Subcutaneous panniculitic T-cell lymphoma developing in a child with idiopathic myelofibrosis.
OBJECTIVE
Subcutaneous panniculitic T-cell lymphoma is reported in a child with idiopathic myelofibrosis. Both disease entities are rarely seen in children.


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.
Division of Hematology/Oncology, Chang Gung Children's Hospital, Taipei, Taiwan.
I J
IJ
Hung
T T
TT
Kuo
C F
CF
Sun
D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000150:complications / Q000473:pathology*; D015434:Panniculitis; D055728:Primary Myelofibrosis / Q000150:complications* / Q000473:pathology; D012878:Skin Neoplasms / Q000150:complications / Q000473:pathology*