Hodgkin’s Lymphoma

Overview

Hodgkin’s lymphoma is a malignancy arising in the lymphoid tissues (mainly lymph nodes). The cell of origin is the “B” lymphocyte.

Etiology: Epstein-Barr virus is thought to play a major role in most cases.

Epidemiology

Hodgkin’s lymphoma has a peak incidence in two age groups (bimodal):

  • Young adulthood (in the 20s)
  • Above 50

Signs and Symptoms

  1. Lymph Node Enlargement:

    • Commonest Feature
    • Most common is enlargement of axillary, cervical, supraclavicular & inguinal nodes
    • Nodes are painless and rubbery
    • Nodes become acutely painful after alcohol intake (2-3% of patients)
  2. Systemic Features (B Symptoms):

    • Low-grade fever
    • Night sweats
    • Weight loss (at least 10% of body weight in less than 6 months)
    • Note: B Symptoms may or may not be present.
    • Pruritus
  3. Other Findings:

    • Splenomegaly (often seen)
    • Hepatomegaly (possible but not very common)
    • Features of nephrotic syndrome (pathology in the glomerulus is minimal change disease)Z

Pel-Ebstein Fever

  • A cyclic pattern of fever, rarely seen in Hodgkin’s patients
  • Fever present for a few days, then absent for a few days (cycle continues)

Diagnostic Feature

The diagnostic feature is the presence of malignant Reed-Sternberg cells (R-S cells), seen on lymph node biopsy.

  • Large cell with two nuclei, looking like “owl eyes

Classification of Hodgkin’s Lymphoma

  1. Classic Hodgkin’s Lymphoma (Most Cases):

    • Lymphocyte-rich: RS cells + excess lymphos.
    • Lymphocyte-depleted: RS cells plus very few lymphos
    • Mixed cellularity: Almost equal number of RS cells and lymphos
    • Nodular sclerosis: RS cells plus lymphos plus fibrous tissue
  2. Nodular Lymphocyte Predominant Hodgkin’s Lymphoma (Only 10% of Cases)

Diagnosis & Workup

  • Diagnosis: Confirmed by lymph node biopsy showing R-S cells. - After diagnosis, workup is done for staging the lymphoma, so that treatment options can be decided
  • Workup:
    • Routine blood tests (CBC, electrolytes, etc.)
    • Imaging:
      • Whole body imaging (CT, MRI, or PET scan) to determine affected lymph nodes.

Stages of Hodgkin’s Lymphoma

Based on which lymph node regions are affected:

  • Stage 1: Involvement of only one lymph node region
  • Stage 2: Involvement of two or more lymph node regions on the same side of the diaphragm.
  • Stage 3: Involvement of two or more lymph node regions on both sides of the diaphragm.
  • Stage 4: Disseminated disease (involvement of other organs like the liver).

A/B Designation:

  • A: No systemic symptoms (B symptoms)

  • B: Presence of systemic symptoms (B symptoms)

  • Stage 1 A, or 1 B

  • Stage 2 A, or 2 B

  • Stage 3 A, or 3 B

  • Stage 4 A or 4 B

Differential Diagnosis

Treatment

  • Radiotherapy: Used alone or with chemotherapy.

    • Mantle field (neck, chest, and axilla)
    • Inverted Y field (abdomen, spleen, and pelvis)
  • Chemotherapy:

    • ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine): Standard initial treatment in the U.S.
    • Other regimens: MOPP, Stanford V(5), BEACOPP
  1. MOPP:
  • Mustargen, Oncovin, Prednisone, Procarbazine
  • Was used in the past. Rarely used now( only in relapse)
  1. ABVD:
  • Adriamycin, Bleomycin, Vinblastine, Dacarbazine
  • Standard initial treatment in the U.S.
  1. Stanford V: Contains multiple drugs Used in some countries

  2. BEACOPP Contains multiple drugs Used mainly in Europe

Prognosis

Prognosis of HD depends on many factors but overall, it’s a curable cancer in many patients

  • Overall 5-year survival rate is 85-90%.
  • Poor Prognostic Factors:
    • Old age
    • Advanced disease (Stage 4)
    • Presence of B symptoms
    • Large intrathoracic tumor mass