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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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