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Intravascular lymphoma presenting with bone marrow involvement and leukemic phase.
Haytham Khoury1, Bakul I Dalal, Stephen H Nantel
1Leukemia/BMT Program of British Columbia, Vancouver General Hospital and Health Sciences Center, Vancouver, Canada. khaytham@uhnres.utoronto.ca
Leukemia & Lymphoma
|July 12, 2003
Summary
A rare case of intravascular lymphoma (IVL) was diagnosed in a 62-year-old man presenting with constitutional symptoms. The diagnosis was confirmed by identifying CD5+ mature B-cells in blood and bone marrow, challenging initial leukemia diagnoses.
Area of Science:
- Hematology
- Oncology
- Immunophenotyping
Background:
- Intravascular lymphoma (IVL) is a rare non-Hodgkin lymphoma characterized by proliferation of malignant lymphocytes within blood vessels.
- Diagnosis can be challenging due to nonspecific symptoms and the absence of a distinct tumor mass.
- Peripheral blood and bone marrow involvement are considered uncommon in IVL, often leading to delayed or missed diagnoses.
Observation:
- A 62-year-old male presented with constitutional symptoms and cognitive decline.
- Initial peripheral blood examination revealed pancytopenia with blast-like cells.
- Bone marrow biopsy showed extensive infiltration by vacuolated, blast-like cells, initially suggesting Burkitt's leukemia/lymphoma.
Findings:
- Immunophenotyping identified the abnormal cells as CD5-positive mature B-cells, prompting re-evaluation of the initial diagnosis.
- Confirmation of a sinusoidal pattern by immunocytochemistry was crucial for revising the diagnosis to intravascular lymphoma (IVL).
- Literature review suggests that peripheral blood and bone marrow involvement in IVL, though rare, may be under-recognized due to infrequent systematic investigation.
Implications:
- This case highlights the importance of considering IVL in the differential diagnosis of unexplained constitutional symptoms and hematologic abnormalities.
- The presence of CD5 expression on malignant cells in IVL, while increasingly reported, requires further investigation to determine its frequency and clinical significance.
- Systematic evaluation of peripheral blood and bone marrow may improve the detection rates of IVL, potentially leading to earlier treatment and better patient outcomes.