A 78-year-old man diagnosed in January 2012 with SS stage III-B/IV-A1 (T4N0M0) with extensive skin involvement, whose personal history was a smoker of 20 cigarettes a day, type 2 diabetes mellitus, hypertension, atrial fibrillation and benign prostatic hyperplasia.
He was diagnosed with lichen planus in 2010 by the dermatology department, presenting with pruritus that was controlled with corticosteroids and systemic antihistamines, PUVA therapy and acitretin. When he was diagnosed with SS, oral chlorambucil was added to the therapy. After 10 months, in November 2012, loss of response was observed starting 2nd line treatment with bexarotene together with gabapentin 300 mg daily with the aim of improving pruritus control.
Skin progression after 2 months of treatment, as well as insomnia and depression, associated with uncontrollable pruritus, led to start with a 3rd line of treatment, administering polychemotherapy according to the CVP scheme (cyclophosphamide, vincristine and prednisone), without doxorubicin due to the comorbidities he presented. Unfortunately, after two cycles of treatment, the patient continued to show no response and no improvement in pruritus. The next available option, extracorporeal photopheresis, was rejected by the reference centre, and in this situation it was decided to administer aprepitant as antipruritic therapy, a use outside the authorised indications.
The Pharmacy Department, together with the Haematology Department, reviewed the literature and compared the doses used in the different published case series, starting with a dose of 80 mg daily for 10 days and continuing with 80 mg every 48 hours. Since this was an off-label indication, the patient was asked for informed consent.
Each pack of Emend® (aprepitant) contains one 125 mg and two 80 mg doses. Since the dose to be used in this case was 80 mg, in order to make use of the 125 mg capsule, it was decided to prepare a suspension of aprepitant. To this end, the contents of the aprepitant4 capsules (125 mg +80 mg +80 mg +80 mg) were extracted and 7 mL of Ora-Plus® vehicle and another 7 mL of Ora-Sweet® were added, resulting in a 20 mg/mL suspension, which was repacked into 4 mL oral syringes (80 mg). The suspension was given a stability of 90 days, stored in a refrigerator. The patient was given both oral and written information about the days on which to take the treatment, the need for refrigeration and the most common adverse effects.
Fifteen days after starting treatment with aprepitant, the patient reported significant improvement in pruritus. So far, pruritus had always been assessed subjectively. It was decided to assess the patient's pruritus using the visual analogue scale (VAS) (0 = no pruritus and 10 = worst imaginable pruritus), and the patient was given a VAS of 5. Given the effectiveness of the treatment and the absence of adverse effects, it was decided to continue with the regimen of 80 mg every 48 hours.
After one week, the patient was admitted for vesicular lesions and pain in the right hemithorax. During admission, pruritus was well controlled. Gabapentin was also discontinued due to treatment-associated tremors and a 4th line treatment for CTCL (pegylated liposomal doxorubicin) was administered. When the patient was discharged from the hospital, he did not take the aprepitant syringes with him by mistake and was off treatment for approximately 1 week. When he returned to the clinic, he reported a worsening of itching, so he restarted treatment with aprepitant. After 3 cycles of chemotherapy, it was decided to discontinue doxorubicin given the progression at cutaneous level. As a last option, weekly gemcitabine was administered, but had to be withdrawn due to poor tolerance. Finally, with a VAS of 9, it was decided to discontinue all chemotherapy and to maintain the best supportive therapy, continuing treatment with aprepitant until the end of the disease.