Extensive work-up for rheumatologic, infectious, and GI etiologies were non-revealing. There was a concern for carcinoid tumor, however 24-hour urine 5-HIAA, chromogranin, and serotonin levels were normal. Biopsies from small bowel and colonic mucosa showed no diagnostic abnormalities. A bone marrow biopsy to investigate the probable causes of pancytopenia showed no findings that were suggestive of underlying dysplasia or infiltrative process.
A transjugular liver biopsy was done for a suspicion of autoimmune hepatitis. The results were consistent with hemophagocytosis. Further testing which revealed hypertriglyceridemia helped complement the remaining criteria for diagnosis of Hemophagocytic Lymphohistiocytosis. She was started on Solumedrol.
A PET scan was done in search of a primary malignancy, and it demonstrated hypermetabolic activity in the left breast at the site of a complex fluid density in the retropectoral area. Biopsy of the seroma was consistent with breast implant-associated anaplastic large-cell lymphoma (BI-ALCL). IHC staining of the atypical cells was positive for CD30 and negative for ALK-1. She underwent capsulectomy and seroma drainage followed by adjuvant CHOEP.
A punch biopsy of one of her left arm scaly skin lesions was done and the pathology revealed paraneoplastic ichthyosis.
Discussion: Breast implant-associated anaplastic large-cell lymphoma is a rare CD30 positive, ALK-1 negative peripheral T cell lymphoma (PTCL) which usually arises around breast implants. The first case was described in 1997, by Keech and Creech. Since then, approximately 200 cases have been reported in literature reviews. In a population-based case-control study from the Netherland, it has been shown that there is an 18-fold increased risk of developing anaplastic large-cell lymphoma arising from breast implants as compared to women without implants. Despite the high odds ratio, the overall incidence is still low: reportedly 1 to 3 cases per 1 million breast implants per year.
The efficacy of different therapeutic approaches used in patients with BI-ALCL to identify an optimal treatment regimen has been evaluated. The conclusion of the study was that patients who underwent complete surgical excision (with or without adjuvant chemotherapy, depending on the extent of the disease) had better overall survival and event free survival than those who underwent limited surgery, chemotherapy, or radiation therapy alone.
Conclusions: BI-ALCL is a rare form of non-Hodgkins PTCL. It is important for internists to be aware of this rare entity to ensure prompt diagnosis and management. It is of great value to include a discussion of BI-ALCL during pre-operative counseling regarding the risks of breast implantation, so that patients are also aware of this entity in order be watchful for any breast tissue changes.