Therapy plans for therapeutic apheresis in Epic HealthLink.

BACKGROUND: A common required duty of pathology resident physicians while rotating on transfusion medicine is the medical oversight of the therapeutic apheresis service. A task often performed on this clinical medicine service is formulating and writing orders for therapeutic apheresis procedures. The EpicCare tool called the therapy plan provides unique advantages over a standard electronic order set for therapeutic apheresis. MATERIALS AND METHODS: Transfusion medicine physicians, apheresis nurses, pharmacists, and information technology professionals collaborated to create therapy plans for three therapeutic apheresis procedures: plasmapheresis, red cell exchange, and photopheresis. RESULTS: Therapy plans were implemented and have been well-received for several years. Over a six-year time period, a total of 613 therapy plans were created and signed. We speculate that this implementation may have increased both physician efficiency and patient safety. CONCLUSION: This article reports our experience using therapy plans in EpicCare in order to raise awareness of this tool and to serve as an encouragement for wider adoption.

Plasmapheresis for Fulminant Wilson's Disease Improves Mental Status and Coagulopathy.

Wilson's disease is a rare genetic condition that affects copper metabolism, resulting in tissue copper accumulation and resultant organ damage. We report a case of a young woman who presents with Wilson's disease complicated by hemolysis, impaired hepatic function, coagulopathy, and acute kidney injury. She was treated with plasmapheresis as a bridge to a liver transplant. Her mental state, renal function, and bilirubin level improved after starting plasmapheresis. She successfully underwent a liver transplant and remained stable post-liver transplant. We share our experience on the use of plasmapheresis in treating Wilson's disease.

Corticosteroids, Plasmapheresis, Argatroban, Rituximab, and Sirolimus Provided Clinical Benefit for Catastrophic Antiphospholipid Syndrome in a Patient with a History of Heparin-Induced Thrombocytopenia.

We report a patient with catastrophic antiphospholipid syndrome who had significant improvement after corticosteroids, plasmapheresis, argatroban, rituximab, and sirolimus. Argatroban was used instead of heparin due to a history of heparin-induced thrombocytopenia.

Unique Presentation of Bortezomib-Associated Thrombotic Microangiopathy Responsive to Therapeutic Plasma Exchange and Eculizumab Therapy.

Thrombotic microangiopathies (TMA) are a rare group of life-threatening hematological conditions characterized by thrombocytopenia and microangiopathic hemolytic anemia. Although our understanding of the pathophysiology and the availability of diagnostic testing has improved for primary TMAs, such as thrombotic thrombocytopenic purpura, the pathophysiology underlying secondary TMAs, including drug-induced TMAs (DITMAs), remains less clear. In this case report, we present the unique case of a patient with a history of multiple myeloma that presented four months after the initiation of bortezomib therapy with a bortezomib-associated TMA that responded to therapeutic plasma exchange (TPE) with plasma replacement and eculizumab therapy. This case demonstrates the possible utility of TPE with plasma replacement and eculizumab therapy in DITMA patients that fail to respond following a trial of holding the suspected medication.

A Case Study in Process Improvement to Minimize Delays from Obtaining Blood for Red Cell Exchange for a Patient with Sickle Cell Disease and Multiple Red Blood Cell Alloantibodies.

The process of procuring several units of red blood cells for red cell exchange can sometimes take several hours to days, especially for patients with multiple clinically significant red cell alloantibodies. This can introduce delays, inconveniences, and even health challenges for the patient. For most planned exchanges, these delays are preventable with some foresight and process modifications that are relatively minor yet high leverage. We report a case study of process improvement whereby the apheresis nurse sends an e-mail to the blood bank when the nurse makes the patient's next red cell exchange appointment as the signal to order blood about 6-8 weeks before the exchange.

Unexplained Hematocrit Increase after Therapeutic Phlebotomy in a Patient with Marked Erythrocytosis.

We report a patient with hereditary erythrocytosis who underwent a therapeutic phlebotomy and had a post-phlebotomy hematocrit that was higher than the pre-phlebotomy hematocrit. We could not discern a reason for this hematocrit increase after phlebotomy. Instead of performing another phlebotomy, we performed an automated red cell depletion via an apheresis instrument. This procedure is essentially a red cell exchange, but 5% albumin is used as the replacement fluid instead of red blood cells. The patient's hematocrit decreased from 80% to 39% after three consecutive daily red cell depletion procedures. We share our experience to report the unusual finding of a patient's hematocrit that increased with phlebotomy and to raise awareness of the red cell depletion procedure.

