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Case Reports
. 2020 May 1;59(9):1203-1209.
doi: 10.2169/internalmedicine.4054-19. Epub 2020 Jan 17.

IgG4-related Disease with a Cardiac Mass

Ikuko Matsumura  1 , Takeki Mitsui  1 , Kenichi Tahara  1 , Hiroaki Shimizu  1 , Kunio Yanagisawa  2 , Takuma Ishizaki  1 , Hiromi Koiso  2 , Makiko Takizawa  1 , Akihiko Yokohama  3 , Takayuki Saitoh  4 , Junko Hirato  5 , Hirokazu Murakami  4 , Hiroshi Handa  1 , Norifumi Tsukamoto  6
Affiliations
Case Reports

IgG4-related Disease with a Cardiac Mass

Ikuko Matsumura et al. Intern Med. .

Abstract

A 69-year-old man with palpitations and decreased blood pressure was referred. Echocardiography showed a mass in the right atrium and cardiac septum. The serum IgG4 level was 1,450 mg/dL. A biopsy of the cardiac mass showed fibrosis with inflammatory cells and increased IgG4-positive plasma cells and lymphocytes. Flow cytometry and polymerase chain reaction of the immunoglobulin heavy chain did not demonstrate monoclonality. He was diagnosed with IgG4-related disease (IgG4-RD). IgG4-RD with a cardiac mass is rare and it is difficult to distinguish it from malignant lymphoma by a pathological examination alone. We therefore performed a biopsy and analyzed the clonality in order to make an accurate diagnosis of IgG4-RD.

Keywords: IgG4-related disease; cardiac mass; non-Hodgkin lymphoma.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
(A, B) Echocardiography and CT showing a right atrial tumor infiltrating the cardiac septum at the diagnosis (white arrow). (C) 18F-FDG-PET/CT showing the accumulation of 18F-FDG in the cardiac muscle, mediastinal lymph nodes, lacrimal glands, salivary glands, and subcutaneous nodules in the bilateral upper arms at the diagnosis (block arrow). (D) An electrocardiogram showing first-degree atrioventricular block.
Figure 2.
Figure 2.
Histological findings of the cardiac mass. (A) Flow cytometry shows that the kappa/lambda ratio is in the normal range. (B) Hematoxylin and Eosin (H&E) staining (×40). Various sizes of follicles are distributed irregularly in the tumor. Slightly atrophic germinal centers containing fibrosis and infiltration of small vessels are observed. (C) H&E staining (×200). Proliferation of immunoblast-like cells and heavy infiltration of mature plasma cells and small lymphocytes are observed in the interfollicular area. (D, E) Staining with CD3 (D) and CD20 (E) shows a mixture of small and medium-sized lymphocytes and immunoblasts. (F, G) Immunohistochemical studies of light chain, kappa (F) and lambda (G), determinants for interfollicular plasma cells, plasma cytoid cells, and B-immunoblasts demonstrate a polyclonal pattern (×100). (H) There are numerous IgG-positive cells in the lesion (×100). (I) Over 50% of the IgG-positive cells are IgG4-positive (×100).
Figure 3.
Figure 3.
PCR for IgH rearrangement in the cardiac biopsy specimen at the diagnosis. The lanes are: 1, patient; 2, clonal control; 3, polyclonal control; and 4, H2O, SM: size marker
Figure 4.
Figure 4.
Clinical course of this patient.
Figure 5.
Figure 5.
Echocardiography and CT showing shrinkage of the cardiac mass after one year.
See this image and copyright information in PMC

References

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