Cutaneous Intravascular Natural Killer-cell Lymphoma: A Rare Case and Review of the Literature

Abstract

Intravascular lymphoma (IVL) is a rare and aggressive variant of extranodal non-Hodgkin's lymphoma, characterized by almost exclusive proliferation of neoplastic lymphocytes within the blood vessel lumen, and a predilection for the skin and brain ( 1 ). Cases of IVL of T-cell or natural killer (NK)-cell lineage are even rarer. We report here a case of cutaneous intravascular NK-cell lymphoma (IVNKL) presenting with multiple erythematous, indurated, tender plaques over both the lower extremities. CASE REPORTA 42-year-old Taiwanese woman visited our dermatology clinic with complaints of increasing numbers of erythematous, indurated, tender plaques over both the lower extremities, especially the thighs, for 7 months (Fig. 1 ). Constitutional symptoms, such as malaise, dizziness, and chilly sensations, were also noted. Under the preliminary impression of panniculitis, a biopsy of the skin of the left thigh was performed. A histological study revealed many distended vessels filled with atypical large lymphoid cells in the dermis and subcutis (Fig. 2A). The tumour cells were all confined within the vessels and had large, irregular hyperchromatic nuclei with ample eosinophilic cytoplasm (Fig. 2B). Scattered mitotic figures were found. Fibrin thrombi mixed Fig. I. A 42-year-old Taiwanese woman with increasing number of erythematous indurated tender plaques over both the lower extremities for 7 months.with atypical lymphoid cells were occasionally found. The vessels were surrounded by a small number of non-neoplastic lymphocytes. Immunohistochemical studies showed that the tumour cells were CD3s% CD56% granzyme B*, ßFl , CD4 , CD5 , CD8% CD20 , CD30 , Pax5 , and TdT . Approximately 99% of the tumour cells were Ki-67-positive. In situ hybridization for Epstein-Barr virus-encoded RNA (EBER) revealed the presence of the Epstein-Barr virus (EBV) (Fig. SI, available at

Painful Cutaneous Plaques on the Lower Legs in a Middle-aged Woman

“…Intravascular LBCL is a subtype of extranodal LBCL and constitutes most intravascular lymphomas. The intravascular neoplastic cells express mature B-cell–associated antigens, and EBV is generally absent in intravascular LBCL …”

Intravascular NK/T-Cell Lymphoma: What We Know about This Diagnostically Challenging, Aggressive Disease

“…IVL is more often of B-cell nature. IVL of T and NK-cell lineage is extremely rare, with a limited number of cases with sufficient immunohistochemical and clonality data reported so far [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 ].…”

Intravascular NK-cell lymphoma: a case report and review of the literature

“…Cerroni et al [ 9 ] 63/M Skin Chemotherapy, died 6 months after diagnosis 8. Liao et al [ 10 ] 42/F Skin Chemotherapy and radiotherapy, alive with disease 14 months after diagnosis 9. Gebauer et al[ 11 ] 72/M Skin, bone marrow, CNS Chemotherapy, died 7 months after diagnosis 10.…”

“…Cerroni et al [ 9 ] + - NA - - NA + + + P 8. Liao et al[ 10 ] + - - - - - + + + ND 9. Gebauer et al[ 11 ] + - NA - - - + + + P 10.…”