Nicole De Simone

Therapeutic plasma exchange in the management of sepsis and multiple organ dysfunction syndrome: A report of three cases

Sepsis with multi organ dysfunction syndrome (MODS) is the most common cause of death in patients in noncoronary intensive care units. Currently, there are no specific treatments that reduce mortality in patients with sepsis and MODS. We report three patients who received therapeutic plasma exchange (TPE) for sepsis with MODS who completely recovered. The first patient, a 3‐year‐old male presented with Methicillin‐resistant Staphylococcus aureus‐associated respiratory, renal, coagulation, hepatic, and neurologic dysfunction. After 5 TPEs, the patient fully recovered. The second patient was a 36‐year‐old pregnant female who developed MODS at 22 weeks of gestation. She had developed respiratory, hepatic, renal, cardiovascular, neurologic, and coagulation dysfunction following pneumonia and concurrent urinary tract infection resulting in an intrauterine fetal demise. After 8 TPEs, the patient was discharged home with only mild residual hepatic dysfunction. The third patient, a 50‐year‐old female with a history of seizure disorder, was found unresponsive in over 100°F heat and diagnosed with Staphylococcus aureus‐associated MODS. Her respiratory, coagulation, neurologic, renal, and hepatic systems were affected. The patient underwent 6 TPEs after which she had marked improvement. In conclusion, TPE may be an effective adjunct therapy in MODS by possibly removing toxic mediators and replacing deficient factors using donor plasma. J. Clin. Apheresis 29:127–131, 2014.

Diagnosis and management of common acquired bleeding disorders.

Acquired bleeding disorders (ABD) are commonly encountered in both inpatient and outpatient settings. ABD can occur due to consumption, decreased synthesis, or inhibition of coagulation factors and platelets. Clinical presentation may vary, ranging from mild bruising to life-threatening hemorrhage. The location, frequency, severity, and provocation of bleeding provide insight into the cause of ABD. Obtaining a good medical, surgical, family, social, and medication history is a crucial step in determining the underlying etiology. Basic laboratory parameters, such as prothrombin time, partial thromboplastin time, thrombin time, fibrinogen, platelet count, and D-dimer levels, aid in further elucidating the reason for bleeding. Optimal management depends on accurate interpretation of the history and laboratory values. Treatment options include administration of vitamin K; blood component transfusion, consisting of plasma, cryoprecipitate, and/or platelets; and blood derivatives, including single and multiple factor concentrates. These products should be used judiciously, due to potential infectious and noninfectious complications, including transfusion-related acute lung injury and transfusion-associated circulatory overload. This article discusses the management of the more common causes of ABD.

Ischemic stroke in a patient with moderate to severe inherited factor VII deficiency

Thrombosis is known to occur in patients with rare inherited bleeding disorders, usually in the presence of a thrombotic risk factor such as surgery and/or factor replacement therapy, but sometimes spontaneously. We present the case of a 72-year-old African American male diagnosed with congenital factor VII (FVII) deficiency after presenting with ischemic stroke, presumably embolic, in the setting of atherosclerotic carotid artery stenosis. The patient had an international normalized ratio (INR) of 2.0 at presentation, with FVII activity of 6% and normal Extem clotting time in rotational thromboelastometry. He was treated with aspirin (325 mg daily) and clopidogrel (75 mg daily) with no additional bleeding or thrombotic complications throughout his admission. This case provides further evidence that moderate to severe FVII deficiency does not protect against thrombosis.

A tale of two ports: an in vitro comparison of flow characteristics for therapeutic plasma exchange: IN VITRO PORT COMPARISON

Tunneled central venous catheters with ports are increasingly used for therapeutic apheresis procedures. Vortex ports have been used as access for therapeutic apheresis procedures, but are not ideal for therapeutic plasma exchange (TPE) procedures due to lower flow rates. We performed an in vitro experiment to compare flow characteristics of the single‐lumen Vortex port (AngioDynamics) with the single‐lumen TidalPort (Norfolk Medical). We used expired red blood cell units and adjusted the hematocrit to 40% with normal saline in a 2‐L bag. We programmed the Spectra Optia (Terumo BCT) to run a 1.0‐volume TPE with 5% albumin as replacement fluid. The TidalPort achieved flow rates of up to 110 mL/min without triggering alarms. Due to crucial alarms, the Vortex Port was not able to run at a flow rate higher than 90 mL/min, and multiple caution alarms were triggered at flow rates of 80 to 90 mL/min. These findings suggest that the TidalPort may be a suitable access option that provides flow rates similar to peripheral or central venous catheters for TPE procedures.

