A 79-year-old female with past medical and surgical histories of stage V chronic kidney disease (CKD), hypertension, heart failure with preserved ejection fraction of 67%, abdominal aortic and right external iliac artery aneurysms status-post endovascular repaired 7/2020 and 11/2020 (EVAR) respectively presented to an academic hospital in United States for management of hypervolemia due to CKD requiring urgent right internal jugular vein (RIJV) catheter placement and hemodialysis. Patient used to be active at baseline, able to perform activities of daily living, but became more sedentary during the past year due to experiencing worsening bilateral lower extremity swelling and exertional dyspnea. Outpatient nephrology has been following patient who was refusing dialysis prior to this admission; however, her worsening symptoms changed her mind.
When patient was initially hospitalized, she was found to have pancytopenia with white blood count (WBC), hemoglobin (Hgb), and platelet count showing 3.0 x 103/uL, 8.9g/ dL (normocytic), and 76 x 103/uL respectively. Nephrology and primary team believed low Hgb and platelet could be explained by anemia of chronic kidney disease and uremic thrombocytopenia. Blood urea nitrogen (BUN) and creatinine (Cr) on admission were 98 and 7.32 respectively. Sepsis was low on the differential since patient did not demonstrate other associated symptoms.
Due to patient’s deteriorating condition, decision was made to urgently place RIJV catheter to begin dialysis, while allowing primary team to begin pancytopenia work-up without delaying critical therapy. Primary team spoke with patient’s outpatient primary care physician who was also a hematologist and learned that at her previous hospitalization one year ago at another institution, there was concern for possible heparin-induced thrombocytopenia but patient was never tested. Subsequently, she was tested and found to have negative heparin-induced thrombocytopenia platelet factor 4 antibody and negative serotonin-release assay on this admission.
Patient was able to tolerate dialysis but had continuous bleeding at the catheter site. Initially, vascular surgery and nephrology believed additional dialysis sessions will improve her platelet count and therefore stop the bleeding. However, she continued to bleed despite receiving more than four dialysis sessions. Vascular surgery was at patient’s bedside daily to change her dressing and suture her site, which did not stop the bleeding. Meanwhile, hematology was consulted regarding her pancytopenia. Possible malignancy work-up was initiated including leukemia, lymphoma, and immune thrombocytopenic purpura (ITP) as well as DICs. Patient was also started on empiric prednisone to cover for possible ITP although it was low on the differential.s
Computed tomography (CT) of abdomen and pelvis found type I endoleak (6.5cm in diameter) at proximal bilateral common iliac arteries (see Image section, Fig. 1). Patient’s coagulation panel was obtained which showed low fibrinogen (94 mg/dL) and d-dimer greater than the upper limit of the test value (> 10,000 ng/mL). Thromboelastogram showed low max amplitude (< 40 mm), low fibrinogen function, low adenosine-5’-diphosphate, low kaolin with heparinase, normal activator F and normal R value, suggesting platelet dysfunction and possible DIC. In addition to having possible dialysis-associated thrombocytopenia, it was theorized that patient may be experiencing chronic DIC secondary to endoleak and required surgical assessment.
Vascular surgery contacted patient’s surgeon at a different institution who performed her endovascular repairs. Immediate outpatient follow-up was recommended once patient was stable to be discharged.
To stabilize patient from blood loss and thrombocytopenia, she was given a total of 2 units of packed red blood cells, 6 units of platelet and 2 rounds of desmopressin during her hospitalization. She was also found to have low fibrinogen and was given 2 units of cryoprecipitate. The combination of anticoagulation used during dialysis, platelets and antifibrinolytic therapies stopped the bleeding. After hemodynamic stabilization, she was discharged after outpatient surgical follow-up and hemodialysis appointments were arranged.