A 40-year-old male presented with progressive weakness, tingling and numbness in lower limbs for 2 years. He noted lower limbs swelling and skin changes over the face, hands and feet for 1 year. He also gave a history of erectile dysfunction and loss of libido for past 6 months. There was no history of bone pain or drug abuse. He had no previous history of tuberculosis, diabetes mellitus or hypertension.\nOn physical examination, bilateral pedal edema was present (Fig. a and b). Skin was thickened and hyperpigmented over the face, fingers of the hands and shin (Fig. a–d). Bilateral gynecomastia and testicular atrophy were also present. Abdominal examination showed hepatomegaly and ascites. Higher mental functions were normal. Examination of the fundi revealed bilateral papilledema, but otherwise cranial nerve examination was unremarkable. Muscle power in upper limbs was normal and predominant distal weakness [Medical Research Council (MRC) grade 4/5 at hip joints and 4-/5 at ankle joints] was present in lower limbs. Deep tendon reflexes in the upper limb were diminished (+1) including biceps, triceps and supinator and absent in lower limbs (knee and ankle). All modalities of sensations including pain, touch, temperature, vibration and joint position senses were impaired below knees. Romberg's sign was positive.\nFull blood count, liver and renal function tests, muscle enzymes (creatine phosphokinase), serum ferritin and vitamin B12 level were normal. Serum total protein was 7.1 g/dl, albumin 3 g/dl, globulin 4.1 g/dl and A : G ratio 1 : 1.3. Fasting and postprandial blood sugar levels were normal. Thyroid function tests revealed hypothyroidism (TSH level: 16.62 mIU/l and normal TSH level 0.4–4 mIU/l). Luteinizing hormone and testosterone levels were 15 (1.8–8.6 IU/l) and 111 (300–1000 ng/dl), respectively. Antinuclear antibody, rheumatoid factor, enzyme-linked immunosorbent assay test for human immunodeficiency virus, serology for hepatitis B and C were negative. Abdominal ultrasonography showed hepatomegaly (16 cm), moderate ascites and multiple enlarged mesenteric lymph nodes along the iliac vessels. Fine-needle aspiration cytology of mesenteric lymph node was inconclusive. Ascitic fluid was an exudate (SAAG < 1.1). Ascitic fluid adenosine deaminase level was normal and no malignant cells were observed. Nerve conduction studies showed sensorimotor demyelinating and axonal type polyneuropathy in both upper (median and ulnar nerves) and lower limbs (peroneal, tibial and sural nerves). Cerebrospinal fluid (CSF) analysis was normal. X-ray of the pelvis showed multiple osteosclerotic lesions involving head of left femur and right iliac crest (Fig. ). X-ray of the skull and spine was normal. The serum protein electrophoresis showed gamma globulinemia with no monoclonal M spike. Immunoelectrophoresis using the immunofixation method revealed a monoclonal IgG lambda band. The bone marrow biopsy from right iliac crest showed large atypical plasma cells in the range of 6–8% (Fig. ). The patient was diagnosed with POEMS syndrome. He was given radiotherapy, followed by a combination therapy of melphalan (16 mg/m2) and dexamethasone. At 6 months and 1-year follow-up, the patient has no worsening of symptoms.
Woman, 65-year-old, with sensory-motor polyneuropathy for 15 years, worsening in the\nprevious 6 months. The patient had diffuse hyperpigmentation, dry skin and hemangiomas\n( and ). She reported hypertrichosis, edema of the legs, facial lipoatrophy,\nleukonychia and weight loss ( and ). Analytically, she presented polyglobulia,\nthrombocytosis, hypotestosteronemia, hyperalbuminemia and elevation of IgG in the\nliquor. The immunochemical study emphasized IgG monoclonal gammopathy of lambda\nchains.\nInvestigations showed small adenopathies, cardiomegaly, hepatosplenomegaly, generalized\nedema and extensive lytic lesion of L5. A biopsy of this lesion showed a lytic\nproliferation of plasma cells, CD138 +, К chains - chains λ +. The myelogram showed\nhypercellularity and eosinophilia. The electromyogram showed slowing of motor and\nsensory nerve conduction of several nerves. An excision of the right breast angioma was\nperformed and was compatible with capillary hemangioma. The patient has begun treatment\nwith dexamethasone, radiotherapy, bortezomib and is waiting for a bone marrow\ntransplant.