We present a case of a 69-year-old woman, with a previous history of arterial hypertension and dyslipidemia. Her medication included amlodipine 5 mg, candesartan 8 mg, hydrochlorothiazide 12.5 mg and nebivolol 5 mg. She presented with a 1-year history of left-side supraclavicular swelling associated with progressive fatigue, anorexia, and weight loss (body weight: 74 to 63 kg). Cervical ultrasound documented multiple cervical lymphadenopathies with the largest lymph node (measuring 3.1 × 1.7 cm) being localized on the left supraclavicular region; and she was referred to the Department of General Surgery. A left supraclavicular lymph node excisional biopsy was performed. The histopathological examination of the specimen was consistent with the diagnosis of herpes virus-8-positive CD (HHV-8 CD, ).\nShe was then forwarded to the Department of Internal Medicine upon lymph node biopsy showed CD. Additional clinical history inquiry was attempted and revealed that, during the last few months, she noted progressive diffuse skin darkening and symmetrical paresthesia of the lower limbs. She denied fever, night sweats, gastrointestinal or respiratory complaints. Neurologic review of symptoms was negative for dysphagia, dysarthria, ocular changes, and bowel and bladder dysfunctions. She had no history of smoking, alcohol abuse, or illicit-drug use. She had no contact with animals or any sick person and did not have any recent travel outside Portugal. She had no family history of neurologic or oncologic diseases.\nOn physical examination, she was afebrile. Her blood pressure was 151/77 mm Hg and heart rate 62 beats/min, and peripheral oxygen saturation was 97% while on room air. She had diffuse cutaneous hyperpigmentation and palpable lymph nodes in her cervical and supraclavicular regions, with the largest measuring 3 × 2 cm. Abdominal examination revealed an enlarged liver and no other abnormality. She had bilateral edema of lower limbs. Neurological examination revealed hypoesthesia below the ankles and tendon hyporreflexia; her muscle strength was decreased in upper and lower extremities with muscle strength grade 4-/5 proximal and grade 4+/5 distal.\nBlood tests showed normal complete blood count, coagulation tests, serum bilirubin (total and conjugated), aspartate and alanine aminotransferases and lactate dehydrogenase. Erythrocyte sedimentation rate and C-reactive protein were within normal range. Renal function, serum calcium and total serum protein were normal. Endocrine screening showed normal thyroid function, a normal glycemic profile (fasting plasma glucose and hemoglobin A1c), and a normal cortisol dosage. Hepatitis B and C, toxoplasmosis, tuberculosis, human immunodeficiency virus (HIV), cytomegalovirus (CMV) and Epstein-Barr virus (EBV) infections were ruled out. Auto-immune screening (antinuclear antibodies (ANA), anti-double-stranded deoxyribonucleic acid (dsDNA) and rheumatoid factor) was negative with no complement consumption. Albumin was 3.2 g/dL (reference range (RR): 3.4 - 5) and serum protein electrophoresis (SPEP) with immunofixation demonstrated a monoclonal immunoglobulin G (IgG)/kappa peak with IgG level of 2,120 mg/dL (RR: 650 - 1,600). Serum free light chains ratio was normal. Bence-Jones proteins were not detected. Bone marrow biopsy showed the presence of reactive-appearing lymphocytes B and T and 5% of plasma cells ().\nPlasma vascular endothelial growth factor (VEGF) measurement was elevated (644 pg/mL, RR: < 115). Human herpes virus-8 (HHV8) was not detected in peripheral blood by polymerase chain reaction.\nThoraco-abdominopelvic computed tomography scan revealed small bilateral pleural effusion, multiple enlarged supra and infra-diaphragmatic lymphadenopathies, hepatosplenomegaly and multiple osteosclerotic lesions (). On transthoracic echocardiography there was a normal systolic function without pulmonary hypertension or pericardial effusion. Electromyography was consistent with sensorimotor peripheral polyneuropathy.\nThus, our patient meets the criteria for the diagnosis of POEMS syndrome in association with CD.\nShe was referred to the Hematology Department, and she started treatment with a combination of bortezomib, cyclophosphamide, and dexamethasone.\nAfter the first cycle, she noticed improvement of paresthesia and IgG level reduced from 2,120 to 960 mg/dL (RR: 650 - 1,600). She has tolerated treatment well without any clinically significant adverse effect.
