Related Subjects:
|Hodgkin Lymphoma
|Non Hodgkin Lymphoma
|Diffuse large B-cell lymphoma
|Intravascular large B-cell lymphoma
|Mantle cell lymphoma
|Marginal Zone Lymphoma
|Gastric (MALT) Lymphoma
|Primary CNS Lymphoma (PCNSL)
|Burkitt's lymphoma
|Follicular Lymphoma
🩸 Intravascular Large B-Cell Lymphoma (IVLBCL) is a rare, aggressive subtype of extranodal non-Hodgkin lymphoma.
It is characterized by the selective proliferation of malignant B-cells within the lumina of small to medium-sized vessels, without forming a distinct tumor mass.
⚠️ Because of its protean manifestations and lack of obvious masses, IVLBCL is frequently misdiagnosed or discovered only at post-mortem.
ℹ️ About
- Selective intravascular growth of malignant lymphocytes obstructing blood vessels. 🧬
- Absence of a discrete mass makes diagnosis particularly challenging.
- Belongs to the spectrum of diffuse large B-cell lymphomas (DLBCL).
🧬 Aetiology & Epidemiology
- A rare subtype of extranodal DLBCL.
- Some cases are EBV-associated (especially in immunosuppressed patients).
- Organ involvement: CNS (≈60%), skin (≈7%), bone marrow/spleen (≈11%), lungs (≈7%).
- Two major clinicopathologic variants:
• Western type: CNS and skin predominant involvement.
• Asian type: Haemophagocytic syndrome with fever, cytopenias, hepatosplenomegaly, and marrow involvement.
👩⚕️ Clinical Features
- Occurs mainly in the elderly.
- 🧑⚕️ Cutaneous findings: Rashes, plaques, or nodules mimicking vasculitis or panniculitis.
- 🧠 CNS disease: Encephalopathy, strokes, seizures, focal neurological deficits due to vascular occlusion.
- 🩺 Systemic: Splenomegaly, fever, B-symptoms, cytopenias.
- Asian variant: often with haemophagocytic syndrome (fever + splenomegaly + cytopenias).
- Can masquerade as infection, vasculitis, or thromboembolic disease.
🧪 Investigations
- FBC: Normocytic anaemia, thrombocytopenia; markedly raised LDH and CRP. 📈
- Skin biopsy: Demonstrates vascular occlusion by large CD20+ B-cells.
- PET-CT: Shows patchy uptake in lung, marrow, renal cortex, or CNS, though absence of a mass is typical.
- Flow cytometry: May show a clonal B-cell population in blood/marrow.
- Lumbar puncture: CSF often negative despite CNS involvement.
- CXR: Usually unremarkable despite systemic illness.
🧫 Pathology
- Neoplastic B-cells confined to vessel lumina, obstructing small capillaries/venules. 🩸
- Cells strongly express CD20 and other pan-B markers (e.g., CD79a, PAX5).
- Histology lacks parenchymal infiltration or nodal involvement in most cases.
💊 Management
- ⚠️ IVLBCL is highly aggressive with poor prognosis if untreated; many diagnoses occur post mortem.
- Systemic chemotherapy: CHOP-based regimens combined with Rituximab (R-CHOP) improve outcomes significantly.
- CNS-directed therapy: Intrathecal methotrexate ± high-dose systemic methotrexate, due to high CNS relapse risk. 🧠
- Supportive care: Manage cytopenias, coagulopathy, infections, and organ failure.
- Early recognition and biopsy (skin or marrow) are critical to survival.
📚 References
