The New England Journal of MedicineImages in Clinical Medicine: Intravascular Large B-Cell Lymphoma

A 53-year-old woman’s case of intravascular large B-cell lymphoma (IVLBCL), a rare and often aggressive extranodal lymphoma, underscores the diagnostic challenges posed by this disease. The patient’s nonspecific symptoms and isolated skin involvement, consistent with the cutaneous subtype, highlight the disease’s heterogeneous presentation.

Key Points

• The patient presented with a 3-month history of worsening vascular skin lesions and a 1-month history of fever.
• Physical examination revealed diffuse telangiectasias, hyperpigmented plaques, and several ulcerated nodules on the skin across the chest, abdomen, and legs.
• Laboratory studies showed a lactate dehydrogenase level of 356 U per liter (reference range, 120 to 250).
• A deep skin-biopsy specimen from the abdomen showed intravascular aggregation of round, atypical lymphocytes.
• Immunohistochemical staining was positive for CD20, PAX-5, and MUM-1 in the neoplastic cells.
• The patient was diagnosed with intravascular large B-cell lymphoma and treatment with systemic chemotherapy was initiated.
• At the 6-month follow-up visit, the patient continued to be in remission.

HCN Medical Memo
People with the cutaneous variant of IVLBCL typically have a better outlook, with research finding an overall 3-year survival rate of 56%. This may be because the presence of skin lesions leads these individuals to seek diagnosis and treatment earlier.

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