Management

• In <10% patients, often elderly with minimal or no splenomegaly and cytopenia, the disease remains relatively stable and may be observed.

• Therapy is required in patients with Hb <10g/dL, neutropenia <1.0 x 109/L, thrombocytopenia <100 x 109/L, symptomatic splenomegaly, recurrent infection, extralymphatic involvement, autoimmune complications, florid leukaemia or progressive disease.

• Supportive management is important particularly in the early stages of therapy where cytopenias can worsen: treat infections promptly. Note: increased incidence of atypical mycobacterial infections in HCL.

• Splenectomy: indicated for massive splenomegaly and beneficial in managing severe pancytopenia in patients with minimal marrow infiltration. Non-curative but 2-15% will normalise FBC for up to 25 years without further therapy. Histology shows characteristic infiltration of red pulp and atrophy of white pulp. Avoid drug therapy for 6 months after splenectomy to assess response.

• Purine analogues are the established first line therapy and most patients achieve a durable CR. CD4 lymphodepletion causes immunosuppression: require P cariniiprophylaxis and irradiated blood products.

• Deoxycoformycin 4mg/m2 IV bolus every 1-2 weeks to maximum response plus 2 cycles (generally 6-10); check creatinine clearance pre-therapy (must be >60mL/min for full dose; half dose >40mL/min) improvement begins after 2 cycles; maximum response generally 4-7 months; 90% objective responses; 75% CR; 15% in continued CR at 8 years; some patients are probably cured.

• Cladribine: infusion of 0.1mg/kg/d x 7 days will produce comparable remission rates; remission duration may be shorter; temporarily myelosuppressive, maximum 1 week after infusion; repeat at 6 months if no CR; avoid cotrimoxazole during infusions (causes rash).

• IFN-a 3 million units SC daily achieves a partial response in up to 80% but CR in <5%; continue to maximum response then cut to three times weekly for 6-24 months or indefinitely; normalisation of blood

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