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Prognostic factors

Thrombocytopenia is an adverse prognostic factor for survival from diagnosis of chronic GvHD. Other factors associated with poor outcome are progressive onset, lichenoid skin rash, elevated bilirubin, poor performance status, alternative donor and sex mismatched donor.

Treatment

Discuss with a senior member of transplant team. General measures

1. Adequate nutrition, vitamin/calorie supplements may be required and severe cases may require TPN.

2. Pneumococcal prophylaxis must be continued lifelong.

3. Consider restarting conventional prophylactic antifungal and antibacterial agents.

4. CMV surveillance is critical (reactivation is more common).

5. P corin/'/prophylaxis must be commenced with cotrimoxazole or nebulised Pentamidine and continued for 6 months after immunosuppres-sive therapy.

6. Psychological support may be required to adjust to chronic disability.

Specific treatment

1. Commonest protocol used is the Seattle regimen of prednisolone and cyclosporin A on alternate days; typically: begin daily prednisolone Img/kg/day with cyclosporin A 10mg/kg/day divided bd.

2. If disease stable or improved after 2 weeks taper prednisolone by 25% per week to target dose of 1mg/kg every other day.

3. After successful completion of steroid taper, reduce cyclosporin A by 25% per week to alternate day dosage of 10mg/kg/day divided bd.

4. If resolved completely at 9 months, slowly wean patient from both medications with dose reductions every 2 weeks. 327

5. Incomplete responses should be re-evaluated after 3 months more therapy; if fail to respond or progress then salvage therapy required.

6. If no response or progression add in azathioprine 1.5mg/kg/d initially (monitor FBC, renal and liver function).

7. Severe refractory cases may respond to thalidomide, tacrolimus, hydroxychloroquine or mycophenolate mofetil (all of which may at least have a steroid sparing effect) or experimental measures such as extracorporeal PUVA therapy or anti-lymphocyte globulin.

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