Sjögren syndrome complicated by acute lymphoblastic leukemia

Fariba Binesh, Mohammad Bagher Owlia, Mohammad Reza Vahidfar, Pegah Kamal, Sara Mirhosseini


Malignant lymphoma is a well-recognized occurrence in Sjögren syndrome; however, progression to acute lymphoblastic leukemia is extremely uncommon. Here we present a patient with Sjögren syndrome complicated with acute lymphoblastic leukemia. A 41-year-old male, who was diagnosed as primary Sjögren syndrome 3 months ago was admitted to our hospital due to hematuria and weakness. The complete blood count showed bicytopenia. Bone marrow biopsy and aspiration cytology confirmed the diagnosis of acute lymphoblastic leukemia. He received combination chemotherapy with prednisolone, doxorubicin, cyclophosphamide, vincristine and intra-thecal methotrexate. Sjögren syndrome and other autoimmune diseases may build a portion of a continuum of lymphoproliferative disorders which have joined with B-cell neoplasms.


Internal Medicine; Cancer; Pathology

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Patel R and Shahane A. The epidemiology of Sjögren’s syndrome. Clin Epidemiol 6:247-255, 2014.

Chiu YH, Chung CH, Lin KT, Lin CS, Chen JH, Chen HC, et al. Predictable biomarkers of developing lymphoma in patients with Sjögren syndrome: a nationwide population-based cohort study. Oncotarget 8:50098-50108, 2017.

Molad Y, Okon E, Stark P, Prokocimer M. Sjö- gren’s syndrome associated T cell large granular lymphocyte leukaemia: a possible common etiopatho-genesis. J Rheumatol 28:2551-2552, 2001.

Brito-Zerón P, Kostov B, Fraile G, Caravia-Durán D, Maure B, Rascón FJ, et al. Characterization and risk estimate of cancer in patients with primary Sjögren syndrome. J Hematol Oncol 10:90, 2017.

Bunim JJ, Talal N. Development of malignant lymphoma in the course of Sjögren’s syndrome. Trans Assoc Am Physicians 76:45-56, 1963.

Valesini G, Priori R, Bavoillot D et al. Differential risk of non-Hodgkin’s lymphoma in Italian patients with primary Sjogren’s syndrome. J Rheumatol 24:2376-2380, 1997.

Alunno A, Maria Comasia Leone, Roberto Giacomelli R, Gerli R, Carubbi F. Lymphoma and lymphomagenesis in primary Sjögren’s syndrome. Front Med 5:102, 2018.

Nocturne G, Mariette X. Sjögren syndrome-associated lymphomas: an update on pathogenesis and management. Br J Haematol 168:317-327, 2015.

Talbot JH. Acute dermatomyositis-polymyositis and malignancy. Semin Arthritis Rheum 6:305-360, 1997.

Bernatsky S, Boivin JF, Jospeh L et al. An international cohort study of cancer in systemic lupus erythematosus. Arthritis Rheum 52:1481-1490, 2005.

M. N. Lazarus, D. Robinson1, V. Mak1, H. Mّller1 and D. A. Isenberg, Incidence of cancer in a cohort of patients with primary Sjögren’s syndrome, Rheumatology 45:1012-1015, 2006.

Brito-Zerón P, Kostov B, Fraile G, et al. Characterization and risk estimate of cancer in patients with primary Sjögren syndrome. J Hematol Oncol 10:90, 2017.

Blanes A, Villar A, Amigo V, Monzo E and Gonzalez Sal M. Primary Sjögren’s syndrome in men: evolution to acute lymphoblastic leukemia. Ann Med Int 8:605-608, 1991.

I. S. Lossos and D. Morgensztern. Prognostic biomarkers in diffuse large B-cell lymphoma. J Clin Oncol 24:995-1007, 2006.

Kamel OW, van de Rijn M, Hanasono MM, Warnke RA. Immunosuppression-associated lympho-proliferative disorders in rheumatic patients. Leuk Lymphoma 16:363-368, 1995.

Bartoloni E, Alunno A and Gerli R. Primary Sjögren’s syndrome as paraneoplastic disorder: a case report. Clin Exp Rheumatol 30:454. 2012.


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