Lymphomas and Lymphoid Neoplasms

Lymphomas and Lymphoid Neoplasms

Lymphomas and Lymphoid Neoplasms

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Lymphoma Classification & Basics - Cellular Chaos Kickoff

  • Lymphoma: Malignant lymphoid cell tumors, primarily in solid tissues (nodes, extranodal). Differentiated from leukemia (marrow/blood primary).
  • WHO Classification: Based on:
    • Cell of origin (B, T, NK)
    • Morphology (cell/tissue appearance)
    • Immunophenotype (CD markers, e.g., CD20, CD3)
    • Genetics (translocations, mutations)
    • Clinical features (age, site, course)
  • Clonality: Hallmark; neoplastic cells from a single progenitor.

⭐ Most Non-Hodgkin Lymphomas (NHLs), ~85-90%, are B-cell derived.

Hodgkin Lymphoma - Reed's Rogue Roster

  • Key: Reed-Sternberg (RS) cells ("owl-eye"); classic are CD30+, CD15+. Reed-Sternberg cell with normal lymphocyte
  • Clinical: Bimodal age (15-35 & >50 yrs), contiguous spread, "B" symptoms (fever, sweats, wt loss >10%).
  • Classical HL (cHL) subtypes (RS cells CD30+, CD15+):
    • Nodular Sclerosis (NSHL): Most common; lacunar cells.
    • Mixed Cellularity (MCHL): EBV common; eosinophils.
    • Lymphocyte-Rich (LRCHL): Best prog.
    • Lymphocyte-Depleted (LDHL): Worst prog; HIV assoc.
  • Nodular Lymphocyte Predominant HL (NLPHL):
    • "Popcorn" (L&H) cells: CD20+, CD45+, BCL6+; CD15-, CD30-. Indolent.
  • 📌 Mnemonic (cHL RS): 15 x 2 = 30 (CD15+, CD30+).

⭐ Pel-Ebstein fever (cyclical fever) is rare but classic.

B-Cell NHLs - Antibody Army Uprising

Burkitt Lymphoma: Starry Sky Histology

NHLOriginGeneticsKey CDsNotes
DLBCLMature BBCL6, MYCCD20Common, aggressive
FollicularGerm Ctr Bt(14;18) BCL2CD10, BCL2Indolent, waxing/waning
CLL/SLLNaive Bdel(13q)CD5, CD23Elderly, smudge cells
BurkittGerm Ctr Bt(8;14) c-MYCCD10, BCL6"Starry sky", jaw/GI
Mantle CellNaive Bt(11;14) Cyc D1CD5, Cyc D1Aggressive, polyposis
Marginal ZMarg Zone Bt(11;18)CD20MALT (H. pylori), extranodal

⭐ CLL/SLL: Most common adult leukemia (West); CD5+, CD23+.

T-Cell & NK-Cell NHLs - T-Team Turmoil

  • Mycosis Fungoides (MF) & Sézary Syndrome (SS):
    • Cutaneous T-cell lymphoma. MF: skin patches, plaques, tumors. SS: erythroderma, Sézary cells (>1000/µL blood).
    • Key: Pautrier's microabscesses (intraepidermal atypical T-cells); Sézary cells (cerebriform nuclei).
    • Immunophenotype: CD3+, CD4+, CD8-. Mycosis Fungoides Skin Lesions
  • Peripheral T-cell Lymphoma, NOS (PTCL-NOS):
    • Diagnosis of exclusion. Heterogeneous group, often aggressive.
  • Anaplastic Large Cell Lymphoma (ALCL):
    • "Hallmark" cells (large, pleomorphic, horseshoe nuclei). CD30+.
    • ALK+ (t(2;5)): younger patients, better prognosis. ALK-: older patients, poorer prognosis.

    ⭐ ALK+ ALCL (t(2;5)) has better prognosis, common in younger patients.

  • Extranodal NK/T-cell Lymphoma, Nasal Type:
    • Aggressive; midline facial destruction (nasal). Angiocentric.
    • Strong EBV association. Immunophenotype: CD3+, CD56+ (NK cell marker).

Lymphoma Diagnosis & Staging - Sleuth & Scale System

  • Diagnosis:
    • Biopsy: Excisional (gold standard).
    • IHC, Flow cytometry: Lineage/markers.
    • Cytogenetics/Molecular (FISH, PCR): Key aberrations.
  • Staging (Ann Arbor):
    • I: Single LN region.
    • II: ≥2 LN regions, same side diaphragm.
    • III: LN regions, both sides diaphragm.
    • IV: Widespread extralymphatic.
    • Modifiers: A/B (symptoms), E (extranodal), S (spleen), X (bulky >10cm).
  • Imaging: PET-CT: Staging & response.
  • Prognosis: IPI for NHL.

⭐ "B" symptoms in lymphoma staging include unexplained fever >38°C, drenching night sweats, and unexplained weight loss >10% in 6 months.

High‑Yield Points - ⚡ Biggest Takeaways

  • Hodgkin Lymphoma: Characterized by Reed-Sternberg cells; typically CD15+, CD30+.
  • Burkitt Lymphoma: "Starry sky" appearance; t(8;14) translocation involving c-myc.
  • Follicular Lymphoma: Associated with t(14;18) (BCL2 gene); generally indolent course.
  • Diffuse Large B-cell Lymphoma (DLBCL): Most common NHL in adults; aggressive clinical behavior.
  • Mantle Cell Lymphoma: t(11;14) leading to Cyclin D1 overexpression; often poor prognosis.
  • Multiple Myeloma: Neoplastic plasma cells, M-protein, CRAB criteria (Calcium, Renal, Anemia, Bone).
  • CLL/SLL: Smudge cells on peripheral smear; CD5+, CD23+ B-cells.

Practice Questions: Lymphomas and Lymphoid Neoplasms

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A cervical lymph node biopsy shows 'starry sky' pattern. Which immunophenotype would best support Burkitt lymphoma?

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Flashcards: Lymphomas and Lymphoid Neoplasms

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Anaplastic large cell lymphoma is a _____-cell lymphoma with large lymphoid cells

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Anaplastic large cell lymphoma is a _____-cell lymphoma with large lymphoid cells

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