Clinicopathologic features and management of blastoid variant of mantle cell lymphoma

Rajesh Shrestha1, Vijaya Raj Bhatt2, Guru Subramanian Guru Murthy3

  • 1a Department of Internal Medicine , Memorial Hospital of Rhode Island , Pawtucket , RI , USA.

Leukemia & Lymphoma
|March 10, 2015
PubMed

Insights

The blastoid variant of mantle cell lymphoma (MCL) is an aggressive subtype with frequent advanced-stage and extranodal involvement. Optimal therapy may involve aggressive immunochemotherapy and stem cell transplant for improved outcomes.

Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Blastoid variant mantle cell lymphoma (MCL) is an aggressive subtype, comprising less than one-third of all MCL cases.
  • It can arise de novo or transform from classical MCL, predominantly affecting men in their sixth decade.
  • Characterized by frequent extranodal involvement (40-60%), advanced stage IV disease (up to 85%), and central nervous system (CNS) involvement.

Purpose of the Study:

  • To review the characteristics, diagnosis, and treatment of the blastoid variant of MCL.
  • To highlight the challenges in diagnosis and the poor response to conventional chemotherapy.
  • To discuss potential therapeutic strategies for improving patient outcomes.

Main Methods:

  • Morphological assessment for diagnosis.
  • Immunophenotyping including CD23, CD10, and CD5 expression analysis.
  • Genetic analysis to identify complex genetic alterations.

Main Results:

  • Diagnosis is challenging, relying on morphology with variable immunophenotypic findings (e.g., CD23/CD10 positivity, CD5 negativity in a subset).
  • Increased complex genetic alterations are observed in the blastoid variant.
  • Poor response to conventional chemotherapy with a short duration of response is noted.

Conclusions:

  • The blastoid variant of MCL presents unique diagnostic and therapeutic challenges.
  • CNS prophylaxis and aggressive immunochemotherapy followed by autologous stem cell transplant may improve remission rates and survival.
  • Further research is essential to enhance understanding and clinical outcomes for this MCL subtype.