Kaposi sarcoma

Signs and symptoms

Single or multiple purple patches or nodes, mainly mouth, skin, conjunctiva, lung, GI tract; +/- enlarged lymph nodes; +/- oedema / pleural effusions.

Children: presentation differs from adults. Enlarged lymph nodes in the neck, axilla, and inguinal regions are most common. Classic skin lesions are absent in nearly half. Adolescents similar to adults; may have woody oedema. CD4 count may be completely normal.

Investigations

Usually clear clinical picture Adults: consider KS even without skin or oral lesions if no response to EPTB therapy within 4 weeks.

Confirm with histology whenever possible.

Children: Look for firm, bulging, enlarged lymph nodes, woody oedema (hard, firm swelling) in the inguinal area / legs; darkened skin or mouth/palate lesions +/- subcutaneous nodules. Some children may have periorbital oedema and sub-conjunctival haemorrhage.

Treatment

All patients: ART, analgesia, symptomatic treatment Child: Refer to oncology KS treatment in children depends on severity of presentation; consult with paediatric specialist.

Adult: Delayed chemotherapy For KS stage T0 (only skin KS without oedema). Start chemotherapy only if no improvement after 3 months on ART or if worsening of KS after ART initiation.

Note: If CD4 count result is not available, stage using T & S only to minimise treatment delays Adult: Immediate chemotherapy For KS stage T1 if: adult KS in mouth (including hard palate) OR internal organs involvement (esp.

lungs or GI tract) OR nodular skin KS OR oedema Note: Ensure strictly IV injection as infiltration causes burns. Document therapy, disease extent, and response in health passport; examine for recurrence at every visit.

Recommended regimen for adults Paclitaxel IV Monitor FBC and LFT at baseline and before every paclitaxel infusion. Transfuse before paclitaxel dose if Hb <8 g/dl. Delay chemotherapy if absolute neutrophil count is below 500.

Monitor clinically for neuropathy and hepatitis.

Give chlorphenamine (Piriton) tablets 4mg + paracetamol 1gr + dexamethasone 12mg IV/PO + cimetidine 400mg or ranitidine 50mg IV/PO 30-60 min before paclitaxel. Monitor for allergy / anaphylaxis (rare).

Paclitaxel vials must be stored in fridge.

Wear gloves, gown, and N95 mask when preparing.

Paclitaxel dosing and administration Dose is based on body surface area m2 (BSA). Read BSA from Figure 8 on page 133 or use online calculator. Round the dose to the nearest 5mg.

Dilute in 500ml normal saline solution, slow IV infusion (1-3 hours).

HIV-related diseases 31 Paclitaxel regimen 100mg/m2 BSA every 2 weeks.

Give 6 cycles. Stop if severe side-effects.

Refer to oncology if no improvement or severe side effects.

Pharmacological

Bleomycin Adult: 15 IU/m2 IV every 2 weeks for 6 cycles Child: 0.5 mg/kg IM every 2 weeks for 6 cycles + Vincristine IV Adult: 2mg IV every 2 weeks for 6 cycles Child: 0.05 mg/kg IV (max 2mg) every 2 weeks for 6 cycles Review after every cycle:

Severe neuropathy / constipation: stop vincristine Sign for lung fibrosis (incl. cough, shortness of breath): stop Bleomycin. Cumulative max. lifetime dose for Bleomycin is 400 units Poor response: Refer for secondary management.

Secondary management Oncology department Doxorubicin or other drugs may be used according to oncology protocols.