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[Intravascular lymphomatosis diagnosed by bone marrow biopsy]
M Nohgawa1, T Moriguchi, M Tsudo
1Hematology Division of Internal Medicine, Kyoto-Katsura Hospital.
[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|October 6, 2000
Summary
Intravascular lymphomatosis (IVL) is a rare cancer where lymphoid cells clog small vessels. Early bone marrow biopsy is crucial for diagnosing this challenging condition, leading to effective chemotherapy treatment.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Intravascular lymphomatosis (IVL) is a rare neoplastic proliferation of lymphoid cells within small vessel lumina.
- Diagnosis is challenging due to the absence of tumor masses and subtle peripheral blood findings.
Observation:
- A 69-year-old woman presented with pancytopenia and elevated LDH and sIL-2R levels.
- Initial bone marrow aspiration was inconclusive, but later biopsy revealed lymphoma cells within bone marrow vessels.
- The patient developed respiratory failure secondary to pulmonary embolisms.
Findings:
- Diagnosis of IVL was confirmed via bone marrow biopsy showing lymphoma cells within vessels.
- Treatment with CHOP chemotherapy regimen led to dramatic improvement in respiratory failure and normalization of peripheral blood counts.
- Subsequent low-dose etoposide therapy prevented relapse.
Implications:
- Highlights the diagnostic difficulty of IVL and the importance of bone marrow biopsy when IVL is suspected.
- Demonstrates the efficacy of the CHOP regimen and etoposide in managing IVL.
- Emphasizes the need for high index of suspicion for IVL in patients with unexplained cytopenias and organ dysfunction.