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Subcutaneous panniculitic T-cell lymphoma developing in a child with idiopathic myelofibrosis
1Division of Hematology/Oncology, Chang Gung Children's Hospital, Taipei, Taiwan.
Insights
This case report details a rare pediatric diagnosis of subcutaneous panniculitic T-cell lymphoma in a child with idiopathic myelofibrosis. The aggressive disease led to rapid deterioration and death despite initial treatment.
Area of Science:
- Pediatric Hematology Oncology
- Dermatopathology
- Rare Diseases
Background:
- Idiopathic myelofibrosis is a rare myeloproliferative neoplasm in children.
- Subcutaneous panniculitic T-cell lymphoma (SPTCL) is an uncommon extranodal cytotoxic T-cell lymphoma.
Observation:
- A 5-year-old girl presented with pancytopenia and constitutional symptoms, diagnosed with idiopathic myelofibrosis.
- She developed proptosis and skin nodules during steroid tapering for an initial transient response.
- Skin biopsy confirmed subcutaneous panniculitic T-cell lymphoma.
Findings:
- The patient experienced high fever, profound pancytopenia, gastrointestinal bleeding, and recurrent infections.
- Computed tomography revealed an orbital mass lesion.
- The child's condition rapidly deteriorated, leading to death from sepsis within 4 months.
Implications:
- This case highlights the rare co-occurrence of idiopathic myelofibrosis and SPTCL in a pediatric patient.
- The aggressive nature of SPTCL in this context underscores the need for early recognition and management.
- This report contributes to the limited literature on pediatric SPTCL and its association with myeloproliferative neoplasms.
Purpose:
Subcutaneous panniculitic T-cell lymphoma is reported in a child with idiopathic myelofibrosis. Both disease entities are rarely seen in children.
Patient And Methods:
A girl aged 5 years and 9 months had pancytopenia and severe constitutional symptoms. Idiopathic myelofibrosis was subsequently diagnosed.
Results:
A transient response was achieved after treatment with a course of high-dose methylprednisolone therapy. However, proptosis and skin nodules developed during tapering of steroid therapy. A computed tomography scan of the orbit also revealed a mass lesion in the right lacrimal gland region. A skin biopsy specimen showed a subcutaneous panniculitic T-cell lymphoma. The clinical course was marked by high fever, profound pancytopenia, massive gastrointestinal bleeding, and severe, recurrent infections. Her condition rapidly deteriorated, and she died from polymicrobial sepsis 4 months after her initial examination.
Conclusions:
Subcutaneous panniculitic T-cell lymphoma is a distinctive clinicopathologic entity that is rarely seen in children. The association of myelofibrosis and peripheral T-cell lymphoma as seen in this has been rarely reported.