Rare coexistence of Rosai–Dorfman disease and nodal marginal zone lymphoma complicated by severe life-threatening autoimmune hemolytic anemia

Abstract Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a rare self-limiting disorder of histiocytes with unknown etiology. Sinus histiocytosis with massive lymphadenopathy is most common in children and young adults and is characterized by painless lymphadenopathy. Histologically there is a proliferation of sinus histiocytes with lymphophagocytosis or emperipolesis. On rare occasions, SHML has been associated with lymphoma, usually involving different anatomic sites and developing at different times. We report a case of concomitant SHML and nodal marginal zone lymphoma involving the same lymph node without involvement of other nodal or extranodal sites. The presence of concomitant SHML within the lymph node involved by nodal marginal zone lymphoma may represent the responsiveness of SHML histiocytes to B-cell–derived cytokines in lymphoproliferative disorders. To our knowledge, this is the first description of concomitant occurrence of SHML and nodal marginal zone lymphoma.

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No evidence of splenic disease in patients with splenic marginal zone lymphoma undergoing splenectomy for autoimmune hemolytic anemia after monotherapy with rituximab

Leukemia & Lymphoma ◽

10.3109/10428194.2016.1157877 ◽

2016 ◽

Vol 57 (11) ◽

pp. 2705-2708 ◽

Cited By ~ 5

Author(s):

Christina Kalpadakis ◽

Gerassimos A. Pangalis ◽

Sotirios Sachanas ◽

Demetra Rontogianni ◽

Penelope Korkolopoulou ◽

...

Keyword(s):

Hemolytic Anemia ◽

Autoimmune Hemolytic Anemia ◽

Marginal Zone ◽

Marginal Zone Lymphoma ◽

Splenic Marginal Zone Lymphoma ◽

Splenic Disease

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A 71-Year-Old Man Presenting with Anemia and a Solitary Skin Lesion Associated with Primary Cutaneous Marginal Zone Lymphoma

Journal of Internal Medicine: Science & Art ◽

10.36013/jimsa.v2i1.53 ◽

2021 ◽

Vol 2 ◽

pp. 25-28

Author(s):

Mariya Khudyakova

Keyword(s):

Bone Marrow ◽

Hemolytic Anemia ◽

Health Care Providers ◽

Autoimmune Hemolytic Anemia ◽

Marginal Zone ◽

Marginal Zone Lymphoma ◽

Low Grade ◽

Care Providers ◽

Bone Marrow Biopsies ◽

Positive Direct

A 71-year-old man with a solitary red lesion on his left arm, which initially thought to be a hematoma, was admitted for inpatient evaluation of anemia, which after a positive direct Coombs test was qualified as autoimmune hemolytic anemia. The patient also presented with hoarseness and biopsies from the swelled nasopharynx and an enlarged right tonsil were obtained. Due to uncertain diagnosis and the patient’s previous history of malignancy, (low grade lymphoproliferative lymphoma) skin and bone marrow biopsies were performed. Unfortunately, the patient’s status rapidly deteriorated as a result of septic shock. The obtained skin, nasopharynx, and tonsil biopsies became available after the patient died and showed marginal zone lymphoma with no evidence of lymphoproliferative neoplasm in the bone marrow. This case demonstrates the diagnostic complexity of nodal marginal zone lymphoma complicated by severe life-threatening autoimmune hemolytic anemia in an elderly patient with multimorbidity, which presents management challenges for health care providers.

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Splenic marginal zone lymphoma with autoimmune hemolytic anemia

Journal of Medicine and Therapeutics ◽

10.15761/jmt.1000123 ◽

2018 ◽

Vol 2 (1) ◽

Author(s):

Jay Suriar ◽

Mra Aye ◽

Mohamed M Musadiq ◽

Jason S Cabot

Keyword(s):

Hemolytic Anemia ◽

Autoimmune Hemolytic Anemia ◽

Marginal Zone ◽

Marginal Zone Lymphoma ◽

Splenic Marginal Zone Lymphoma

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Simultaneous Occurrence of Rosai–Dorfman Disease and Nodal Marginal Zone Lymphoma in a Patient with Sjögren’s Syndrome

Case Reports in Hematology ◽

10.1155/2018/7930823 ◽

2018 ◽

Vol 2018 ◽

pp. 1-7

Author(s):

Vadim R. Gorodetskiy ◽

Wolfram Klapper ◽

Natalya A. Probatova ◽

Vladimir I. Vasilyev ◽

Elena V. Rozhnova

Keyword(s):

B Cell ◽

Marginal Zone ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Marginal Zone Lymphoma ◽

Rosai Dorfman Disease ◽

Nodal Marginal Zone Lymphoma ◽

Sjögren‘S Syndrome

We present an exceptionally rare case of co-occurrence of Rosai–Dorfman disease (RDD) and nodal marginal zone lymphoma (NMZL) in a 60-year-old Caucasian female with a 20-year course of Sjögren’s syndrome (SS). In response to treatment for lymphoma, the patient presented a short positive response, followed by a rapid progression of the disease accompanied by the development of the peripheral facial nerve palsy. We failed to detect Epstein–Barr virus (EBV) in the NMZL/RDD sample by EBV-encoded RNA (EBER) in situ hybridization but identified genomic DNA of EBV by polymerase chain reaction. A second biopsy revealed EBV-positive diffuse large B-cell lymphoma (DLBCL), not otherwise specified. The identical clonal immunoglobulin heavy chain gene rearrangements in the NMZL and DLBCL pointed to their clonal relationship. Though the role of EBV in the pathogenesis of some lymphomas is well-known, there have been only few cases of EBV-induced transformation of low-grade B-cell lymphoma into high-grade lymphoma and no cases of a patient with an NMZL background. To our knowledge, this is the first report of a concomitant occurrence of RDD and NMZL in a SS patient.

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