A 61-year-old male patient was admitted to our hospital with a prior one-year history of cutaneous hyperpigmentation. Loss of appetite, abdominal distension, constipation, dry skin, less sweat, and insomnia were concomitant symptoms. Ten months prior to admission, his symptoms became severe and were accompanied by symmetrical pitting edema, lower extremity numbness, and weakness in the left lower limbs. Brain MRI showed cerebral infarction, and the patient was treated appropriately. One month later, he was diagnosed with hypothyroidism and Addison’s disease (AD) for severe edema of the lower extremities, unexplained cutaneous pigmentation, and higher ACTH levels (Tables and ). Hydrocortisone 20 mg and Levothyroxine 12.5 μg per day as well as diuretic therapy were administered, and the symptoms mildly improved. After discharge from the hospital, he gradually stopped the diuretic drugs and the doses were adjusted to hydrocortisone 40 mg and Levothyroxine 200 μg per day based on the lab tests. Concomitantly, he experienced pain and numbness in his lower limbs. Since the onset of illness, his general condition was poor. The patient suffered from decreased appetite, poor sleep, weight loss of 15 kg, and hyposthenia (Fig. ).\nThe patient’s past history showed he was a carrier of hepatitis B virus for 60 years, psoriasis for 40 years with external steroid use, had a 10-year history of type 2 diabetes mellitus controlled by insulin glargine and voglibose, and had hypertension for 2 months. In addition, he was diagnosed with depression two months prior and was treated with flupentixol and melitracen tablets without obvious improvement.\nExamination showed T 36.1°C, P 75 bpm, R 16 tpm, BP 140/85 mmHg, H 176 cm, W 62 kg, and BMI 20 kg/m2. The patient had diffuse cutaneous pigmentation of his skin and mucous membranes, especially the areolas and armpits. His superficial lymph nodes were not palpable. He had edema of the eyelids and pitting edema of the upper and lower extremities (Figs. , , , , ). Neurological examination showed grade IV muscle strength in his left lower limbs. His physiological reflexes were normal, and his pathological reflexes were not elicited. His thyroid function and adrenal function were abnormal (Tables and ).\nMild anemia was noted, with HBsAg(+), HBsAb(−), HBeAg(−), HBeAb(+), and HBcAb(+). Rheumatoid factor, anti-nuclear factor, and anti-HIV were negative. Serum electrolytes revealed normal sodium levels. BNP was 2010 ng/L (450–900), and TNI < 0.012 ng/ml (0–0.12). Albumin was 34 g/L (35–55), and fasting blood glucose (FBG) was 5.3–6.5 mmol/L (3.6–5.8). Electroneuromyography disclosed polyneuropathy of the upper and lower extremities. The results of an adrenal CT scan and a head MRI were negative. Abdomen B-ultrasound presented splenomegaly (4.8 × 13.8 cm) and ascites. UCG indicated pericardial effusion with 59% EF. Bone marrow aspirate showed medullary phagocytosis. No M protein was found via serous protein electrophoresis but an immunofixation examination revealed a monoclonal IgAλ peak. The diagnosis POEMS syndrome was made.\nThe patient was initially treated with a very expensive drug, Revlimid (lenalidomide capsules 25 mg), which is generally used for multiple myeloma (MM) patients. He then underwent autologous stem cell transplantation (ASCT) and experienced significant improvement of his hyperpigmentation and weight loss(Fig. ). Hydrocortisone and Levothyroxine were gradually decreased. His diabetes was also relieved.