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.