The acronym of the POEMS syndrome (also known as Crow-Fukase syndrome), refers to: P, polyneuropathy; O, organomegaly; E, endocrinopathy; M, monoclonal gammopathy; S, skin changes. Such patients suffer polyneuropathy, endocrinological symptoms, skin changes, hypertrichosis, organomegaly and osteosclerotic changes .
The POEMS syndrome, characterized by the combination of the five main features, is a rare multisystemic disorder complicating plasma cell dyscrasia, mainly osteosclerotic myeloma and solitary plasmocytoma [1,2]. This report describes the clinical course of a 45-yr-old male, 66 kg body weight and 182 cm height, with POEMS syndrome in which a regional anaesthetic was successfully utilized for a biopsy of the right femur. To our knowledge, anaesthesia for patients with the POEMS syndrome has not been reported.
Our patient was admitted to the hospital following a 9-months' history of a painful sensorimotor, chronic demyelinating polyneuropathy in association with diffuse wasting, facial lipo-atrophy, melanodermia, hypertrichosis, cutaneous thickening, ascites, and hepatomegaly. He was also hypothyroid. Serum protein immunoelectrophoresis showed two monoclonal gammopathies: IgG kappa (28 g L−1) and IgA lambda (2.65 g L−1). In the operating room 500 mL Ringer's lactate solution was infused intravenously over 15 min and he was monitored continuously by a 5-lead electrocardiogram and pulse oximetry. Arterial pressure was measured non-invasively every 3 min over the right brachial artery.
The patient was placed in the right lateral decubitus position and a 16-gauge Tuohy needle and catheter were inserted successfully at the L3-L4 interspace. Five minutes after a test dose of lidocaine 2 mL 2%, prilocaine 5 mL 2% was administered to establish an epidural block. However, this led to a decrease in blood pressure from 125/60 mmHg to 110/55 mmHg; the patient had no symptoms of nausea or light-headedness. Subsequently prilocaine 3 mL 2% was given without affecting arterial pressure significantly. A total of 10 mL of local anaesthetic was injected over 30 min to achieve a block to the level of T8. We chose prilocaine because of its rapid onset, intermediate duration and low toxicity. The surgical procedure, which lasted 30 min, was uneventful.
In addition to the five primary clinical features, a constellation of other clinical manifestations may be associated with POEMS syndrome such as fever, peripheral oedema, ascites, pleural effusions, polyeythemia or thrombocytosis. Although no accepted set of criteria is available to make this diagnosis, most authors agree that at least three of the features summarized in the acronym need to be fulfilled . Although the aetiology is still unclear, vascular factors  proinflammatory cytokines such as tumour necrosis factor alpha (TNF-alpha) and interleukin 6 (IL-6) , and paraprotein specificity for neuroendocrine tissue  have been suggested to play a causative role.
Our patient had hypothyroidism: severe hypotension, and even cardiac arrest, has been reported after induction of anaesthesia in such patients . This probably results from myocardial depression, which is less responsive to catecholamines than in the case of normal patients. There is extreme sensitivity to anaesthetic agents, narcotics, and analgesics . Because of these risks we preferred epidural anaesthesia for the right upper femur biopsy and used relative small doses of local anaesthetic because of the increased intra-abdominal pressure from the ascites. The advantages of regional anaesthesia include reduction in the neuroendocrine stress response, improved postoperative pulmonary function, reduced thrombotic complications and reduced perioperative cardiac morbidity .
Department of Anesthesiology
Department of Orthopaedics
Department of Anesthesiology; Faculty of Medicine Cerrahpasa; University of Istanbul; Turkey
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