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Multiple Systemic Lesions in a Middle-aged Woman With a History of Lymphoma

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her mid-60s, who was in remission following successful combination chemotherapy for Burkitt lymphoma 8 years previously, presented with dyspnea, fatigue, general malaise, and presyncope. On examination, she was normotensive, afebrile, and clinically euvolemic. Laboratory testing showed a hemoglobin level of 12.1 g/dL (to convert to grams per liter, multiply by 10), white blood cell count of 9900/µL (to convert to ×109 per liter, multiply by 0.001), erythrocyte sedimentation rate of 76 mm/h, lactate dehydrogenase level of 139 U/L (to convert to microkatals per liter, multiply by 0.0167), and normal coagulation profile. Serum immunoglobulin quantitation showed normal immunoglobulin (Ig) G, IgA, and IgM levels of 1590 mg/L, 303 mg/L, and 94 mg/L, respectively (to convert to grams per liter, multiply by 0.01). Blood culture findings were negative.

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Biatrial intracardiac mass and multiple systemic lesions secondary to IgG4-related disease

D. Perform an endomyocardial biopsy

The patient underwent transcatheter endomyocardial biopsy, endobronchial ultrasonography-guided biopsy, and excisional biopsy of her nasal septal mass. The histology of the sampled tissues showed chronic inflammatory infiltrate with no evidence for malignancy. Immunohistochemistry analyses confirmed a large number of IgG4+ plasma cells. Based on the histology results, our final diagnosis was IgG4-related disease (IgG4-RD). Her cardiac mass was considered inoperable given its complex anatomy and multichamber involvement. She initially received high-dose steroid therapy with prednisone over subsequent months with minimal radiological improvement. Rituximab was added as a second agent, which she received at 6-month intervals for a total duration of 24 months. This resulted in significant reduction in the size of the cardiac mass and right Meckel cave lesions, as demonstrated on her repeat cardiac and brain magnetic resonance imaging (Figure 2), suggestive of IgG4-RD.

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Article Information

Corresponding Author: Harry Rakowski, MD, Division of Cardiology, Toronto General Hospital, 585 University Ave, Toronto, ON M5G 2N2, Canada (dr.harry.rakowski@uhn.ca).

Published Online: September 22, 2021. doi:10.1001/jamacardio.2021.3566

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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2.
Kamisawa  T , Zen  Y , Pillai  S , Stone  JH .  IgG4-related disease.   Lancet. 2015;385(9976):1460-1471. doi:10.1016/S0140-6736(14)60720-0PubMedGoogle ScholarCrossref
3.
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Kamisawa  T , Funata  N , Hayashi  Y ,  et al.  A new clinicopathological entity of IgG4-related autoimmune disease.   J Gastroenterol. 2003;38(10):982-984. doi:10.1007/s00535-003-1175-yPubMedGoogle ScholarCrossref
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Deshpande  V , Zen  Y , Chan  JKC ,  et al.  Consensus statement on the pathology of IgG4-related disease.   Mod Pathol. 2012;25(9):1181-1192. doi:10.1038/modpathol.2012.72PubMedGoogle ScholarCrossref
6.
Mavrogeni  S , Markousis-Mavrogenis  G , Kolovou  G .  IgG4-related cardiovascular disease.   Eur J Radiol. 2017;86:169-175. doi:10.1016/j.ejrad.2016.11.012PubMedGoogle ScholarCrossref
7.
Khosroshahi  A , Carruthers  MN , Deshpande  V , Unizony  S , Bloch  DB , Stone  JH .  Rituximab for the treatment of IgG4-related disease.   Medicine (Baltimore). 2012;91(1):57-66. doi:10.1097/MD.0b013e3182431ef6PubMedGoogle ScholarCrossref
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