Trapped in the Vasculature: Delving into Intravascular B-cell Lymphoma

Area of Science:

• Hematology
• Oncology
• Rare Diseases

Background:

• Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL).
• Diagnosis is challenging due to rarity and neoplastic cells proliferating within blood vessels.
• Presents with vague symptoms and may involve hemophagocytic syndrome; atypical cells in peripheral blood are uncommon (approx. 5%).

Purpose of the Study:

• To report a case of IVLBCL in a 65-year-old male presenting with fever and fatigue.
• To highlight the diagnostic process and treatment of this rare lymphoma.
• To emphasize the importance of a complete diagnostic workup for IVLBCL.

Main Methods:

• Diagnosis based on peripheral blood smear, bone marrow examination, flow cytometry, and immunohistochemistry.
• Radiological evaluation included positron emission tomography (PET) scan.
• Cerebrospinal fluid (CSF) examination was performed as part of the workup.

Main Results:

• A 65-year-old male presented with fever and fatigue, with 5% atypical blast-like cells in peripheral blood.
• IVLBCL was diagnosed via bone marrow aspirate and trephine biopsy.
• The patient received six cycles of R-CHOP chemotherapy with intrathecal methotrexate and showed no recurrence at 1-year follow-up.

Conclusions:

• IVLBCL requires a comprehensive diagnostic approach, integrating laboratory and radiological studies.
• Timely diagnosis and appropriate treatment are essential for managing this aggressive lymphoma.
• Complete workup aids in differential diagnosis and determining patient prognosis.