A 69-year-old woman who had been experiencing generalized weakness and leg swelling for a year presented to the clinic. Over the past 10 months she had developed a distended abdomen and her hands and feet were numb and cold. She also reported that her mouth and eyes felt dry at times. She had lost 5 kg in weight over the past 12 months but had not experienced any fever or night sweats. Her medical history included hypertension and diabetes, but these were well controlled. Family history showed that her husband had been treated for tuberculosis (TB) 33 years ago.
Six months previously, she had visited the clinic of a local hospital and underwent an ultrasonographic examination that showed ascites. She underwent paracentesis and was prescribed diuretics but had a poor response. Two months later, an abdominal computed tomography (CT) scan showed multiple lymph node swellings with high calcified shadows, and a CT-guided peritoneal lymph node biopsy showed lymphatic tissue and granuloma formations. At that time, a diagnosis of TB was made. The patient was prescribed triple anti-tuberculotic treatment (i.e., isoniazid 300 mg qd, ethambutol 0.75 g qd, and rifapentine 0.6 g bi-weekly). One month later, because of persistent ascites, 1 g  pyrazinamide qd and 10 mg prednisolone qd were added to her treatment regimen, but her response was poor.
At the current clinic visit, physical examination showed normal vital signs, but she had several 0.2-0.4-cm red nodules on her forehead, chest, and abdominal wall (Figure 1). The patient also had perioral hyperpigmentation (Figure 2) and enlarged lymph nodes in the axillary areas, but her heart and lung examinations were unremarkable. Abdominal examination showed a distended abdomen, slight epigastric tenderness, and shifting dullness, with no rebound tenderness. Bowel sounds were heard four times/minute, and there was pitting oedema in both legs. Neurological examination showed slight weakness, with a muscle strength of 4/5 in her four extremities. The patient's laboratory, biopsy, and image results are shown in Table 1. The patient also exhibited the following: signs of nerve damage; slowed nervous transmission; IgG lambda type M protein positivity; numerous sclerotic bones with increased densities (Figure 3); VEGF level of 1595 pg/ml; multiple lymph node swellings. An axillary lymph node biopsy showed clustered plasma cells (Figure 4). Elevated cortisol levels, subclinical hypothyroidism, hyperpigmentation, paracentesis, and pitting oedema in both of legs were also noted.
A diagnosis of POEMS syndrome was made because the patient had two mandatory criteria (i.e., peripheral neuropathy and monoclonal plasma cell disease), three major criteria (i.e., sclerotic bone lesions, an elevated VEGF level, and lymph node hyperplasia disease), and three minor criteria (i.e., endocrine abnormalities, skin hyperpigmentation, and ascites and leg edema). After determining the diagnosis, we initiated lenalidomide-based chemotherapy (i.e., lenalidomide 25 mg qd for three weeks and dexamethasone 40 mg weekly for four weeks). The patient was also prescribed diuretics for her oedema and 25 microg levothyroxine od for subclinical hypothyroidism. Three months after the two courses of treatment, the patient's ascites had gradually reduced and her body weakness had improved significantly, although she still experienced slight numbness in her hands and feet. At her most recent follow-up examination, her condition was stable.
This case study was approved by Medical Ethics Committee of the Aerospace Center Hospital in Beijing, China and signed informed consent was obtained from the patient for publishing her anonymised data. The reporting of this study conforms to CARE guidelines.