The patient arrived at our emergency department (ED) for an acute abdominal pain with caudal extension reaching both lower limbs, associated with dysentery occurred during the two previous days with a progressive worsening trend.
He was hospitalized in the cardiac rehabilitation centre where he was admitted two weeks before to recover from heart failure exacerbation. During hospitalization, he was treated with high doses of diuretics and water restriction to reduce preload and improve heart performance.
The patient had a history of diabetes mellitus not requiring insulin, chronic obstructive pulmonary disease on long-term low flow oxygen, and a recent laboratory diagnosis of active PM (reported leukocytosis > 30,000 / uL with a gene activating mutation of the thrombopoietin receptor MPL gene) yet untreated (no palliation or cytoreduction).
He had first of all gone to his local rural ED where he underwent an abdominal computerized tomography (CT) scan with contrast enhancement, which revealed extensive thrombosis spreading to the superior mesenteric artery, and to both renal and common iliac arteries. Owing to the severe extensive state of the thrombosis, he was transferred to our central ED with a request for a vascular surgical and haematological consultation.
At first glance, his clinical appearance was poor. The patient arrived in a diaphoretic, hypotensive, and oliguric (e.g. diuresis of less than 20ml/hour) state. Limbs were cold, hypotrophic, and pale with a long nail bed capillary refill time and weak peripheral pulses.
Despite marked abdominal pain, his abdomen was tractable with valid peristalsis. Thoracic evaluation revealed predominant respiratory crackles accompanied by dyspnoea and a mild decrease in SpO2 (93% SpO2 when breathing air). Blood gas analysis showed mild hypoxaemia (58 mmHg pO2; 0.21 FiO2) with a severe, mixed pCO2 (48 mmHg; pH 7.28; HCO3- 12 mEq/L).
Laboratory tests revealed leukocytosis (73,780 cells/uL), volume depletion with haemoconcentration (59.2% haematocrit, 6,860,000 erythrocyte cells/uL, Hb 18.4 g/dL), a tendency towards thrombocytosis (platelet count = 464,000 cells/uL), acute renal damage (2.48 mg creatinine/dl), no significant biliary or hepatocellular alteration (normal transaminases, alkaline phosphatase and gamma-glutamyl transferase values), and high serum osmolality (313mOsm/kg). (Table 1)
The ultrasound scan showed complete collapse of the inferior vena cava. He was admitted with a diagnosis of acute abdominal pain, severe dehydration, and acute kidney injury.
Strict clinical monitoring (vital signs and urine output) was implemented with telemetry equipment, along with the best possible supportive therapy while awaiting general stabilization. Pain was relieved using opiates (i.e. intravenous morphine). The marked hypotensive state, favoured by the ongoing dysentery, contributory cause towards the relapse in the haematological disorder, was countered by increasing fluids. Suitable hydration was implemented with saline infusion with the aim not only of ensuring adequate blood circulation (together with bicarbonate infusion and oxygen supplementation) but also of decreasing blood viscosity. The patient’s general thrombotic state prevented any surgical approach. Unfractionated heparin (UFH) was therefore administered to maintain haemodynamic stability and delay, without halting, the unrelenting threatening advancement of DlC triggered by aggressive MPD. Despite prompt therapeutic support, the overt establishment of DlC, secondary to the Hyperviscosity Syndrome (HVS) caused by MPD and dehydration, went beyond clinical reversibility and led to a multiple organ dysfunction syndrome (MODS), with secondary death from septic shock the next morning.