Background Non-Hodgkin lymphomas (NHL) are 3 to 45 times more common in rheumatic diseases (RD) than in the general population. The spectrum of NHL varies in different studies.
Objectives To describe the most common types of NHL diagnosed in rheumatic patients from 2008 to 2010 in the Institute of Rheumatology of RAMS, Moscow.
Methods 185 patients (female-148, male-27) aged 19 to 80 years old (median-52 years old) with various RD and predictors of NHL development (massive enlargement of lacrimal and salivary glands, cytopenia, lymphadenopathy, splenomegaly, monoclonalsecretionIg) were conducted an oncohematological survey, including incisional biopsies with subsequent immunomorphological study, trephine biopsies and myelograms, T-and B-cell clonality determination by PCR in peripheral blood and tissue samples.
Results One hundred and ten (65.5%) patients (primary Sjögren’s syndrome (pSS) - 58, rheumatoid arthritis (RA)–25, systemic scleroderma (SSD)-4 and chronic hepatobiliary diseases (CHD)-6) were diagnosed with NHL. The diagnosis of RD was withdrawn with subsequent identification of primary NHL in 8 cases, myeloproliferative diseases in 5 cases and primary AL-amyloidosis in 4 patients. B-cell NHL were found in 97 (88%) and T-cell in 13 (12%) patients respectively. Immunoglobulin-secreting variant of lymphoma was observed in 30 (29.7%) patients with NHL. In RA B-cell (48%) and T-cell (52%) lymphomas were represented in equal proportions. Marginal zone lymphoma (MZL) was most frequently detected in pSS, SSD and CHD, whereas T-cell leukemia of large granular lymphocytes was more often diagnosed in RA (n=9). Plasma cell dyscrasia (PD) ranked second more frequent in pSS (n=5) and RA (n=5). T-cell splenic lymphoma (RA - 4), chronic lymphocytic leukemia (pSS - 1, RA - 2) and diffuse large B-cell lymphoma (RA-1, pSS-2) were diagnosed less frequently. Seventeen (76.5%) of the 22 examined patients with RA, SSD and CHD had associated Sjögren’s syndrome. Three patients simultaneously developed two types of NHL.
Conclusions A prospective study has shown that low-grade MZL, PD, T-cell leukemia of large granular lymphocytes and γδ-T-cell splenic lymphoma are the most common in RD. Primary NHL, PD, AL-amyloidosis and myeloproliferative disorders often have clinical features similar to rheumatic diseases.
Disclosure of Interest None Declared
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