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[Intravascular large B-cell lymphoma with pontine involvement successfully treated with R-hyper-CVAD/R-MTX-Ara-C

Tomonori Nakazato1, Kazuhito Suzuki, Ai Mihara

  • 1Department of Hematology, Yokohama Municipal Citizen's Hospital.

[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|April 10, 2010
PubMed

Insights

Intravascular large B-cell lymphoma (IVLBCL) with central nervous system (CNS) involvement can be effectively treated. A novel alternating chemotherapy regimen achieved complete remission in a challenging case.

Area of Science:

  • Hematology
  • Oncology
  • Neurology

Background:

  • Intravascular large B-cell lymphoma (IVLBCL) is a rare extranodal non-Hodgkin lymphoma.
  • Diagnosis can be challenging due to the absence of palpable lymphadenopathy and tumor cells confined to the vasculature.
  • Central nervous system (CNS) involvement is a serious complication, necessitating aggressive treatment strategies.

Observation:

  • A 47-year-old woman presented with fever, cough, hepatosplenomegaly, and pancytopenia.
  • Bone marrow biopsy revealed atypical lymphoid cells positive for CD20 and CD79a within capillaries, confirming IVLBCL.
  • Despite normal neurological exams and CSF, a T2-hyperintense lesion in the pons on head MRI indicated CNS involvement.

Findings:

  • The patient received alternating R-hyper-CVAD and R-MTX-Ara-C chemotherapy with intrathecal MTX.
  • A single cycle of R-hyper-CVAD led to the disappearance of the pontine lesion on MRI.
  • Five cycles resulted in complete remission, demonstrating a positive response to the treatment regimen.

Implications:

  • The R-hyper-CVAD/R-MTX-Ara-C alternating regimen is a viable and effective treatment for IVLBCL with CNS involvement.
  • Early detection and aggressive therapy are crucial for improving outcomes in this rare lymphoma subtype.
  • This case highlights the importance of advanced imaging in diagnosing occult CNS disease in IVLBCL.

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