A challenging case of facial oedemas revealing a systemic peripheral T-cell lymphoma, not otherwise specified - PubMed
5 hours ago
- #T-cell lymphoma
- #facial oedema
- #PTCL-NOS
- A 20-year-old Polynesian patient presented with facial oedemas and fever, leading to a diagnosis of systemic peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS).
- Diagnosis was challenging due to heterogeneous clinical presentations and required ruling out infectious and autoimmune causes.
- Positron emission tomography and muscle biopsy revealed subcutaneous hypermetabolism and atypical CD3+ T-cell lymphocytic infiltrate, confirming T-cell lymphomatous proliferation.
- Magnetic resonance imaging identified cerebral involvement, and palpebral biopsy showed infiltration of atypical T lymphocytes expressing multiple markers.
- Treatment with polychemotherapy and haplo-identical allogeneic stem cell transplantation (HSCT) resulted in a complete metabolic response.
- The case highlights the importance of repeated biopsies and expert reviews for diagnosing PTCL-NOS, which has a variable and poor prognosis.