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Hodgkin's lymphoma
Symptoms and signs

When to suspect

Consider HD in patients with unexplained lymphadenopathy that presents as follows:

• In young adults with cervical, supraclavicular, or mediastinal lymphadenopathy
• When accompanied by "B symptoms" which include pruritus, fevers, night sweats, or unexplained weight loss (>10% in <6 months), especially when signs or symptoms persist longer than 1 month.
• Consider occult HD in patients with severe, persistent, unexplained fevers or night sweats, even if detailed investigation shows no lymphadenopathy or hepatosplenomegaly.
• Consider HD in an asymptomatic patient if an anterior mediastinal mass is found on routine chest x-ray.

1. Lymph node enlargement

Hodgkin’s disease is primarily a disease of the lymph nodes and commonly presents as an asymptomatic lymphadenopathy that may progress to predictable clinical sites.

Location of lymphadenopathy

There is considerable evidence that HL begins in a single group of lymph nodes and then spreads to contiguous lymph nodes, especially in NSHL or MCHL.[1] This important observation, first made over 50 years ago, continues to form the basis for determination of treatment strategies in patients with apparently localized HL treated with radiation therapy alone.

• Cervical and/or supraclavicular involvement (94%).[2]
• Mediastinal involvement (70%).
• Axillary involvement (36%).
• Inguinal involvement (9%).

• All patients with axillary or inguinal involvement also had neck disease.

• Disseminated lymphadenopathy is rare in patients with Hodgkin’s disease, as is involvement of Waldeyer’s ring and occipital, epitrochlear, posterior mediastinal, presacral, popliteal, hypogastric and mesenteric sites.
• Most patients with NSHL or MCHL have a central pattern of lymph node involvement (cervical, mediastinal, paraaortic).

2. Extranodal involvement (rare)

Hodgkin’s disease may affect extranodal tissues by direct invasion (contiguity; the so-called E lesion) or by hematogenous dissemination (stage IV disease). The most commonly involved extranodal sites are the spleen, lungs, liver, and bone marrow.

• The spleen is involved more frequently in patients with adenopathy below the diaphragm, systemic symptoms, and MC histologic type.
• Involvement of the liver in an untreated patient is rare and almost always occurs with concomitant splenic involvement.
• Infiltration of the bone marrow is usually focal and almost invariably associated with extensive disease and systemic symptoms.

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3. Systemic symptoms

About 30% of patients experience systemic symptoms. They include fever, drenching night sweats, or weight loss >10% in < 6 months (so-called B symptoms) and chronic pruritus. These symptoms occur more frequently in older patients and have a negative impact on prognosis.

Clinical manifestations of subtypes

1. Nodular sclerosis

• Incidence: 80%
• Gender: Females have a slightly higher incidence.
• Age: 15-40yrs.
• Mediastinal and supradiaphragmatic lymphadenopathy is common.
• The disease mainly presents in the earlier stages (I-III).

2. Mixed cellularity

• Incidence: 20%
• Age: 30-50yrs.
• Abdominal lymphadenopathy and splenic involvement is common.
• The disease mostly presents in later stages (II-IV).

3. Lymphocyte predominant

• Incidence: 5%.
• Gender: Males.
• Age: 20-40yrs.
• Commonly presents as localized disease. Characterized by late relapses and transformation to high-grade B-NHL.
• The disease mainly presents as stage I-IIA.

4. Lymphocyte depletion

• Incidence: <5%.
• Gender: Males.
• Age: 40-80yrs.
• Underdeveloped countries.
• HIV positive individuals.
• Commonly presents as late stage disease with organ involvement (liver and bone marrow) and without lymphadenopathy.
• The disease mainly presents as stage III-IVB.

References

1. Mauch P, Kalish L, Kadin M, et al. Patterns of presentation of Hodgkin’s disease. Cancer 1993;71:2062–71.
2. Rosenberg SA, Kaplan HS. Evidence for an orderly progression in the spread of Hodgkin's disease. Cancer Res. 1966;26:1225-31.

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