An 88-year-old man was admitted to our facility with altered mental status, hypotension (blood pressure range: 71-84/47-57 mmHg in the right arm supine position), fever (104oF), and tachycardia (heart rate: 140-150 beats/min) on arrival at the emergency room. Prior to admission, he was on hemodialysis for the past three months for end-stage renal disease secondary to rapidly progressive glomerulonephritis (has a right permacath). He was receiving intermittent heparin flushes along with dialysis to maintain the patency of the extracorporeal circuit. Other significant past medical history included a splenectomy in 2007. Clinical manifestations, imaging tests, and blood cultures suggested septic shock secondary to Streptococcal pneumonia. The patient was started on meropenem and vancomycin. A left internal jugular catheter and arterial line (in the right upper extremity) were placed for fluid resuscitation and blood pressure monitoring, respectively, and the patient was managed per surviving sepsis guidelines. On day three of hospitalization, the patient started to complain about a right-hand pain at the site of the arterial catheter. The physical examination was remarkable for a swollen and cyanotic right upper extremity, especially the second and third fingers (Figure ), with a barely palpable radial pulse compared to the left side. Arterial Doppler of the upper extremities was obtained, with findings indicative of significant right-sided arterial insufficiency. Further evaluation by venous duplex ultrasound identified a massive thrombus in the axillary, brachial, and basilic veins of the right arm with the solely spared ulna vein being hugely engorged (Figures -).\nWe considered the possibility of catheter-induced venous thrombosis, sepsis-associated disseminated intravascular coagulation and heparin-induced thrombocytopenia as working diagnoses. Given the suspicion of heparin-induced thrombocytopenia, we discontinued heparin immediately while the patient’s peripheral smear and coagulation cascade were investigated. Although the patient had low platelets (Table ), prolonged prothrombin time (16 (normal: 11-13 sec)), activated partial thromboplastin time (63 (normal: 25-35 sec)), elevated fibrin degradation products (>40 (normal: <10 mcg/ml)), which were suspicious for sepsis-related disseminated intravascular coagulation; normal factor VII (77 (normal: 50-150%)), high VIII levels (192 (normal: 50-150%)), normal haptoglobin (163 (normal: 36-195 mg/dl)), mildly decreased hemoglobulin (range: 12.1-12.9 (normal:13.5-17.5 g/dl)), and the presence of very few schistiocytes (<0.5%) on a peripheral smear made disseminated intravascular coagulation less likely. Laboratory data were remarkable for a significant drop in the platelet count from 234*109/L (on initiation of hemodialysis) to 44*109/L (the day of hospital admission) over the past three months. Based on the 4T score, heparin-induced thrombocytopenia was highly suspected and argatroban was initiated at 1 mg/kg/min. Vascular surgery consultation was obtained to address the gangrene in the second and third digits of the right upper extremity. However, considering his critically septic situation with multiple morbidities, a decision was made not to proceed with invasive maneuvers. On hospital day four, heparin-platelet factor 4 (PF4) antibodies (1.67 (normal: <0.4), heparin inhibition: >50%), and serotonin release assays returned positive (using enzyme-linked immunosorbent assay), confirming heparin-induced thrombocytopenia. On hospital day eight, repeated venous duplex demonstrated normal compressibility and spontaneous flow in the vein of the right upper extremity. The platelet count recovered and no further thrombotic complications were observed. Though we were able to manage the patient’s advanced gangrene with argatroban, his second and third fingers were amputated in the end.
A 64-year-old man with a history of obesity (BMI 38 Kg/m^2), arterial hypertension, atrial fibrillation, type 2 diabetes, chronic kidney disease, obstructive sleep apnea, and chronic venous insufficiency presented to the Emergency Department complaining of dyspnea, fever, and chest pain which started 7 days before. The patient was hypoxemic in room air (SpO2 80% and PaO2 43 mmHg). Inferior limbs appeared normal. A molecular swab formalized diagnosis of SARS-CoV2-related pneumonia. Respiratory distress progressed despite non-invasive-ventilation and hemodynamic instability requiring vasoactive support ensued. The patient was intubated and transferred to the ICU. The patient received invasive protective ventilation and hemodynamic support with noradrenaline. Gas exchange progressively worsened to a PaO2/FiO2 ratio of 70 mmHg. He developed a shock, and his lower limbs started to appear mottled. Lung and cardiac ultrasound were compatible with severe interstitial pneumonia and with no signs of pressure overload of the right heart. His coagulation profile was highly altered, as both aPTT and INR were elevated (respectively 2.28 seconds and 6.42), platelets were 164,000/mm3, and D-dimer was 117,521 UI/ml. Coagulation parameters continually worsened (), and treatment with one unit of fresh frozen plasma and vitamin K was required. Inferior limbs’ color progressively worsened, becoming frankly cyanotic, while pulses were still normal. Doppler ultrasound showed bilateral thrombosis of tibial and small saphenous veins. A continuous infusion of iloprost was started, as PCD was suspected (). Continuous infusion of unfractionated heparin was instituted, at a dose of 500-1000 IU per hour, according to aPTT, as well as continuous veno-venous hemodiafiltration as the patient developed acute kidney injury. The patient’s general conditions worsened (), and despite maximal treatment, he passed away after eight days of ICU care.