When Vasculitis Is Not Vasculitis: A Case Report in Which Dynamic Immunophenotyping Revealed Hidden...

Area of Science:

• Hematology
• Oncology
• Dermatology

Background:

• Angioimmunoblastic T-cell lymphoma (AITL) is a rare, aggressive peripheral T-cell lymphoma.
• AITL often presents with symptoms mimicking autoimmune diseases, complicating diagnosis.
• Cutaneous manifestations are frequent in AITL and can cause diagnostic delays.

Purpose of the Study:

• To highlight a case of AITL misdiagnosed as IgA vasculitis due to its initial presentation.
• To emphasize the importance of early lymph node biopsy in suspected AITL cases.
• To illustrate the rapid progression and diagnostic challenges of AITL.

Main Methods:

• A case report of a 67-year-old woman with a recurrent skin rash.
• Diagnostic workup included skin biopsy, immunofluorescence, flow cytometry, and lymph node biopsy.
• Treatment involved corticosteroids initially, followed by staging for chemotherapy, but the patient deteriorated.

Main Results:

• Initial presentation mimicked IgA vasculitis with rash, but evolved to include fever, lymphadenopathy, cytopenias, and splenomegaly.
• Lymph node biopsy confirmed Angioimmunoblastic T-cell lymphoma (AITL).
• The patient experienced rapid deterioration and died of septic shock despite planned chemotherapy.

Conclusions:

• AITL can present misleadingly as IgA vasculitis.
• The presence of cytopenia, lymphadenopathy, or an aggressive clinical course warrants consideration of AITL.
• Timely diagnosis through early lymph node biopsy is critical for managing AITL.