required 10 units of platelets and 4 units of FFP. Subsequently, Factor IX and X levels were found to be 70% of normal (Mizutani & Ward 1990). Routine coagulation studies are unreliable predictors of the likelihood of bleeding. Of 41 patients with amyloid who had bled, only 20% had an abnormal prothrombin time. Bleeding can follow mild trauma and may require surgical intervention.The gastrointestinal tract was the source in 18, a further eight bled after diagnostic procedures, and three had haematuria. Coagulation tests were normal in all eight who bled after diagnostic procedures (Yood et al 1983).An additional factor contributing to bleeding is amyloid infiltration of the blood vessels (Chow et al 1993).

6. In the reactive form, the additional problems are those of the primary associated disease, eg rheumatoid arthritis.

7. The larynx is the most common site for localised amyloidosis. Hoarseness was the commonest symptom and the false vocal cords were the most likely site to be affected (Lewis et al 1992). Laryngofissure and temporary tracheostomy were required in a 9-year-old child, in spite of which recurrence occurred 18 months later (O'Halloran & Lusk 1994). Urgent tracheal intubation, followed by tracheostomy, was needed for laser resection of subglottic amyloidosis (Woo et al 1990).A fatality occurred in a patient with undiagnosed localised laryngeal amyloidosis.The 38-year-old man had a 3-week history of hoarseness and coughing. Massive haemorrhage and suffocation took place in the middle of the night.At autopsy, a typical amyloid ulcer was found in the subglottis, and the airway was filled with blood (Chow et al 1993). Tracheal involvement can occur concurrently. In a patient with primary amyloid, massive cervical lymphadenopathy resulted in severe airway obstruction (Leach et al 1999).


1. The diagnosis should be suspected in the patient with multiple organ involvement, especially in the presence of a disease known to be associated with the AA form.

2. Careful assessment of the heart, lungs, and liver for impairment of function. Surgical decisions, and anaesthetic management, should be in the light of this information. Cardiac involvement should be suspected in all patients with heart failure who have chamber wall and valve leaflet thickening on echocardiography, normal chamber size, and low ECG voltages (Dubrey et al 1998). Such patients have a poor prognosis. Measurement of QT dispersion has been suggested as a method to predict patients in whom the likelihood of arrhythmias is high (Kirvela et al 1994). From a 12-lead ECG, the QTc should be calculated manually from the onset of the QRS to the end of the T wave, defined as a return to the TP baseline. On three consecutive cycles, the mean QTc is calculated, and the QT dispersion is measured as the difference between the maximum and minimum QTc. An increase in the QT dispersion reflects variations in ventricular recovery of excitability, and therefore an increased risk of arrhythmias. If bradycardias develop, atropine may be ineffective, and isoprenaline and pacing may be required (Tallgren et al 1995).

3. If severe diastolic dysfunction is present, atrial thrombi should be sought on echocardiography and the appropriate prophylaxis given.

4. In the presence of macroglossia, difficulties in intubation may be experienced. Postoperatively, the patient should be nursed in an intensive recovery area.Treatment of laryngeal amyloid should be as for any upper airway lesion causing obstructive symptoms.

5. Coagulation studies should be performed, but even when these are normal the possibility of haemorrhage should be anticipated.A normal result does not guarantee that haemorrhage will not occur.

6. Epidural anaesthesia was given to a patient with secondary amyloidosis undergoing Caesarean section. She had been admitted at 34

weeks' gestation with hypertension and deteriorating renal function in a transplanted kidney (Weir & McLoughlin 1998).


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Dubrey SW, Cha K,Anderson J et al 1998 The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. Quarterly Journal of Medicine 91:141—7.

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Hosenpud JD, DeMarco T, Frazier OH et al 1991 Progression of systemic disease and reduced long term survival in patients with cardiac amyloidosis undergoing heart transplantation. Circulation 84: 338-43.

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Kotani N, Hashimoto H, Muraoka M et al 2000 Fatal perioperative myocardial infarction in four patients with cardiac amyloid.Anesthesiology 92: 873-5.

Leach DB, Hester TO, Farrell HA et al 1999 Primary amyloidosis presenting as massive cervical lymphadenopathy with severe dyspnoea: a case report and review of the literature. Otolaryngology—Head & Neck Surgery 120: 560-4.

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O'Halloran LR,Lusk RP 1994 Amyloidosis of the

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