Disseminated Hodgkin Lymphoma: Definition and Clinical Presentation
Disseminated Hodgkin lymphoma refers to Stage IV disease, characterized by diffuse (multifocal) involvement of one or more extralymphatic organs with or without associated lymph node involvement, or isolated extralymphatic organ involvement with distant (nonregional) nodal involvement. 1
What "Disseminated" Means in HL
Stage IV disease represents the most advanced stage of Hodgkin lymphoma, where the cancer has spread beyond the lymphatic system into organs such as the liver, bone marrow, lungs, or other extralymphatic sites. 1 This is distinct from:
- Stage III: Lymph node regions on both sides of the diaphragm (which may be what you're describing with "lymphadenopathy on both sides of the diaphragm") 1
- Stage IV: Actual organ involvement beyond just lymph nodes 1
Why Bilateral Diaphragmatic Involvement is Classical
Lymphadenopathy on both sides of the diaphragm alone represents Stage III disease, not disseminated (Stage IV) disease. 1 This presentation is actually quite common in classical Hodgkin lymphoma because:
- HL typically spreads in a contiguous, predictable pattern through the lymphatic system from one nodal region to adjacent regions 2
- Stage III disease (both sides of diaphragm) is considered "advanced stage" along with Stage IV, and represents a substantial proportion of HL presentations 1
- The disease can involve the spleen (IIIS) or localized extranodal sites (IIIE) while still being Stage III 1
Critical Distinction for Staging
The key difference between Stage III and Stage IV (disseminated) is:
- Stage III: Lymph nodes on both sides of diaphragm ± spleen ± localized extranodal extension 1
- Stage IV: Diffuse/multifocal organ involvement (liver parenchyma, bone marrow, lung parenchyma beyond direct extension) 1
Clinical Implications
Stage III disease with lymphadenopathy on both sides of the diaphragm is classified as "advanced stage" but is NOT the same as disseminated disease. 1 Both Stage III and IV are treated with longer courses of chemotherapy (typically 6-8 cycles) often without radiation, but the distinction matters for:
- Prognostic scoring: The International Prognostic Score (IPS) specifically includes Stage IV as an adverse factor 3
- Treatment intensity: Stage IV may warrant more aggressive initial therapy 4, 5, 6
- Bone marrow biopsy indication: Required in advanced-stage disease to confirm or exclude Stage IV 1
Symptom Modifiers
Each stage is further classified as "A" (no systemic symptoms) or "B" (presence of fever >38°C, night sweats, or >10% weight loss in 6 months). 1, 3 B symptoms are present in a significant proportion of advanced-stage patients and influence prognosis and treatment decisions.