med. 1997; 25: 171Google Scholar 1996UnknownHD, NOSHD before RDD9Krzemieniecki et al,13Krzemieniecki K. Pawlicki M. Marganska K. Parczewska J. The Rosai-Dorfman syndrome in a 17-year old woman with transformation into high-grade lymphoma. A rare disease presentation.Ann Oncol. 1996; 7: 977Abstract Full Text PDF PubMed Scopus (18) Google Scholar 199617/FHigh grade, NOSNHL 5 y after RDD10Lossos et al,14Lossos I.S. Ikon E. Bogomolski-Yahalom V. Ron N. Pollack A. Sinus histiocytosis with massive lymphadenopathy: report of a patient with isolated renotesticular involvement after cure of non-Hodgkin's lymphoma.Ann Hematol. 1997; 74: 41-44Crossref PubMed Scopus (32) Google Scholar 199767/MSmall lymphocyticNHL 12 y before RDD11Lu et al,15Lu D. Estampilla O.C. Manning Jr., J.T. Medeiros L.J. 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: 414-419Crossref PubMed Scopus (87) Google Scholar 200062/FFL grade IIConcurrent1230/FHD, LPConcurrent1328/MHD, LPConcurrent1463/FFL grade IConcurrent15Menzel et al,16Menzel C. Hamscho N. Dobert N. et al.PET imaging of Rosai-Dorfman disease: correlation with histopathology and ex-vivo beta-imaging.Arch Dermatol Res. 2003; 295: 280-283Crossref PubMed Scopus (26) Google Scholar 2003?/FNHL, NOSNHL 6 y before RDD16Garel et al,17Garel L. Lucaya J. Piqueras J. Clinical quiz. Large cell anaplastic lymphoma coexisting with Rosai-Dorfman disease.Pediatr Radiol. 2004; 34: 509-510PubMed Google Scholar 20048/FAnaplastic large cellConcurrent17Shoda et al,18Shoda H. Oka T. Inoue M. et al.Sinus histiocytosis with massive lymphadenopathy associated with malignant lymphoma.Intern Med. 2004; 43: 741-745Crossref PubMed Scopus (14) Google Scholar 200464/MDiffuse large B cellConcurrent18Moore et al,2Moore J.C. Zhao X. Nelson E.L. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and diffuse large B-cell lymphoma: a case report.J Med Case Rep. 2008; 2: 70Crossref PubMed Scopus (25) Google Scholar 200833/FDiffuse large B cellConcurrent19Luca Di Tommaso et al,19Di Tommaso L. Rahal D. Bossi P. Hepatic Rosai-Dorfman disease with coincidental lymphoma: report of a case.Int J Surg Pathol. 2010; 18: 540Crossref PubMed Scopus (9) Google Scholar 201065/FRelapsed FLConcurrent20Cvetkovic et al,20Cvetkovic Z, Celeketic D, Cvetkovic B. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) evolving into Hodgkin lymphoma: a case report. Presented at 33rd World Congress of the International Society of Hematology. 10-13 Oct 2010, Jerusalem, Israel.Google Scholar 201039/FHD, NSHD 2 y after RDD21Pang et al,21Pang C.S. Grier D.D. Beaty M.W. Concomitant occurrence of sinus histiocytosis with massive lymphadenopathy and nodal marginal zone lymphoma.Arch Pathol Lab Med. 2011; 135: 390-393PubMed Google Scholar 201180/FNodal MZLConcurrent22Wu et al,22Wu W. Cao K. Li Y. Primary splenic diffuse large B-cell lymphoma in a patient with Rosai-Dorfman disease.Am J Med Sci. 2012; 344: 155-159Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar 201242/MDiffuse large B cellConcurrent23Akria et al,3Akria L. Sonkin V. Braester A. Cohen H.I. Suriu C. Polliack A. Rare coexistence of Rosai-Dorfman disease and nodal marginal zone lymphoma complicated by severe life-threatening autoimmune hemolytic anemia.Leuk Lymphoma. 2013; 54: 1553-1556Crossref PubMed Scopus (12) Google Scholar 201350/MNodal MZLConcurrent24Fernandez-Vega et al,4Ferandez-Vega I. Santos-Juanes J. Ramsay A. Cutaneous Rosai-Dorfman disease following a classical hodgkin lymphoma, nodular sclerosis subtype.Am J Dermatopathol. 2014; 36: 280-281Crossref PubMed Scopus (7) Google Scholar 201451/FHD, NSConcurrent25Garg et al,5Garg K. Singh H. Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) and anaplastic large cell lymphoma.EJCRIM. 2017; 4: 70Google Scholar 201716/MAnaplastic large cellConcurrent26Present case54/MNHL, mantle cellConcurrentFL, Follicular lymphoma; HD, Hodgkin disease; LP, lymphocyte predominant; MC, mixed cellularity; MZL, marginal zone lymphoma; NHL, non-Hodgkin lymphoma; NOS, not otherwise specified; NS, nodular sclerosis. Open table in a new tab FL, Follicular lymphoma; HD, Hodgkin disease; LP, lymphocyte predominant; MC, mixed cellularity; MZL, marginal zone lymphoma; NHL, non-Hodgkin lymphoma; NOS, not otherwise specified; NS, nodular sclerosis.