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Lymphoma Basics - Genesis & Types

  • Lymphomas: Malignant clonal proliferation of lymphocytes or their precursors.
  • Typically arise in lymph nodes; can involve extranodal sites.
  • Broadly classified into two main types:
    • Hodgkin Lymphoma (HL)
    • Non-Hodgkin Lymphoma (NHL)
  • Key distinction:
    • HL: Characterized by pathognomonic Reed-Sternberg (RS) cells.
    • NHL: More common, diverse group; lacks RS cells.

⭐ Lymphomas are broadly classified into Hodgkin Lymphoma (HL), characterized by Reed-Sternberg cells, and Non-Hodgkin Lymphoma (NHL), a more diverse group.

Hodgkin Lymphoma - Classic Reed's Story

  • Pathognomonic Cell: Reed-Sternberg (RS) cell - large, binucleated or multinucleated, prominent eosinophilic nucleoli ('owl-eye' appearance). Reed-Sternberg cell with owl-eye appearance

  • B Symptoms: 📌 Fever (>38°C), drenching night sweats, unexplained weight loss (>10% in 6 months).

  • HL Subtypes:

    SubtypeFeaturesPrognosis
    Nodular SclerosisMost common, young adults, mediastinal massGood
    Mixed CellularityEBV association (~70%), older adultsFair
    Lymphocyte-RichBest prognosis, few RS cellsExcellent
    Lymphocyte-DepletedRarest, HIV/immunocompromised, many RS cellsPoor

⭐ Nodular Sclerosis is the most common subtype of Hodgkin Lymphoma in young adults, often presenting with a mediastinal mass and associated with good prognosis.

  • Simplified Ann Arbor Staging:

Non-Hodgkin Lymphoma - Diverse & Diffuse

  • Heterogeneous group of lymphoid malignancies; more common than Hodgkin. Often disseminated at diagnosis. Classified by cell type (B, T, NK) & aggressiveness.
  • 📌 NHL Translocations: Follicular $t(14;18)$ (BCL2), Mantle $t(11;14)$ (Cyclin D1), Burkitt $t(8;14)$ (c-MYC).

Common NHL Subtypes:

NHL TypeOriginKey Feature(s) / TranslocationMarkers
DLBCLB-cellMost common, aggressiveCD20+
FollicularB-cellIndolent, BCL2 ($t(14;18)$)CD20+, CD10+
Mantle CellB-cellAggressive, Cyclin D1 ($t(11;14)$)CD20+, CD5+
BurkittB-cellHighly aggressive, c-MYC ($t(8;14)$), 'starry sky'CD20+, CD10+
Marginal Zone (MALT)B-cellIndolent, chronic inflammation (e.g., H. pylori)CD20+
Mycosis FungoidesT-cellCutaneous, Pautrier's microabscessesCD3+, CD4+
ALCLT-cellALK+ ($t(2;5)$ good prognosis), "hallmark" cellsCD30+, ALK+/-

⭐ Burkitt Lymphoma, an aggressive B-cell NHL, is associated with $t(8;14)$ translocation involving the c-MYC oncogene and classically shows a 'starry sky' appearance on histology due to tingible body macrophages.

Lymphoma Management - Dx & Rx Blueprint

  • Diagnosis (Dx):
    • Gold Standard: Excisional LN biopsy.
    • Key Tests: IHC, Flow cytometry.
    • Staging: Ann Arbor; PET-CT (staging, response). BMB.
    • Labs: CBC, LDH, Uric acid.
  • Prognostic Scores:
    ScoreKey Factors
    IPI (NHL)Age >60, ↑LDH, PS ≥2, Stage III/IV, >1 extranodal site
    IPS (HL)Alb <4, Hb <10.5, M, Age ≥45, Stg IV, WBC ≥15k, Lymph <0.6k/<8%
  • Treatment (Rx) Overview:
    • HL: ABVD, BEACOPP; ISRT.
    • NHL: Aggressive (DLBCL) → R-CHOP. Indolent (FL) → Watch & Wait / R-chemo.
    • Relapsed/Refractory: Salvage, ASCT, CAR-T.

⭐ PET-CT scan is essential for initial staging, assessing treatment response (e.g., using Deauville criteria), and detecting relapse in most lymphomas.

Interim PET in DLBCL: SUVmax reduction and Deauville score

High‑Yield Points - ⚡ Biggest Takeaways

  • Hodgkin Lymphoma (HL) is characterized by pathognomonic Reed-Sternberg cells.
  • Ann Arbor staging is crucial for both HL and Non-Hodgkin Lymphoma (NHL).
  • NHL is more common than HL; Diffuse Large B-cell Lymphoma (DLBCL) is the most common aggressive NHL.
  • Burkitt Lymphoma: features t(8;14) (c-myc oncogene), "starry sky" appearance.
  • Follicular Lymphoma: features t(14;18) translocation (BCL-2 overexpression).
  • Mantle Cell Lymphoma: features t(11;14) translocation (Cyclin D1 overexpression).

Practice Questions: Lymphomas

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What is the primary basis for the Working Formulation in the classification of non-Hodgkin's lymphoma?

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Flashcards: Lymphomas

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What is the most common presentation of Mantle-cell lymphoma?_____

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What is the most common presentation of Mantle-cell lymphoma?_____

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Lymphomas - Free Indian Medical PG Review