Eruptive Telangiectasia as a Presenting Manifestation of Intravascular NK-cell Lymphoma: a Rare Case Report and Review of the Literature
Keywords:
Epstein-Bar virus, NK-cell lymphoma, Intravascular lymphoma, TelangiectasiaAbstract
Intravascular NK-cell lymphoma (IVNKL) is a very rare neoplasm. Although intravascular B-cell lymphoma is recognized as a common variant of mature B-cell neoplasm, IVNKL has not even been categorized by the World Health Organization (WHO) classification. To date, a few cases of IVNKL have been reported. The most common presentation is erythematous patches and plaques on trunk and extremities. Only a single case presented with blanching erythematous patches that resemble telangiectasia. Here, we report a case of IVNKL presented with eruptive telangiectasia, papules and plaques on the upper trunk and extremities accompanied with progressive weight loss. Skin histopathology showed atypical mononuclear cell infiltrations within lumina of blood vessels. Immunohistochemical study revealed NK cell phenotype with PCR and DNA sequencing for EBV were positive.. Thorough investigations were done and showed no internal organ involvement. Treatment was started with prednisolone. Unfortunately, the patient passed away 2 months after the diagnosis because of tumor lysis syndrome.
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