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• W3091995603 abstract "A 56-year-old Afro-Caribbean male presented for a right-sided above-knee amputation 6 years previously. He had been diagnosed with adult T-cell leukaemia lymphoma (ATLL), secondary to human T-cell lymphoma virus (HTLV-1) infection. Sezary syndrome was diagnosed 2 years later. Combined chemotherapy, radiotherapy and phototherapy had ameliorated the progression of the disease. However, possibly as a result of thalidomide chemotherapy, arterial thrombo-embolism of the patient's right lower leg had necessitated a below-knee amputation 6 months prior to his current admission. He was otherwise well. He remained on daclizumab (humanised anti-Tac antibody) and topical emollients only. He exhibited florid erythroderma and widespread dermal lichenification (caused by scratching). The patient refused regional anaesthesia alone, but consented to general anaesthesia augmented by epidural anaesthesia. Intravenous access was complicated by the condition of the patient's skin. A 16G cannula was inserted and sutured into position to avoid the use of adhesive dressings (which would either be ineffective on emolliated skin or would remove the damaged skin upon removal). An arterial saturation probe was attached to the earlobe, due to onychogryphosis of the fingernails. After pre-oxygenation, general anaesthesia was induced with propofol 300 mg and fentanyl, and maintained with isoflurane 0–3% in a 30/70 mixture of oxygen and air. The airway was maintained satisfactorily with a laryngeal mask. An epidural catheter was sited at the level of L1/2; an alcohol swab was used to degrease the surrounding skin before an adhesive dressing was applied to the catheter. Plain 0.25% bupivacaine 15 ml were infused into the epidural space, and an opioid-free epidural infusion was continued postoperatively. The patient was carefully positioned on the operating table, with extra padding applied to pressure areas. Despite active warming, the nasopharyngeal temperature fell from 37.6 °C to 35.3 °C during the 2-h operation. Anaesthesia and recovery were otherwise uneventful. Epidural analgesia was discontinued on day 3 postoperatively, and the patient discharged from hospital on day 12. Sezary syndrome is a rare (incidence of less than 1 : 100 000) chronic, cutaneous form of T-cell lymphoma [1]. It is more prevalent in middle age, in black-skinned people and in males. Clinical manifestations usually involve generalised erythroderma (hence ‘red man syndrome/ l’homme rouge'), lymphadenopathy, hepatosplenomegaly, intense pruritus (with atrophic, lichenified skin due to chronic scratching), alopecia, nail dystrophy and ankle oedema [2]. Pulmonary involvement (50%) is often asymptomatic, but mediastinal/hilar lymphadenopathy, parenchymal nodules and pleural effusions may occur [3]. Patients are prone to localised and systemic infections by skin flora, particularly Staphylococcus aureus. Circulating Sezary cells are present in the majority of patients who exhibit erythroderma [4]. Treatment may involve use of immunosuppressants drugs, notably cyclosporin and steroids. Basal dermal blood flow may be increased fivefold (to 5 l.min−1) in erythroderma, and further increased if the patient is hyperpyrexial [5]. This can result in high output cardiac failure, which may be exacerbated by anaemia, hypercatabolism, hypercapnia, spinal/epidural anaesthesia and use of vasodilators. A hyperdynamic circulation results in quicker inhalational and intravenous induction of anaesthesia, although cardiovascular instability may result from precipitant myocardial depression. Careful fluid replacement is required to match increased evaporative and operative fluid loss. Marked heat loss may also occur, particularly when homeothermic regulation is depressed by anaesthetic agents, necessitating careful core temperature monitoring, and the use of active fluid and patient warmers. Conversely, patients with erythroderma have a reduced capacity for sweating, and anticholinergic drugs may precipitate hyperthermia." @default.
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• W3091995603 date "2002-07-18" @default.
• W3091995603 modified "2023-09-25" @default.
• W3091995603 title "Sezary syndrome" @default.
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• W3091995603 doi "https://doi.org/10.1046/j.1365-2044.2002.02752_23.x" @default.
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