Intravascular NK/T-cell lymphoma: a report of five cases with cutaneous manifestation from China.
BACKGROUND
Intravascular lymphoma is a rare type of lymphoma that frequently affects the skin and is usually of B-cell origin. This lymphoma type is very rare and not recognized as a separate entity in the 2008 World Health Organization classification of hematopoietic and lymphoid tissue tumors.


METHODS
We reported five cases of intravascular NK/T cell lymphoma with cutaneous manifestation and reviewed 12 published cases involving Chinese patients with similar characteristics.


RESULTS
All five patients were adults who exhibited red or brown patches or plaques on the lower extremities or trunk; four cases were associated with B symptoms; one case developed subsequent to a lymphoma on the face (possibly extranodal NK/T cell lymphoma, nasal type). Histopathologically, all patients exhibited abnormal, medium-sized intravascular lymphocytes in the dermis and subcutaneous tissues. All patients were positive for CD2, CD3ϵ, CD56 and cytotoxic proteins. All cases were Epstein-Barr virus (EBV) positive. Four of FIVE patients died of lymphoma within a few months of diagnosis.


CONCLUSIONS
Intravascular NK/T-cell lymphoma is a rare highly aggressive and EBV-associated lymphoma that is prone to develop in Chinese patients. The relationship between intravascular NK/T-cell lymphoma and extranodal NK/T-cell lymphoma, nasal type, requires clarification.
Department of Dermatology, Xijing Hospital, The Fourth Military Medical University, Xian, China.
Lei
L
Wang
Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Siyuan
S
Chen
Department of Dermatology, The Third Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
Han
H
Ma
Department of Dermatology, Shandong Jining No. 1 People's Hospital, Jining, China.
Dongmei
D
Shi
Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Changzheng
C
Huang
Department of Dermatology, The Third Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
Chun
C
Lu
Department of Dermatology, Xijing Hospital, The Fourth Military Medical University, Xian, China.
Tianwen
T
Gao
Department of Dermatology, Xijing Hospital, The Fourth Military Medical University, Xian, China.
Gang
G
Wang
D000293:Adolescent; D000328:Adult; D001402:B-Lymphocytes / Q000473:pathology; D018801:CD2 Antigens / Q000378:metabolism; D017252:CD3 Complex / Q000378:metabolism; D019002:CD56 Antigen / Q000378:metabolism; D005260:Female; D006801:Humans; D054391:Lymphoma, Extranodal NK-T-Cell / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D035583:Rare Diseases; D012878:Skin Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D019043:Vascular Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*
D018801:CD2 Antigens; D017252:CD3 Complex; C115335:CD3E protein, human; D019002:CD56 Antigen; C522918:NCAM1 protein, human
Epstein-Barr virus; angiotropic lymphoma; extranodal NK/T-cell lymphoma, nasal type; intravascular NK/T-cell lymphoma; intravascular lymphoma