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The New England Journal of MedicineCase 15-2024: A 73-Year-Old Woman with Worsening Rash

Identifying Angioimmunoblastic T-Cell Lymphoma: Diagnostic Challenges and Clinical Management

A 73-year-old woman presented with a worsening rash, rectal pain, and a complex medical history including eczema, psoriasis, and type 2 diabetes. Her condition evolved rapidly, leading to a diagnosis of angioimmunoblastic T-cell lymphoma (AITL). This case highlights the diagnostic challenges and clinical management strategies for AITL, emphasizing the importance of comprehensive evaluation and timely intervention in patients with complex presentations.

Key Points:

  • Initial Presentation:
    • A 73-year-old woman experienced worsening rectal pain and was evaluated in the emergency department.
    • Laboratory tests showed elevated white-cell count, absolute lymphocyte count, absolute eosinophil count, and plasma lactate level.
  • First Hospital Admission:
    • CT scan revealed a perianal collection.
    • The patient was treated with intravenous piperacillin-tazobactam and vancomycin.
    • A punch biopsy showed no cancer evidence, and the patient was discharged with amoxicillin-clavulanic acid and doxycycline.
  • Rash Development:
    • Two days after discharge, the patient developed an erosive rash in the groin area, progressing to the perineum and lower abdomen.
    • She was readmitted with skin breakdown, purulent drainage, and a diagnosis of Stevens-Johnson syndrome was considered.
  • Second Hospital Admission:
    • Examination revealed multiple ulcerative plaques and widespread erythema.
    • Laboratory tests showed leukocytosis, lymphocytosis, eosinophilia, and elevated C-reactive protein and IgE levels.
    • Treatment included intravenous acyclovir, ceftriaxone, and vancomycin.
  • Diagnostic Findings:
    • Preliminary skin biopsy showed HSV infection and psoriasiform dermatitis.
    • CT scans revealed a multilobulated mass in the lung and lymphadenopathy.
    • A biopsy of an inguinal lymph node confirmed angioimmunoblastic T-cell lymphoma (AITL).
  • Clinical Management:
    • AITL accounts for approximately 18.5% of mature T-cell lymphomas and is often associated with systemic symptoms.
    • Initial treatment typically includes aggressive chemotherapy with CHOP regimen, followed by high-dose chemotherapy and stem-cell rescue.
    • Despite treatment, the patient experienced rapid deterioration and succumbed to septic shock.

Epstein-Barr Virus (EBV)-positive cells are detected in up to 85–95% of AITL biopsies, the virus being principally located in large B-cell blasts. (Cancers)

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