Hodgkin lymphoma

Hodgkin lymphoma
The histological hallmark of Hodgkin lymphoma (HL) is the presence of Reed–Sternberg cells, large malignant lymphoid cells of B cell origin
Nodular lymphocyte-predominant HL is slowgrowing, localised and rarely fatal. Classical HL is divided into four histological subtypes from the appearance of the Reed–Sternberg cells and surrounding reactive cells. The nodular sclerosing type is more common in young patients and in women. Mixed cellularity is more common in the elderly. Lymphocyte-rich HL usually presents in men. Lymphocyte-depleted HL is rare and probably represents large-cell or anaplastic non-Hodgkin lymphoma.
WHO pathological classification of Hodgkin lymphoma
Nodular lymphocytepredominant HL 5%
Classical HL
Nodular sclerosing 70%
Mixed cellularity 20%
Lymphocyte-rich 5%
Lymphocyte-depleted Rare
Epidemiology and aetiology of Hodgkin lymphoma
• Slight male excess (1.5 : 1)
• Median age 31 yrs; first peak at 20–35 yrs and second at 50–70 yrs
Aetiology
• Unknown
• More common in patients from well-educated backgrounds and small families
• Three times more likely with a past history of infectious mononucleosis but no definitive causal link to Epstein–Barr virus infection is proven
Clinical features
There is painless, rubbery lymphadenopathy, usually in the neck or supraclavicular fossae; the lymph nodes may fluctuate in size. Young patients with nodular sclerosing disease may have large mediastinal masses which are surprisingly asymptomatic but may cause dry cough and some breathlessness.
Hepatosplenomegaly may be present but does not always indicate disease in those organs. Spread is contiguous from one node to the next and extranodal disease, such as bone, brain or skin involvement, is rare.
Clinical stages of Hodgkin lymphoma (Ann Arbor classification)
Stage I
Involvement of a single lymph node region (I) or extralymphatic site (IE)
Stage II
Involvement of two or more lymph node regions (II) or an extralymphatic site and lymph node regions on the same side of (above or below) the diaphragm (IIE)
Stage III
Involvement of lymph node regions on both sides of the diaphragm with (IIIE) or without (III) localized extralymphatic involvement or involvement of the spleen (IIIs), or both (IIISE)
Stage IV
Diffuse involvement of one or more extralymphatic tissues, e.g. liver or bone marrow
Each stage is subclassified:
A No systemic symptoms
B Weight loss > 10%, drenching sweats, fever

Investigations
Treatment of HL depends upon the stage at presentation; therefore investigations aim not only to diagnose lymphoma but also to determine the extent of disease
• FBC may be normal. If a normochromic, normocytic anaemia or lymphopenia is present, this is a poor prognostic factor. An eosinophilia or a neutrophilia may be present.
• ESR may be raised.
• Renal function tests are required to ensure function is normal prior to treatment.
• Liver function may be abnormal in the absence of disease or may reflect hepatic infiltration. An obstructive pattern may be caused by nodes at the porta hepatis.
• LDH measurements showing raised levels are an adverse prognostic factor.
• Chest X-ray may show a mediastinal mass.
• CT scan of chest, abdomen and pelvis permits staging. Bulky disease (> 10 cm in a single node mass) is an adverse prognostic feature.
• Lymph node biopsy may be undertaken surgically or by percutaneous needle biopsy under radiological guidance







Management
Historically, radiotherapy to lymph nodes alone has been used to treat localised stage IA or stage IIA disease effectively, with no adverse prognostic features. Fertility is usually preserved after radiotherapy. Young women receiving breast irradiation during the treatment of chest disease have an increased risk of breast cancer and should participate in a screening programme. Patients continuing to smoke after lung irradiation are at particular risk of lung cancer.
The majority of HL patients are now treated with chemotherapy and adjunctive radiotherapy. The ABVD regimen (doxorubicin, vinblastine, bleomycin and dacarbazine) is widely used. Patients with advanced-stage disease are most commonly managed with chemotherapy alone. Patients with disease which is resistant to therapy may be considered for autologous HSCT
Prognosis
Over 90% of patients with early-stage HL achieve complete remission when treated with chemotherapy followed by involved field radiotherapy, and the great majority are cured. Between 50 and 70% of those with advanced-stage HL can be cured.