An Audit of Thrombophilia Testing in Patients with Ischemic Stroke or Transient Ischemic Attack: The Futility of Testing

OBJECTIVES Many patients admitted with an ischemic stroke or transient ischemic attack (TIA) undergo thrombophilia testing. There is limited evidence to support this practice. We examined the effect of thrombophilia testing on management of patients admitted with an ischemic stroke or TIA. MATERIALS AND METHODS In this retrospective observational single-center study, we identified patients who were admitted with stroke or TIA and underwent thrombophilia testing over a 45-month period. We reviewed their electronic medical records to assess whether testing affected clinical management, defined as anticoagulation treatment by the time of discharge due to a positive test result. Secondary endpoints included potential misdiagnosis due to false positive results and cost of testing. RESULTS Testing was performed in 143 patients with a stroke or TIA. Forty-four patients (31%) had at least 1 positive test result. The most common positive tests were an elevated factor VIII activity (18% of patients tested) and decreased protein S activity (11% of patients tested). Both of these tests are subject to acute phase effects. Testing altered clinical management in only 1 patient (1% of total patients tested). Thirty-three patients (75%) have the potential for carrying a misdiagnosis due to a positive test that was never repeated for confirmation or repeated too soon after the initial positive test. The annual cost of testing was approximately

Mathematical calculation of lifespan of transfused RBCs in sickle cell disease patients

62,000. CONCLUSIONS Thrombophilia testing in the acute inpatient setting rarely impacted the clinical management of patients admitted with a stroke or TIA. By avoiding thrombophilia testing, both the potential for misdiagnosis and health care costs can be reduced. Therefore, we have discontinued thrombophilia testing in in-patients with a diagnosis of stroke.

Blind and Confused

BACKGROUND Transfusion of donor red blood cells (RBCs) remains an important part of management of sickle cell disease (SCD). However, the survival characteristics of transfused donor RBCs in SCD patients have not been well studied. We sought to calculate survival kinetics of transfused RBCs in SCD patients since it is unclear whether transfused RBCs get destroyed at faster rate as innocent bystander or persist longer due to decreased destruction capacity such as functional splenectomy. STUDY DESIGN and methods Forty-one SCD patients who had undergone at least 3 RBC exchange procedures were inlcuded. Interval between the procedures, both pre-procedure and post procedure hematocrits, HbA% and HbS% were collected. We developed a mathematical model to calculate RBC lifespan for donor RBCs. RESULTS Donor RBCs exhibited average lifespan of about 120days (121.1±13.9 days), which was similar to reported survival of RBCs in normal recipients. However, significant variation between patients were observed with lifespan ranging from 75.6-148.5 days. Intrapersonal variations were small in most cases. CONCLUSION The calculated survival of donor RBCs in SCD recipient, based on certain laboratory values, appears to be similar to that of normal recipient. However, inter-personal variations were large, suggesting different RBC kinetics in a subset of patients, which calls for further research to better understand underlying pathophysiology. This knowledge of RBC survival would be very helpful in individualized management of patients on chronic RBCx.

Extracorporeal Photopheresis Is Effective in Treating Graft-Versus-Host Disease: A Retrospective Analysis

A 62-year-old man developed confusion and was diagnosed as having encephalitis. The etiology was not identified. He continued to have cognitive impairment but remained clinically stable. Five months later, he woke with bilateral vision loss. On neurological examination, he had no light perception bilaterally. The remainder of the neurological examination results were normal. Magnetic resonance imaging of the brain revealed multiple brain lesions. He was treated with steroids and plasmapheresis, with mild improvement in vision. He was then transferred to a long-term care facility, where he developed increasing confusion and ultimately died. An autopsy was performed; the differential diagnosis, neuropathology, and final diagnosis are discussed here.