The blastic variant (BV) form of @entity1073 ( @entity1073 ) is considered to be a @entity1893 ( @entity1893 ). In order to determine its clinico-biological features and response to therapy we studied 33 @entity1 (17%) out of 187 suffering from @entity1073 who were diagnosed with a BV of @entity1073 . Blastic variant was diagnosed according to histopathological patterns, immunophenotyping, and @entity4910 gene rearrangement and/or @entity4910 overexpression. Three @entity1 initially diagnosed with large cell @entity1893 were classified as BV. @entity1 received front-line therapy including @entity3726 -like regimen or CVP (n = 29), or @entity5805 (n = 4) and @entity3726 or ESAP as second-line therapy. High-dose intensification with @entity8 ( @entity8 ) was performed in 11 cases (autoSCT, n = 8; alloSCT, n = 3). All but two @entity1 were in complete remission (CR) at the time of transplant ( @entity3432 , n = 5; @entity10911 , n = 4). Clinical and biological characteristics did not differ from those of the common form of @entity1073 . The median age was 62 years (29-80), with a sex ratio (M/F) of 2.6:1. Of the 33 @entity1 , 66% had extranodal site involvement, 85% had an Ann Arbor stage IV, and 82% had @entity1897 . @entity1669 cells were seen in 48% of cases. Twelve @entity1 (36%) entered a @entity3432 with a median duration of 11 months. Fifteen @entity1 (46%) failed to respond and rapidly died of progressive disease. Second-line therapy led to a 26% (6/23) @entity10911 rate. Nine @entity1 relapsed after high-dose therapy. Twenty-two of the 33 @entity1 (66%) died of refractory or progressive disease. Median @entity1349 ( @entity1349 ) time was 14.5 months for the 33 BV @entity1 as compared to 53 months for the 154 @entity1 with a common form of @entity1073 , P <0.0001. In the univariate analysis, @entity1349 was influenced by age, extranodal site involvement, circulating @entity1358 , and international prognosis index (IPI). In the multivariate analysis, only IPI affected @entity1349 : @entity1 with IPI > or =2 had 8 months median @entity1349 as compared to 36 months median @entity1349 for @entity1 with IPI <2, P = 0.003. Blastic variant is one of the worst forms of @entity1893 . An improved recognition of BV of @entity1073 is required, particularly in high-grade @entity5893 + @entity1893 using immunophenotyping and @entity4910 molecular study. Standard therapy using @entity4349 or even high-dose intensification produce poor results and an alternative treatment should be proposed to such @entity1 .

Blastic variant of XXXX : a rare but highly aggressive subtype.

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients'] @entity3432 :: ('1378', 'Gene') :: ['CR1'] @entity8 :: ('MESH:D007674', 'Disease') :: ['SCT', 'stem cell transplantation'] @entity10911 :: ('1380', 'Gene') :: ['CR2'] @entity4349 :: ('CHEBI:48120', 'Chemical') :: ['anthracycline'] @entity1669 :: ('MESH:D013967', 'Disease') :: ['Circulating lymphomatous'] @entity3726 :: ('1649', 'Gene') :: ['CHOP'] @entity4910 :: ('595', 'Gene') :: ['bcl1', 'cyclin D1'] @entity5893 :: ('921', 'Gene') :: ['CD5'] @entity5805 :: ('MESH:D002699', 'Chemical') :: ['chlorambucil'] @entity1897 :: ('MESH:D010523', 'Disease') :: ['peripheral lymphadenopathy'] @entity1358 :: ('MESH:D002292', 'Disease') :: ['lymphomatous cells'] @entity1073 :: ('MESH:D020522', 'Disease') :: ['MCL', 'mantle cell lymphoma'] @entity1893 :: ('MESH:D008228', 'Disease') :: ['NHL', "very aggressive subtype of non-Hodgkin's lymphoma"] @entity1349 :: ('MESH:D011475', 'Disease') :: ['OS', 'overall survival']
@entity1073 :: (MESH:D020522,Disease) :: ['mantle cell lymphoma']
