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SAT0452 Prevalence of B-cell Clonality and Lymphomas in Patients with IGG4-Related Disease: Single-Center Experience
  1. E. Sokol1,
  2. V. Vasilyev1,
  3. S. Smirnova2,
  4. T.N. Safonova3,
  5. S. Palshina4,
  6. E. Rodionova4,
  7. N. Kokosadze5,
  8. A. Kovrigina2
  1. 1Nasonova Research Institute of Rheumatology, Moscow, Russia
  2. 2Hematology Research Center
  3. 3Research Institute of Eye Diseases
  4. 4Nasonova Research Institute of Rheumatology
  5. 5N.N. Blokhin Russian Cancer Research Center, Moscow, Russian Federation


Background IgG4-related disease (IgG4-RD) is a distinct entity, characterized by tumefactive fibro-inflammatory lesions rich in polyclonal IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis and often elevated serum IgG4 concentrations. There is evolving data that IgG4-RD is associated with lymphomas.

Objectives To evaluate the prevalence of B-cell clonalityand malignant lymphoproliferationin patients with IgG4-RD.

Methods Single – center retrospective study. We reviewed medical records of patients treated in Nasonova Research Institute of rheumatology from 2010 to 2014. 15 patients with biopsy proven IgG4-RD were included (10 pts with definite IgG4-RD, 5pts with possible/probable IgG4 according to comprehensive diagnostic criteria of IgG4-RD (Umehara H et al., 2011). B-cell clonality was detected in PCR analysis of immunoglobulin (Ig) V-D-J genes heavy chain rearrangements (FR1, FR2, FR3) (on frozen tissue section or paraffin embedded).

Results 15 patients were included. The mean age was 39.5 years (range 19-67), mean duration of the disease before the diagnosis of IgG4-RD 55.9 months (range 5-204). The mean number of organs involved was 2.6 (range 1-4): orbit 10, salivary glands 9, lymph nodes 7, biliary tract 1, breast 1, bones 1, soft tissues of the neck 1 and nasal cavity or paranasal sinuses 8. Thirteen patients (86.7%) had elevated serum IgG4 levels >135 mg/dl at baseline (mean 919.4 mg/dl, range 81-3990 mg/dl). In 8 patients serum protein electrophoresis with immunofixation was performed and in 2 cases (25%) paraproteinemia was detected (tracer IgGκ - 1, monoclonal IgMκ 3.2 g/l - 1). These 2 patients demonstrated B-cell clonality in the tissue specimens as well (in bone marrow and submandibular salivary gland respectively). However, pathological and immunohystochemical examination didn't prove the existence of lymphoma. Altogether B-cell clonality was detected in 3 patients (20%): by PCR analysis of Ig heavy chain rearrangements in 2 cases (in submandibular salivary gland-1 and in bone marrow – 1), by PCR analysis of Ig kappa chain rearrangements (Vκ-Jk and Vκ-KDE/Intron RSS-KDE) in 1 case. The latter patient didn't have monoclonal secretion in the serum, however she exhibited pathological characteristics of MALT-lymphoma of the lacrimal gland with κ chain restriction. The diagnosis of IgG4-RD and MALT lymphoma in this patient was made simultaneously. In 1 patient clonality was uncertain (clonal variant exceeded polyclonal background three times).

Conclusions It seems that IgG4-RD can act as a background for lymphoma formation. In our research 3 pts (20%) had B-cell clonality in the affected tissues and 1 patient (6.7%) developed IgG4-related MALT-lymphoma of lacrimal gland. B-cell clonality does not necessarily constitute lymphoma, but these patients require very close follow-up. Serum electrophoresis with immunofixation and PCR analysis of B-cell IgH/L chain rearrangements is strongly recommended in patients with IgG4-RD.

Disclosure of Interest None declared

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