Pathology
Pathology/C/14
Diffuse large B cell lymphoma, Burkitt lymphoma
びまん性大細胞型B細胞リンパ腫(DLBCL)/バーキットリンパ腫
- タグ
- High-yield / ポイント
A) Diffuse Large B-Cell Lymphoma (DLBCL)
Overview
- Most common type of lymphoma in adults
- Aggressive (high-grade), fast-growing B-cell lymphoma
Pathogenesis
- Frequent gene mutations:
- BCL6 gene rearrangements
- t(14;18) → BCL2 overexpression
- Loss-of-function mutations
- Two subtypes by cell of origin:
- GCB type (germinal center B-cell origin)
- ABC type (activated B-cell origin) — worse prognosis
Morphology
- No follicular pattern — diffuse proliferation of large cells
- Two main cell types:
- Centroblasts: large cells seen in reactive follicles
- Immunoblasts: antigen-activated lymphocytes, normally in paracortex
- Occasional large anaplastic cells
- High mitotic activity
Clinical features
- Any age; peak at 60 years
- Rapid lymph node enlargement
- Frequent extra-nodal manifestation: GI-tract, mediastinum, CNS
- Without treatment: aggressive and fatal
- With anti-CD20 (rituximab/MabThera): ~50% curable
B) Burkitt Lymphoma
Overview
- Aggressive (high-grade), fast-growing B-cell lymphoma
- Subtypes:
- Endemic (African): affects children; jaw tumors
- Sporadic (Europe/North America): children and young adults; abdominal tumors
- Post-transplant/immunodeficient: essentially HIV patients
Pathogenesis
- t(8;14) → fuses MYC gene to IgH → MYC overexpression → massive cell proliferation
- EBV infection plays a pathogenic role → expands B-cell pool → increases risk of malignant transformation
Morphology
- Neoplastic cells are molded against each other
- Round/oval nuclei with several nucleoli; basophilic cytoplasm containing lipid vacuoles
- Very high proliferation rate → numerous mitotic figures and apoptotic bodies
- Tingible-body macrophages phagocytose apoptotic debris → surrounded by clear space → “starry sky” appearance
Clinical features
- Aggressive tumor with rapid growth
- Mostly children or young adults
- Extra-nodal manifestation:
- Maxilla and mandible → Endemic type
- Abdomen → Sporadic type
- Leukemic manifestation may occur
- Intensive chemotherapy → potentially curable
💡 High-yield: DLBCL = most common adult lymphoma; aggressive; BCL6/BCL2; ~50% curable with anti-CD20. Burkitt = t(8;14)/MYC overexpression; EBV association; starry sky pattern; lipid vacuoles in cytoplasm; Endemic → jaw; Sporadic → abdomen.