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Subcutaneous T-cell lymphoma presenting as panniculitis in children: report of two cases
Y F Chan1, K C Lee, H Llewellyn
1Histopathology Laboratory, Auckland Children's Hospital, New Zealand.
Insights
Peripheral T-cell lymphoma presenting as panniculitis in children can have distinct clinical presentations. Hemophagocytic syndrome indicates a poor prognosis, while some cases show indolent courses responsive to less aggressive treatment.
Area of Science:
- Pediatric Oncology
- Dermatology
- Hematology
Background:
- Peripheral T-cell lymphoma (PTCL) can manifest with subcutaneous involvement.
- Panniculitis, an inflammation of subcutaneous fat, can be a rare presentation of PTCL in children.
Observation:
- Two pediatric cases of subcutaneous PTCL presenting as panniculitis were analyzed.
- One child developed hemophagocytic syndrome and succumbed to disseminated fungal infection despite chemotherapy.
- The second child, with milder symptoms and no hemophagocytosis, achieved remission with prednisone alone.
Findings:
- Subcutaneous T-cell lymphoma is a distinct clinicopathological entity with varied presentations.
- Florid hemophagocytic syndrome is associated with a poor prognosis in this condition.
- Immunophenotypic analysis is crucial for differentiating from other panniculitis causes.
Implications:
- Early diagnosis and appropriate treatment are vital for managing subcutaneous T-cell lymphoma.
- The presence or absence of hemophagocytic syndrome influences prognostic assessment and treatment strategy.
- Combination chemotherapy is generally indicated, but indolent cases may allow for deferred aggressive treatment.
Abstract:
Two cases of peripheral T-cell lymphoma with primarily subcutaneous involvement and clinically presenting as panniculitis were reported in two children. One child developed florid hemophagocytic syndrome and was treated by combination chemotherapy but died 14 months later of disseminated fungal infection. The other child had mild systemic symptoms but no histological evidence of hemophagocytosis. Despite a more florid lymphomatous infiltrate, he attained a complete remission after a short course of prednisone and remains symptom-free at 3 years. Our report confirms that subcutaneous T-cell lymphoma is a distinct clinicopathological entity and highlights the two modes of clinical presentation. The development of florid hemophagocytic syndrome indicates a bad prognosis. This entity must be distinguished from other causes of panniculitis and immunophenotypic analysis of the atypical cells is essential in the diagnosis. Combination chemotherapy is the treatment of choice, but in patients who pursue an indolent course the administration of aggressive chemotherapy may be deferred.
