Intravascular large B-cell lymphoma with cutaneous manifestations: a clinicopathologic, immunophenotypic and molecular study of three cases.
BACKGROUND
Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare type of malignant lymphoma characterized by exclusive or predominant growth of neoplastic cells within the lumen of blood vessels. Cases in the literature predominantly involve the skin and central nervous system, with special emphasis on the 'cutaneous variant'.


METHODS
Three cases of IVLBCL with cutaneous manifestations, including two systemic IVLBCL and one cutaneous variant, were described in this study. In all cases, clinical presentation and follow-up data were meticulously evaluated and immunophenotypic and molecular studies were performed.


RESULTS
All three cases displayed the B-cell phenotype and showed monoclonality with immunoglobulin heavy chain gene rearrangement. Bcl2 was expressed in the two systemic IVLBCL cases with fatal outcomes. The third patient with the 'cutaneous variant' achieved complete remission and a longer survival time of 15 months after chemotherapy.


CONCLUSIONS
Skin manifestations and neurological findings, although to different degrees, are important clues to the diagnosis of IVLBCL. As most IVLBCL are grouped into the post-germinal center B-cell subtype of diffuse large B-cell lymphoma, Bcl2 expression may be correlated with a worse prognosis in IVLBCL. The cutaneous variant of IVLBCL has a significantly better outcome than that of systemic IVLBCL.
Kong
Yun-Yi
YY
Department of Pathology, Cancer Hospital, Shanghai, China.
Dai
Bo
B
Sheng
Wei-Qi
WQ
Yang
Wen-Tao
WT
Wang
Chao-Fu
CF
Kong
Jin-Cheng
JC
Shi
Da-Ren
DR
D000368:Aged; D005260:Female; D015972:Gene Expression Regulation, Neoplastic; D015322:Gene Rearrangement, B-Lymphocyte; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000096:biosynthesis*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D008297:Male; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000096:biosynthesis*; D012878:Skin Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000378:metabolism / Q000473:pathology
D007143:Immunoglobulin Heavy Chains; D019253:Proto-Oncogene Proteins c-bcl-2