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Pathology

Clonal proliferation of abnormal B-lymphocytes (Reed-Sternberg cells) with reactive proliferation of other T- and B-lymphocytes.

Aetiology

Often no cause found, may follow EBV infection, increased incidence if HIV positive, increased risk in first degree relatives of affected individuals
Usually affects young adults / adolescents

Signs

Painless cervical lymphadenopathy, hepatomegaly, splenomegaly, SVC obstruction, bronchial obstruction, pleural effusion, infection, e.g. shingles

Symptoms

‘B’ symptoms common (weight loss, anorexia, sweats, fever, fatigue), pruritus, painful lymph nodes on drinking alcohol

Investigations

Bloods: FBC – normochromic normocytic anaemia, thrombocytosis, 
lymphocytopenia, Raised ESR and ALP
Imaging: CT Chest, abdomen and Pelvis for staging using Ann Arbor System
Bone Marrow Aspirate Used for staging using Ann Arbor System

Treatment

Medical: Chemotherapy, Autologous stem cell transplant in relapsed disease
Allogeneic stem cell transplant in refractory/multiply relapsed disease .

Prognosis

Variable depending on stage
Stage I/II: Good risk, classical HL 5 year overall survival 95%
Stage III/IV: Poor risk classical HL 5 year overall survival 59%
Early: Infection, bone marrow failure, organ damage
Late: Reduced fertility, premature menopause, secondary malignancy, lung 
fibrosis, lung / thyroid toxicity

Key Facts

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Key Images

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Key References

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