| Objective:To report and analyze a case of hydroa vacciniforme-like lymphoma to review the pathology, clinical
features, diagnosis, treatment and prognosis of this kind of lymphoma, in order to improve its understanding for clinic.Methods:Reported
a case with 7 years history of Hydroa Vacciniforme and translated into systemic T-cell lymphoma cases finally, through the skin biopsy,
pathologic and immunohistochemical, TCR gene rearrangement, laboratory test, MRI and imaging examination, we made a clinical
diagnosis and treatment plan, then to find outcome. The final is the literature review.Results:The clinical features of this case are facial
edema, papulovesicles and blister, nasal septum and inferior nasal concha were involved. At the same time, the patient had fever,
lymphadenopathy. Monoclonal T cell receptor (TCR)-gamma gene rearrangement in the skin biopsy, lymphocytes and plasma cells
infiltrated in the dermis, CD3+, CD4+, CD8+, CD30+, CD56 +, EBER (+）, MIB-1 60 %. With treatment of interferon in the early stage, it
could be controlled well for 7 years. After that disease progressed, the patient was diagnosed Hydroa vacciniforme-like cutaneous T cell
lymphoma and accepted MESA chemotherapy. Finally, she presented the symptoms of central nervous system and died.Conclusion:In
the early stage, hydroa vacciniforme-like lymphoma is resembled to hydroa vacciniforme very much. It can be confirmed by pathological
biopsy and immunohistochemical analysis. This disease progresses fast and has poor prognosis, parts of the patients are effective to
interferon and steroid, but the effect of chemotherapy is still unclear. |
