A 26-year-old man presented with 2 months of progressive encephalopathy, right inferior quadrantanopia, right hemiparesis, and facial swelling (Figure, A and B). An MRI of the brain revealed an enhancing left parietal lesion (Figure, C–E). Fluorodeoxyglucose-PET demonstrated hypermetabolism in the left parietal lesion, face, and abdominal musculature (Figure, F–G). Abdominal muscle, face, and left parietal lesion biopsies confirmed CD8+, CD30− T-cell lymphoma. His lymphoma improved with methotrexate and temozolomide (Figure, H and I).
Figure. Cutaneous and Imaging Findings in T-Cell Lymphoma.
Periorbital and perioral swelling (A and B); an MRI of the brain demonstrating a T2-hyperintense left parietal lesion and chronic right arachnoid cyst (C), with heterogeneous enhancement (D), and notable blooming or susceptibility artifact on susceptibility-weighted sequences from the paramagnetic hemosiderin in the associated blood products (E); fluorodeoxyglucose-PET demonstrating hypermetabolic activity throughout the subcutaneous facial lesions (F, arrow) and right internal abdominal oblique muscle (G, arrow); an MRI of the brain demonstrating reduction in size (H) and enhancement (I) of the left parietal lesion 2 months after treatment with high-dose methotrexate and temozolomide.
Nine years earlier, he was diagnosed with hemophagocytic lymphohistiocytosis (HLH) in the setting of fevers, elevated ferritin and soluble CD25, cytopenia, and bone marrow hemophagocytosis. There was no systemic or CNS evidence of lymphoma. His HLH resolved with dexamethasone and etoposide.
HLH can develop secondary to lymphoma.1,2 Our case highlights a unique presentation of lymphoma secondarily against a history of HLH, suggesting suspicion for T-cell lymphoma remains important when patients with hyperinflammatory syndromes develop neurologic and cutaneous symptoms.
Contributions
L. Lu: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data. A. Zhu: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data. C.Y Itoh: drafting/revision of the article for content, including medical writing for content. R.P Coburn: drafting/revision of the article for content, including medical writing for content. R. Mustafa: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data.
Study Funding
The authors report no targeted funding.
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
References
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