Mantle Cell Lymphoma, Blastoid Variant

Mantle Cell Lymphoma, Blastoid Variant

C. Cameron Yin, MD, PhD

Lymph node involved by MCL, blastoid variant. In this case, a “starry sky” pattern is present and the neoplastic cells resemble, in part, lymphoblasts. This case carried the t(11;14)(q13;q32).

Lymph node involved by MCL, pleomorphic variant. In this case, the neoplastic cells are large and resemble, in part, large B-cell lymphoma. This case carried the t(11;14)(q13;q32).



  • Mantle cell lymphoma (MCL), blastoid variant (BV)


  • Mantle cell lymphoma, lymphoblastoid variant

  • Mantle cell lymphoma, large cell variant

  • Mantle cell lymphoma, blastic


  • Clinically aggressive type of MCL with high-grade cytologic features: 2 major variants

  • 2 variants of MCL as stated in 2001 WHO classification

    • Classical and pleomorphic

  • 2 aggressive variants of MCL as stated in 2008 WHO classification

    • Blastoid and pleomorphic



  • Virtually all cases of MCL carry t(11;14)(q13;q32)

  • Juxtaposes CCND1 at 11q13 with IgH at 14q32 and results in Cyclin-D1 overexpression, Rb phosphorylation, and release of E2F

  • Facilitates cell cycle progression from G1 to S phase

Other Cytogenetic and Molecular Genetic Abnormalities

  • P53 overexpression and mutations

  • P16/INK4a deletions and mutations



  • Incidence

    • MCL represents approximately 6% of all non-Hodgkin lymphomas

    • MCL-BV represents at least 10-15% of all MCL cases

      • Frequency is probably higher in patients who are followed for long periods of time


  • Median age: 6th to 7th decades

  • Clinical features of MCL-BV are comparable to those of typical MCL

  • Male predominance (M:F = 2-3:1)

  • B symptoms in 30-50%; may be more common in patients with MCL-BV

  • Lymphadenopathy, generalized

  • Extranodal involvement is common

    • Gastrointestinal tract is most common site

    • Unusual extranodal sites can be involved in patients with MCL-BV

  • Most patients present with Ann Arbor clinical stage III/IV

  • MCL International Prognostic Index (MIPI) often high

    • Based on: Age, ECOG performance status, serum lactate dehydrogenase (LDH), blood leukocyte count

  • Rare prolymphocytoid variant of MCL is probably a form of MCL-BV

    • Patients present with high leukocyte count (often > 100 K/uL) and poor prognostic features

      • Extensive bone marrow involvement

      • Prominent splenomegaly

      • Often short survival


  • Aggressive chemotherapy &/or stem cell transplantation

    • R-HyperCVAD used at many institutions

      • Rituximab, hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone alternating with methotrexate and cytosine arabinoside

    • R-CHOP is inadequate therapy for long-term cure

      • Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone


  • Short durations of clinical response after chemotherapy and poorer overall survival

  • MCL-BV patients have poor overall survival

    • Reported to be 14.5 months in 1 study


Radiographic Findings

  • Widespread lymphadenopathy

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Mantle Cell Lymphoma, Blastoid Variant

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