Blastic variant of mantle cell lymphoma: a rare but highly aggressive subtype

The blastic variant (BV) form of mantle cell lymphoma (MCL) Is considered t o be a very aggressive subtype of non-Hodgkin's lymphoma (NHL). In order to determine Its clinico-biological features and response to therapy we studi ed 33 patients (17%) out of 187 suffering from MCL who were diagnosed with a BV of MCL. Blastic variant was diagnosed according to histopathological p atterns, Immunophenotyping, and bcl1 gene rearrangement and/or cyclin DI ov erexpression. Three patients Initially diagnosed with large cell NHL were c lassified as BV. Patients received front-line therapy including CHOP-like r egimen or CVP (n = 29), or chlorambucil (n = 4) and CHOP or ESAP as second- line therapy. High-dose Intensification with stem cell transplantation (SCT ) was performed in 11 cases (autoSCT, n = 8; alloSCT, n = 3). All but two p atients were in complete remission (CR) at the time of transplant (CRI, n = 5; CR2, n = 4). Clinical and biological characteristics did not differ fro m those of the common form of MCL. The median age was 62 years (29-80), wit h a sex ratio (MIF) of 2.6:1. Of the 33 patients, 66% had extranodal site I nvolvement, 85% had an Ann Arbor stage IV, and 82% had peripheral lymphaden opathy. Circulating lymphomatous cells were seen in 48% of cases. Twelve pa tients (36%) entered a CRI with a median duration of 11 months. Fifteen pat ients (46%) failed to respond and rapidly died of progressive disease. Seco nd-line therapy led to a 26% (6/23) CR2 rate. Nine patients relapsed after high-dose therapy. Twenty-two of the 33 patients (66%) died of refractory o r progressive disease. Median overall survival (OS) time was 14.5 months fo r the 33 BV patients as compared to 53 months for the 154 patients with a c ommon form of MCL, P < 0.0001. In the univariate analysis, OS was influence d by age, extranodal site involvement, circulating lymphomatous cells, and international prognosis Index (IPI). In the multivariate analysis, only IPI affected OS: patients with IPI greater than or equal to2 had 8 months medi an OS as compared to 36 months median OS for patients with IPI <2, P = 0.00 3. Blastic variant is one of the worst forms of NHL. An improved recognitio n of BV of MCL Is required, particularly in high-grade CD5(+) NHL using Imm unophenotyping and bcl1 molecular study. Standard therapy using anthracycli ne or even high-dose Intensification produce poor results and an alternativ e treatment should be proposed to such patients.