Use of Iatrogenic Lipid Emulsion and Subsequent Plasmapheresis for the Treatment of Amitriptyline Overdose.

Plasmapheresis for the treatment of hypertriglyceridemia is relatively uncommon and mostly reported either in patients experiencing hypertriglyceridemia-induced acute pancreatitis or patients with therapy-resistant familial hypercholesterolemia. Standard therapies for hypertriglyceridemia include dietary modification and lipid-lowering medication. For severe hypertriglyceridemia, the risk of pancreatitis increases significantly as triglyceride levels increase above 1000 mg/dL, and current therapies are unable to reduce triglyceride levels rapidly enough. Here, we report a case of a 48-year-old male patient who presented to the emergency department due to an amitriptyline overdose. In addition to being started on IV sodium bicarbonate therapy, an intravenous 20% fat emulsion bolus at 1.5 mL/kg was administered followed by 0.25 mL/kg/min infusion for 4 hours as a strategy to absorb lipophilic amitriptyline. Two days posttreatment, he was noted to have substantial hypertriglyceridemia (serum triglycerides: 6,475 mg/dL). His amylase was within the normal range at 37 U/L (reference range: 20-100 U/L), his lipase was low at 40 U/L (reference range: 75-390 U/L), and he was without evidence of any clinical sequelae secondary to hypertriglyceridemia (e.g., pancreatitis). Due to the severity of his hypertriglyceridemia, plasmapheresis was initiated urgently for rapid reduction in serum triglyceride levels to prevent pancreatitis and end-organ damage. He underwent a 1-plasma volume exchange with 5% albumin as the replacement fluid. This reduced his triglyceride levels to 185 mg/dL (reference range: 3-149 mg/dL). His symptoms secondary to his amitriptyline overdose were also resolved. Here, we report a 2-step process of intravenous lipid emulsion followed by plasmapheresis for amitriptyline overdose.

Successful Treatment of Incomplete Susac Syndrome with Simultaneous Corticosteroids and Plasmapheresis Followed by Rituximab.

We present a case report of a patient with incomplete Susac syndrome. He had cognitive impairment, corpus callosum lesions, and vestibulocochlear dysfunction on brainstem auditory evoked responses. He was treated with methylprednisolone and plasmapheresis, improved, and then, also received rituximab. His improvement has been lasting as of this writing. This case shares our experience with a successful treatment of this rare condition that is incompletely understood and lacks well-established treatment guidelines.

Plasmapheresis, Rituximab, and Ceftriaxone Provided Lasting Improvement for a 27-Year-Old Adult Male with Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS).

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) is a specific autoimmune response to group-A streptococcal (GAS) infections in children and adolescents with a sudden onset of neuropsychiatric disorders including obsessive-compulsive disorder (OCD) or tic-like symptoms. We present a case report of a 27-year-old male patient who had lasting improvement with plasmapheresis, rituximab, and ceftriaxone. Our patient first developed sudden psychosis and confusion after GAS infections at age 17. He had elevated anti-streptolysin O (ASO) titers, negative urine drug screen, no ETOH in blood, normal CBC, normal TSH, normal salicylate, normal acetaminophen, and a normal head CT. The tentative diagnosis of PANDAS was made, and the patient was thereafter treated with antipsychotics, antibiotics, tonsillectomy, and IVIG which resulted in remissions and relapses of his neuropsychiatric symptoms. Once he reached age 27, he received a trial of therapeutic plasma exchange (TPE), rituximab, and ceftriaxone. This eventually resulted in sustained benefit and minimal fluctuations of his clinical symptoms. Our report is noteworthy in three ways.One, he is a 27-year-old adult with PANDAS.Two, he improved after TPE, rituximab, and ceftriaxone. Our literature search yielded minimal data on the use of plasmapheresis for nonteenage adults with PANDAS. Three, he had unusual symptoms of PANDAS, as the typical OCD and/or tic-like symptoms were not observed.

Pooled platelet concentrates provide a small benefit over single-donor platelets for patients with platelet refractoriness of any etiology.

