Clinical features of aggressive lymphomas

~50% of cases; rapidly progressive if untreated.

Diffuse large B-cell lymphoma: most common lymphoma diagnosis; occurs at all ages, generally >40 years; presents with localised stage I or II disease in 50% of patients but disseminated extranodal disease is not uncommon; constitutional symptoms in 33%; extranodal sites in 30-40% most commonly GI. Ascites and pleural effusions are common end-stage symptoms.

T-cell rich B-cell lymphoma: subtype of DLBCL; occurs in younger patients; more aggressive with early BM involvement.

Mature (or peripheral) T-cell lymphomas: a number of different conditions; most common T-cell NHL in the West but more common in Far East; median age 56; 9:3 ratio 2:1; variable clinical behaviour; nodal form generally more aggressive and less responsive to therapy than DLBCL; heterogeneous group of extranodal forms; 80% stage III-IV at diagnosis; constitutional symptoms, BM and skin involvement common; 41% 5 year survival with combination therapy.

Mycosis fungoides: mature T-cell lymphoma; presents as localised or generalised plaque or erythroderma; lymphadenopathy in 50%; median survival 10 years but prognosis poor with lymphadenopathy, blood (fflSezory syndrome p190) or visceral involvement .

Angio-immunoblastic lymphadenopathy: constitutional symptoms, generalised lymphadenopathy, hepatosplenomegaly, skin rash, polyclonal hypergammaglobulinaemia, DAT+ve haemolytic anaemia and eosinophilia; 33% of patients progress to immunoblastic lymphoma; poor prognosis.

Mantle cell lymphoma: usually elderly; median 63 years; B symptoms 50%; usually disseminated at diagnosis: BM involvement 75%, GI involvement 15-20%; poor therapeutic outcome: partial responses and eventual chemoresistance; median survival 3-4 years (fflp184).

198 Mediastinal large B-cell lymphoma: typically occurs in women <30 years; anterior mediastinal mass sometimes causes superior vena caval obstruction; tendency to disseminate to other extranodal sites including CNS; cure rate with therapy similar to LBCL.

Anaplastic large cell lymphoma: usually occurs in younger patients and children; typically as lymphadenopathy at a single site; favourable prognosis as curable with chemotherapy; 64% overall 5 year survival.

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