Intravascular large B-cell lymphoma associated with silicone breast implant, HLA-DRB1*11:01, and HLA-DQB1*03:01 manifesting as macrophage activation syndrome and with severe neurological symptoms: a case report

Abstract

BackgroundSilicone implants have been successfully used for breast augmentation and reconstruction in millions of women worldwide. The reaction to the silicone implant is highly variable; it can lead to local inflammatory symptoms, and sometimes to systemic symptoms and disease. Over 80 cases of anaplastic lymphoma kinase-negative anaplastic large cell lymphoma have been reported in patients with silicone breast implants and have been accepted as a new clinical entity. To the best of our knowledge, an intravascular large B-cell lymphoma associated with a silicone breast implant has not been reported previously.Case presentationA 48-year-old Caucasian woman who presented with high fever was found to have splenomegaly on physical examination. A laboratory diagnosis revealed pancytopenia, hypertriglyceridemia, and hyperferritinemia. She developed signs of altered sensorium, hemiparesis, aphasia, and cauda equina syndrome. On further evaluation, she fulfilled the necessary five out of eight criteria for diagnosis of macrophage activation syndrome/hemophagocytic lymphohistiocytosis. Dexamethasone administration was followed by prompt improvement; however, 3 days later she again manifested high fever, which persisted despite administration of immunoglobulin and cyclosporine A. Her silicone breast implant was considered a possible contributor to her macrophage activation syndrome and was therefore removed. A histological examination of the capsule tissue showed an extensive lymphohistiocytic/giant cell foreign body reaction suggestive of autoimmune/inflammatory syndrome induced by adjuvants. However, the histological examination unexpectedly also revealed an intravascular large B-cell lymphoma.ConclusionsThe genetic background of our patient with silicone breast implants might have predisposed her to three rare and difficult to diagnose syndromes/diseases: macrophage activation syndrome/hemophagocytic lymphohistiocytosis, autoimmune/inflammatory syndrome induced by adjuvants, and intravascular large B-cell lymphoma. The simultaneous manifestation of all three syndromes suggests causal interrelationships. Human leukocyte antigen testing in all women who undergo silicon breast implantation could in the future enable us to better evaluate the risk of potential side effects.Electronic supplementary materialThe online version of this article (doi:10.1186/s13256-016-0993-5) contains supplementary material, which is available to authorized users.

Chronic Pulmonary Silicone Embolism from Breast Augmentation Is Not a Common Finding in Explanted Lungs

“…Reported pulmonary complications from silicone breast implants include silicone thorax [ 15 , 16 ], pleural effusions [ 17 ], silicone-related complaints [ 18 – 20 ], siliconosis, autoimmune/inflammatory syndrome induced by adjuvants (ASIA) [ 21 – 23 ], and anaplastic large cell lymphoma [ 24 , 25 ]. The presumed pathogenesis behind these complications is the inflammatory response elicited by migration of silicone particles into tissues [ 26 ].…”

Splenic marginal zone B-cell lymphoma associated with ruptured breast implants: case report and review of the literature

“…While the argument can be made for loco-regional lymphomas occurring as a result of close-proximity immunologic stimuli, the data is less convincing as to whether disrupted implants are responsible for systemic lymphomas. The medical literature currently describes two patients with compromised silicone implants who presented with follicular lymphoma and intravascular large B-cell lymphoma outside of their breast tissue [ 3 , 4 ]. However, systemic lymphomas arising in women with breast implants could be purely coincidental.…”

“…The medical literature currently contains twelve cases of B-cell lymphoma occurring in patients with breast implants, including follicular lymphoma, primary cutaneous follicle center lymphoma, intravascular large-cell lymphoma, diffuse large B-cell lymphoma, marginal zone lymphoma, and plasmablastic lymphoma ( [1][2][3][4][5][6][7][8][9][10][11], summarized in Table 1). An association between B-cell lymphomas and implants has been previously dismissed given the heterogeneity of such lymphoma cases.…”

Intravascular Large B Cell Lymphoma of the Breast: A Rare Entity

“…The relationship between silicone implants, MAS/HLH and IVLBCL is yet to be understood and this association needs to be explored. 20 Though this report is seen in the setting of a breast implant, we included this in our report to highlight the quotient of surprise and rarity of this lesion in this location be it breast or the capsule.…”