PLAYING DRESS UP! WHEN TO SUSPECT LYMPHOMA IN A DRESS-LIKE PRESENTATION.

Case Presentation: Drug induced hypersensitivity syndrome (DIHS/DRESS) is a severe reaction to an identifiable drug, manifesting as a rash, eosinophilia, lymphadenopathy, fever and frequently internal organ involvement. Though recovery is often achieved with early identification and withdrawal of the offending agent, mortality can reach 5-10% due to resulting fulminant hepatic failure, multiorgan failure, myocarditis or hemophagocytosis. Similar clinical presentations have also been seen in viral infections, malignancies and autoimmune diseases, making definitive diagnosis challenging.An 88 year old woman with HFpEF and aortic stenosis, presented 1 month post-TAVR with atrial fibrillation with RVR, decompensated heart failure and pneumonia. She was diuresed, rate-controlled and treated with ceftriaxone and azithromycin. However, her leukocytosis persisted. CT chest revealed new diffuse adenopathy compared to previous imaging, suspicious for lymphoma. While awaiting lymph node biopsy results, she developed eosinophilia and a diffuse rash involving the face, chest, arms, and legs. Her biopsy returned with atypical cells but inconsistent with lymphoma. Notably, she had prior episodes of rash development related to daily furosemide usage. The Allergy service was consulted given concern for DRESS, suspecting the beta-lactam or furosemide as the trigger. While trialing other diuretics prior to prednisone initiation, a second lymph node biopsy returned with a diagnosis of Angioimmunoblastic T-cell lymphoma (AITL). IV methylprednisolone was initiated with bumetanide, with improvement in eosinophilia, rash and volume status. She was discharged of prednisone as initial treatment for her AITL, with planned oncology follow-up to discuss combination chemotherapy options.

Discussion: AITL is the most common peripheral T-Cell lymphoma (20% of cases) and tends to be aggressive, with 5-year mortality of 30% with a 1.7 year median survival. On presentation, AITL can include generalized lymphadenopathy, fever, hepatosplenomegaly, anemia, eosinophilia and polyclonal hypergammaglobulinemia. There have been several reports describing this DRESS-mimicker. In a 2019 letter to the Journal of Allergy and Clinical Immunology: In Practice, a series including 5 AITL and 17 DRESS cases were discussed, specifically outlining differences between these diagnoses when lymphadenopathy was a prominent feature. Of note, all cases included European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scores greater than 4, indicating “probable” DRESS diagnosis. Clinical features more prevalent in the AITL group included: low drug causality probability, shorter latent period, less hepatic involvement, greater lymphadenopathy and lymph nodes or spleen with lymphoma specific tissue identified on biopsy.

Conclusions: In our patient, the latent period from the beta-lactam administration was less than the typical 2-8 weeks, and hepatic involvement was notably absence. The delay in rash presentation compared to lymph node and WBC abnormalities was also inconsistent. In conclusion, any DRESS-like presentation with predominant lymphadenopathy and atypical features should be strongly considered for lymph node biopsy given it’s significant phenotypic overlap with AITL.