A 40-year-old man with recurrent fainting, hypotension, lower limb edema and oliguria with body weight gain and secondary erythrocytosis

Abstract

Dr. Berti de Marinis, Dr. Allemand: In November 2009, a 40-year-old man was urgently hospitalized after orthostatic syncope secondary to severe hypotension. Three days before admission, he had experienced profuse sweating and contracted diuresis after intense effort (a long run to reach a stadium to see a rugby match) followed by the onset of fever, sore throat and a flu-like syndrome associated with palpitations. The past medical history was relevant to hypothyroidism treated with hormone replacement therapy, and previous episodes of abdominal pain due to bilateral kidney stones, treated with lithotripsy. Physical examination on arrival at the emergency department showed pallor, body mass index 32.93, body weight 102 kg, redness of the pharynx, hardening of the calves with no signs of edema. The vital signs were: temperature 37.6 C, blood pressure (BP) 92/54 mmHg, heart rate (HR) 108 beats per min, respiratory rate 16 breaths per min, oxygen saturation by pulse oximetry 100% without oxygen supplementation. Laboratory analyses revealed: hemoglobin (Hb) 227 g/l, hematocrit (Ht) 64.3%, white blood cells (WBC) 18.84 9 10/l, neutrophils (N) 14.71 9 10/l, platelets 289 G/L, serum creatinine 151 lmol/l, blood urea nitrogen (BUN) 10.5 mmol/l, urine specific gravity 1084, and C-reactive protein (CRP) 13.82 mg/l, troponin 0.01 lg/l. Findings on electrocardiography, chest radiography and echocardiography were normal, there were no signs of deep vein thrombosis on compression ultrasound (CUS), and only a modest splenomegaly was found on abdominal ultrasound (diameter 12.5 cm). Given the increase in d-dimer levels (508 ng/l), pulmonary embolism was excluded by multislice computed tomography. The patient was administered 2.5 l of saline, and was admitted to our internal medicine ward with a diagnosis of ‘‘hemoconcentration secondary to dehydration’’. He arrived with sustained hypotension, tachycardia and fainting, prompting the intravenous infusion of more fluids (1,000 ml of saline and 500 ml of colloids), followed by a gradual return to normal blood pressure values and adequate diuresis. Urea, creatinine and urine specific gravity also returned to normal. Further laboratory and instrumental analyses performed on admission to the ward showed normal levels of IgG, IgA, IgM and complement fractions, and a low level of serum protein (49.2 g/l) with a normal albumin concentration, but no measurable IgG k monoclonal gammopathy with elevated b2 microglobulin (2.65 mg/l) and the absence of Bence Jones protein. Any presence of infection, renal insufficiency, nephrotic/nephritic syndrome or hepatic or cardiac insufficiency was ruled out. The hematocrit remained high (about 60%) despite the return to a normal plasma volume. Therapeutic phlebotomy was performed to prevent any complications of hyperviscosity with a return to a normal hematocrit. Erythropoietin assay was normal, and the search for the V617F Jak2 mutation was negative, excluding polycythemia vera. G. Berti de Marinis I. Bertozzi E. Allemand F. Tezza M. L. Randi F. Fabris (&) Department of Medical and Surgical Sciences, Padua University Medical School, via Giustiniani 2, 35128 Padova, Italy e-mail: fabrizio.fabris@unipd.it