A 45-year-old man, Caucasian, was referred to an urgent internal medicine clinic in 2014 for assessment of recent onset peripheral edema and hematologic abnormalities. His medical history was notable for alcoholism (with a prior episode of pancreatitis), untreated chronic hepatitis C infection, depression, and vitamin B12 deficiency. Neuropathy, described as the sensation of “pins and needles”, was reported 1 year before presentation.\nNerve conduction studies in patients with POEMS syndrome show slowing of nerve conduction that is more predominant in the intermediate than distal nerve segment. The sensation in the knee and distal weakness were reduced. He presented normal cardiac, liver and renal function. He could not use a prosthesis due to edema of the leg. The skeletal survey was normal and the computed tomography for the chest, abdomen and pelvis showed splenomegaly, borderline lymphadenopathy.\nAn episode of necrotizing fasciitis leading to right below elbow amputation and left Symes amputation was also performed. He was living on the street and in shelters. His left foot prosthesis had broken several months previously. When he was assessed for a replacement prosthesis, he was noted to have peripheral edema that prevented prosthesis fitting. Routine laboratory investigations showed hypogonadism, polycythemia, thrombocytosis, and neutrophilia. In the internal medicine clinic, the patient complained of paraesthesias, subjective sensory loss, and a tingling, burning pain in his distal extremities. Moderate peripheral edema and hepatosplenomegaly were found on examination. Abdominal ultrasound showed hepatosplenomegaly with the spleen measuring 13.4 cm. The patient did not have a biopsy or a Fibroscan to determine his fibrosis status. Bilirubin levels were in the normal range for the entire period of time. Alanine aminotransferase (ALT) fluctuated 26–30 IU, and aspartate aminotransferase (AST) fluctuated 36–39 IU. Hepatitis C viral load was 2.1 × 106.\nAn echocardiogram revealed normal biventricular function. The skeletal survey was normal.\nLaboratory investigations were remarkable for hemoglobin 190g/L, platelets 687 × 109/L, neutrophils 37.4 × 109/L, total testosterone 2.2 nmol/L (normal range, 5.5–25.2), and prolactin 24.3 mcg/L (<15). Blood film examination showed normal morphology. Electrolytes, creatinine, alanine and aspartate aminotransferases, bilirubin, C-reactive protein, and urinalysis were normal. Serum protein electrophoresis demonstrated polyclonal gammopathy. Analysis of peripheral blood for JAK2 mutation and BCR-ABL fusion product was negative. The immunofixation-revealed <1 gm/L amount of lambda light chain.\nPOEMS syndrome was considered despite the normal results of the serum protein electrophoresis. Computed tomography of the chest, abdomen, and pelvis confirmed hepatosplenomegaly and showed prominent retroperitoneal lymph nodes. Electromyography showed a demyelinating polyneuropathy. Lumbar puncture was performed; albumin-cytologic dissociation was seen on analysis of cerebrospinal fluid. Bone marrow aspiration and biopsy revealed hypercellular marrow with 5% plasma cells.\nSerum protein electrophoresis was repeated, again showing polyclonal gammopathy; the kappa/lambda ratio in peripheral blood was normal. Serum protein electrophoresis was repeated with immunofixation and instructions to look carefully for a low-titer monoclonal protein. An IgA M-protein with lambda light chain was identified, with a titer of less than 1 g/L. A 24-h urine collection for protein electrophoresis was not feasible given the patient’s homelessness and mobility impairment.\nVEGF level was markedly elevated at 2800 pg/mL (normal 30–90). Due to the diagnostic value of VEGF, the patient was referred to the malignant hematology clinic with a new diagnosis of POEMS syndrome. The treatment with dexamethasone and lenalidomide was initiated. Eight months later, his edema and neuropathic symptoms had improved somewhat and his VEGF level had decreased to 1828 pg/mL despite some non-adherence with treatment. At 17 months from diagnosis, his VEGF level had further decreased to 1022 pg/mL, although his edema and symptoms of polyneuropathy did not show further improvement.\nStem cell harvest was performed at 20 months from diagnosis. At 22 months from diagnosis, he was admitted to hospital with bilateral infected leg ulcers. He improved quickly with antibiotics but refused to restart dexamethasone and lenalidomide. His VEGF level fell to 540 pg/mL at 29 months from diagnosis despite a 7-month gap in treatment; however, his peripheral edema had worsened to the point that his left foot prosthesis once again did not fit, and his neuropathic pain had become less responsive to analgesics. At this point, he was not taking his medication. In addition, he also began drinking heavily. This fact led to a remission of POEMS. The levels of VEGF increased to 3480 pg/mL. This fact clearly demonstrates the link between the clinical and laboratory manifestation of POEMS syndrome and alcohol misuse.
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.