BACKGROUND: At our institution, patients with platelet refractoriness (of any etiology) are sometimes switched from apheresis platelets to pooled platelets before human leukocyte antigen (HLA)-matched units become available. STUDY DESIGN AND METHODS: Seven patients were analyzed. Platelet counts were available from 57 single-unit transfusions (26 pooled, 31 apheresis). A mixed linear effects model was used and significance was determined using a likelihood ratio test. RESULTS: When analyzed as the only fixed effect in the model, the use of pooled versus single-donor units and time from transfusion to post-transfusion blood sampling each showed a significant effect on platelet count increments. A mixed linear effect model including both factors showed that transfusing a pooled unit correlated with a 4500±2000/µL greater platelet count increment compared with a single-donor unit, and an increase in time from transfusion to post-transfusion blood sampling lowered the platelet count increment by 300±100/µL per hour. CONCLUSION: A small but potentially clinically relevant benefit was observed in transfusing pooled random-donor platelets compared with single-donor units for patients with platelet refractoriness (of any etiology).

Sequential Immune Thrombocytopenia (ITP) and Thrombotic Thrombocytopenic Purpura (TTP) in an Elderly Male Patient with Primary Sjogren's Syndrome: When in Doubt, Use the PLASMIC Score.

INTRODUCTION: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy due to an acquired autoantibody to ADAMTS13 that requires a boutique treatment, urgent plasma exchange. Thus, TTP is often termed a "cannot miss" diagnosis. CASE: We report a patient with TTP who had a history of immune thrombocytopenia (ITP), had atypical demographics for TTP, and had also met criteria for primary Sjogren's syndrome. This exceedingly rare combination presented a temptation to dismiss TTP as a diagnosis. Discussion. Our case further demonstrates the practical utility of using the PLASMIC score as a tool that can help identify patients with TTP even when the patient has statistically rare characteristics.

Transfusion Medicine Education for Anesthesiology Residents: A Novel Curriculum.

Transfusion medicine education for anesthesiology residents was an unmet need at our institution. Our goal was to develop a detailed and organized curriculum to introduce the essentials of transfusion medicine to first-year anesthesiology residents during a 3-week shared rotation. Based on our observations and the residents' feedback, the curriculum was modified after each year with the goal of creating a more resident-focused educational experience. Here, we report our 3-year experience of creating, teaching, and revising this curriculum to 35 residents, and we share our detailed curricula and learning aids (including over 100 directing questions for reading assignments) so that the interested reader can begin using them immediately.

Intrahepatic Cholestasis of Pregnancy Refractory to Multiple Medical Therapies and Plasmapheresis.

Objectives We report on a patient suffering from intractable itching secondary to intrahepatic cholestasis of pregnancy (ICP) unresponsive to conventional medical therapies. She was started on a regimen of therapeutic plasma exchange (TPE), which is often efficacious in relieving patient's itching from all causes of cholestasis, including ICP. Methods We performed a retrospective review of a patient's medical record. Results Following initial TPE, the patient reported dramatic relief of her itching and consequent insomnia. However, this effect was short lived, as subsequent TPE provided minimal relief, and may have actually worsened her itching. Out of concern for poor fetal outcomes, delivery was induced at 34 weeks gestational age. The child had an uncomplicated neonatal intensive care unit stay following delivery, and the mother reported > 90% relief of her symptoms 2 weeks after delivery. Conclusion TPE often provides longer term relief of itching because of ICP; however, it is not a panacea for these symptoms, and sometimes only delivery of the fetus can relieve maternal symptoms. In addition to the refractoriness to TPE, the case is also unusual for the early onset of ICP symptoms and the comorbidity of hepatitis C.

Ventriculoperitoneal Shunt-Associated Vancomycin-Resistant Meningitis Complicating a Patient Undergoing Plasmapheresis for Presumed Systemic Lupus Erythematosus-Associated Transverse Myelitis.

Objectives We report a patient who had a working diagnosis of transverse myelitis secondary to a lupus exacerbation. As his clinical course evolved, continued vigilance clarified that he was in fact suffering from a nosocomial infection caused by vancomycin-resistant Enterococcus (VRE). Methods We performed a retrospective review of the patient's medical record. Results Despite radiographic improvement, he did not improve clinically following immunosuppressive therapy including plasma exchange. Once meningitis was diagnosed and treated, he improved rapidly. Conclusion The prospect of transverse myelitis may confound the diagnosis of VRE meningitis, a uniquely treated and potentially deadly infection.

Photopheresis Provides Significant Long-Lasting Benefit in Nephrogenic Systemic Fibrosis.

Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is a rare complication of exposure to gadolinium-based contrast agents in patients who have significantly decreased renal function. Manifestations include fibrosis of the skin and other tissues. Effective therapies are lacking. Photopheresis has been tried with variable rates of improvement, and small numbers of cases (20 as of 2016) have been reported of NSF patients treated with photopheresis. We report a case of patient with nephrogenic systemic fibrosis who was treated with photopheresis and demonstrated significant lasting improvements.