[Intravascular NK-cell lymphoma: a clinicopathologic study and literature review].
OBJECTIVE
To study the clinicopathologic features and disease outcome of intravascular natural killer-cell lymphoma (IVNKL).


METHODS
The histologic features, immunohistochemical findings and results of in-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) were analyzed in 2 novel cases of IVNKL. Seven cases of IVNKL previously reported in the literature were reviewed.


RESULTS
The patients were a 68-year-old woman and a 22-year-old man. They both presented with erythematous patches and nodules on their trunk and extremities. Skin biopsies confirmed the diagnosis of IVNKL. The tumor cells were positive for CD3, CD56, granzyme B and EBER. Both patients died 2 months after the diagnosis. Amongst the 9 reported cases, including those from the literature, the male was 4 cases, the female was 5 cases. The mean age of the patients was 45.7 years and the median age was 47 years. Skin lesions represented the commonest clinical manifestations. Multiple organ involvement was found in 7 cases and central nervous system was involved in 3 cases. Six patients died during 2 to 17 months of follow-up. The median survival was 9 months and the one-year survival rate was (35.6±18.6)%. The clinical outcome of the patients with multiple organ involvement was worse than that with skin manifestations only. The difference however was not statistically significant (P=0.083).


CONCLUSIONS
IVNKL is a rare disease. Diagnosis should be made according to typical histologic findings, immunophenotype and EBER in-situ hybridization results. The overall prognosis of IVNKL is poor. Early diagnosis and treatment before multiorgan involvement may be helpful in improving the clinical outcome.
Department of Pathology, Beijing Friendship Hospital, Capital University of Medical Science, Beijing 100050, China.
Lei
L
Jiang
Jian-lan
JL
Xie
Xiao-ge
XG
Zhou
D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017252:CD3 Complex / Q000378:metabolism; D019002:CD56 Antigen / Q000378:metabolism; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D053804:Granzymes / Q000378:metabolism; D006801:Humans; D007694:Killer Cells, Natural / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D008228:Lymphoma, Non-Hodgkin / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D012367:RNA, Viral / Q000378:metabolism; D019043:Vascular Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
D017252:CD3 Complex; D019002:CD56 Antigen; C099537:Epstein-Barr virus encoded RNA 1; C106732:Epstein-Barr virus encoded RNA 2; C522918:NCAM1 protein, human; D012367:RNA, Viral; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D053804:Granzymes; D011241:Prednisone