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.
The patient is a 78-year-old woman who presented to emergency department with 2 days of gradually worsening right lower extremity (RLE) swelling, pain, and paresthesia that was limiting mobility. She was independent with a clinical frailty score of 2. Her past medical history was significant for diabetes mellitus with Hgb a1c 10.5 and a provoked left lower extremity DVT 30 years earlier treated with a course of warfarin and permanent IVC filter placement. She denied prior hypercoagulability workup and reported that her first episode of thrombosis followed a prolonged airplane ride. She was not anticoagulated at the time of presentation. She denied a history of dysrhythmias, prior embolic events, recent travel, injury, surgery, illness, or known exposure to COVID-19. She denied a family history of blood clots or hypercoagulability.\nPhysical examination revealed an RLE that was severely taut with cyanosis with nonpalpable peripheral pulses and multiphasic signaling on Doppler and tenderness to the mid-thigh. The contralateral limb was soft, nontender with color appropriate of ethnicity and palpable peripheral pulses. An RLE venous duplex ultrasound scan was performed with noncompressible veins and loss of spontaneous flow from the right popliteal vein extending to the right common femoral vein consistent with DVT. On presentation, she did not display hemodynamic instability nor complained of cardiopulmonary symptoms.\nBecause of progressive symptoms concerning for PCD and imminent development of venous gangrene in a patient with good functional status, she was admitted directly to the angiography suite for CDT and pharmacomechanical thrombectomy with tissue plasminogen activator (tPA) and heparin. Angiogram revealed thrombus extending proximal to the IVC filter in the mid-IVC (). She was transferred to the intensive care unit for continued CDT with tPA (1 mg/h) and heparin (500 u/h) through a Uni∗Fuse catheter. tPA was discontinued overnight for a fibrinogen of 82 mg/dL. The next morning, she reported subjective improvement of symptoms and return of palpable peripheral pulses with resolution of pain. She returned to the angiography suite for catheter removal and completion venogram that revealed persistent thrombus burden, and the decision to remove the remainder of the clot was made to decrease the risk of recurrence. Using the Penumbra Indigo CAT8 system (Penumbra, Alameda, Calif), mechanical suction thrombectomy, and balloon venoplasty were performed with successful recanalization (). She was transferred to the floor, heparin was discontinued, and rivaroxaban initiated.\nOn postoperative day 2, she was noted to have expressive aphasia and left-sided hemiplegia. A noncontrast computed tomography and computed tomography angiogram of the head and neck were performed without evidence of a hemorrhage or significant carotid or aortic arch atherosclerotic disease. International normalized ratio was therapeutic at 2.0. Given the concern for stroke, a diffusion-weighted magnetic resonance imaging of the brain was obtained () and was notable for the focal area of restricted diffusion within the right thalamus. Because of recent anticoagulation and thrombolysis, she was not a candidate for systemic tPA. Heparin was not reinitiated because of therapeutic international normalized ratio on rivaroxaban. She was transferred to a tertiary center with neurological and neurosurgical capabilities where she underwent a transesophageal echocardiogram with bubble study that revealed a PFO with an atrial septal aneurysm and left-to-right flow noted with color Doppler and a moderate right-to-left shunt during bubble study but no left atrial mass or thrombus. Rivaroxaban was continued and her neurological status improved. She was discharged to a rehabilitation facility with improved left-sided weakness on postoperative day 3. After 2 weeks of rehabilitation, the patient was discharged with a modified Rankin score of 1. Patency of the RLE veins was unknown at the time of discharge, but a repeat duplex ultrasound scan at 8 months after initial presentation showed chronic thrombus in the right common femoral vein, but the RLE venous system remained patent.\nBecause of multiple episodes of thrombosis, she was placed on lifelong anticoagulation with apixaban. Hypercoagulable disorder was suspected but was deferred by her hematologist as diagnosis would not change plan for management.