Therapeutic plasma exchange in refractory Susac's syndrome: A brief report.

Susac's syndrome (SuS) is an autoimmune endotheliopathy that typically presents with the clinical triad of encephalopathy, hearing loss, and branch retinal artery occlusion. It has a wide range of possible presentations, and its pathogenesis remains uncertain. Fulminant and refractory cases are difficult to treat, and no standard treatment protocol has been established. However, therapeutic plasma exchange (TPE) has been described as an adjunctive therapy in several SuS cases. Herein we present a case of a 63-year-old male with debilitating encephalopathy and recent hearing and vision loss, who responded favorably to TPE. Given this and other published reports of plasma exchange therapy for SuS, treatment protocols should consider TPE in early stages of disease.

Guidelines on the Use of Therapeutic Apheresis in Clinical Practice - Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Ninth Special Issue.

The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis (TA) in human disease. In the Ninth Edition, the JCA Special Issue Writing Committee has incorporated systematic review and evidence-based approaches in the grading of evidence and categorization of apheresis indications to make recommendations on the use of apheresis in a wide variety of diseases and conditions. This edition has largely maintained the general layout and concept of a fact sheet introduced in the Fourth Edition (2007). Each fact sheet succinctly summarizes the evidence for the use of TA in a specific disease or medical condition. The Ninth Edition of the JCA Special Issue comprises 91 fact sheets and 166 graded and categorized indications. This includes seven new fact sheets, nine new indications on existing fact sheets, and eight changes in the category for existing indications. The Ninth Edition of the JCA Special Issue seeks to continue to serve as a key resource that guides the utilization of TA in the treatment of human disease.

Transfusion Blood Bank (Recipient) Testing.

Pretransfusion and post-transfusion recipient testing are routine blood bank functions. This article presents a review of request and sample requirements, routine and extended typing and antibody evaluation, and post-transfusion circumstances requiring additional work-up. Although the regimented approach of blood banking fundamentals may be viewed as tedious, these steps are defined and designed to prevent potentially fatal ABO-incompatible transfusions and improve the overall safety of transfusion medicine patients.

Directed Donation: Special Considerations and Review for Contemporary Clinical Practices.

Background: Directed blood donation is defined as the donation of blood or its components for the purpose of transfusion into a specified individual. Directed blood donation holds historic significance, and although practices as of 2021 encourage voluntary, nonrenumerated blood donations, public interest in directed donation remains. Requests to discuss the risks and benefits of directed donations are a common inquiry for transfusion medicine, transplant, and hematology/oncology professionals. This narrative review discusses the history of directed donation and summarizes directed donation considerations in the context of modern transfusion practices. Methods: We conducted a systematic search of PubMed for published literature on the topic of directed blood donation and gathered information about its benefits and potential harms with respect to the variety of products used in transfusion medicine. Results: The drawbacks of directed donation include transfusion-transmitted infection risk, alloimmunization risk, increased transfusion-associated graft vs host disease risk, decreased expediency in treatment, and increased administrative burdens. However, a role remains for directed blood donation in specific patient populations, such as individuals with rare blood types or immunoglobulin A deficiencies, because of the difficulties in finding compatible blood for transfusion. Conclusion: Clinicians should consider the risks and benefits when discussing directed blood donations with patients and family members.

Non-Human Leukocyte Antigen Antibody-Mediated Lung Transplant Rejection: The Other Anti-A.

BACKGROUND: Acute rejection of lung allografts is an important contributor to morbidity and mortality in the transplant patient population, resulting in the dysfunction and destruction of the graft by the host's immune system via cellular or antibody-mediated mechanisms. Acute cellular rejection (ACR) is more common and better characterized than antibody-mediated rejection, which to date lacks any widely agreed upon, standardized set of diagnostic criteria. We present a case of AMR attributable to a rare phenomenon, non-human leukocyte antigen (HLA) antibodies. CASE REPORT: A 50-year-old male underwent an uneventful single lung transplant for pulmonary sarcoidosis. Donor and recipient blood type was A positive. No pretransplant donor-specific antibodies were identified. Flow cytometric crossmatch was negative. The postoperative course was significant for a single-unit transfusion of packed red blood cells on postoperative day (POD) 1 and persistent asymptomatic Serratia marcescens in bronchial washes despite ongoing levofloxacin treatment. A surveillance biopsy (POD 34) showed no evidence of rejection. One week later (on POD 41), the patient presented with fever, shortness of breath, and imaging abnormalities of the grafted lung. Inpatient antibiotic escalation to cefepime, ertapenem, and meropenem resolved the positive cultures and fever, but the patient's respiratory function continued to decline, requiring intubation and extracorporeal membrane oxygenation. High-dose steroids and therapeutic plasma exchanges were initiated for suspected acute rejection. During the workup, a newly developed anti-A1 red blood cell antibody was identified. Despite supportive efforts, the patient died on POD 55, 14 days after symptomatic presentation. CONCLUSION: This case highlights the clinical significance of AMR in lung allografts, as well as the need to investigate both HLA and non-HLA antibody sources in pulmonary transplant rejection refractory to treatment.

Babesia parasitemia rebound after red blood cell exchange.

Babesiosis is an increasingly recognized disease which may benefit from therapeutic apheresis (Category II/Grade 2C). Vulnerable populations include the splenectomized, those aged >50, those with malignancies, and the immunocompromised. In the setting of parasite levels > 10%, significant anemia, renal impairment, pulmonary compromise, or hepatic dysfunction, RBC exchange can rapidly reduce parasite burdens and decrease the bioavailability of proinflammatory cytokines. No previous report has shown such a rapid rebound in parasitemia despite adequate organism removal. Herein, we report a case of severe babesiosis in a splenectomized 56 year old male with a past medical history significant for benign multiple sclerosis. Following a week of flu-like symptoms, the patient presented to an outside hospital with anemia, elevated bilirubin, thrombocytopenia, and 15% of his RBCs containing Babesia forms on a peripheral smear. Despite initiation of appropriate antimicrobials, subsequent transfer to our facility revealed worsening parasitemia (25%), tachypnea, and hypoxia. An emergent two volume RBC exchange was performed, resulting in 15% post-exchange parasitemia. Twelve hours later, the parasitic burden had climbed to 30%. A second RBC exchange reduced the parasite burden to 1.5%. His post-procedural course was significant for diminishing periodic increases in parasitemia despite continued antimicrobial therapy. Rapid increases in parasitic burden following RBC exchanges can occur and post-procedural surveillance of parasitemia should be closely monitored to expedite additional exchanges.

Primary clear cell adenocarcinoma of the colon: a case report and review.

A case of primary clear cell adenocarcinoma of the colon, a rare oncologic variant, was diagnosed in a 25-year-old man who presented with partial bowel obstruction. To understand better the pathology of this neoplasm, a retrospective review of Entrez PubMed entries describing primary clear cell adenocarcinoma of the colon and/or rectum was performed. Only 13 previous cases of primary clear cell adenocarcinoma of the colon and/or rectum have been reported, with an average presentation age of 57 years and generally afflicting the descending colon of men. Herein we present a case occurring in the distal ascending colon of the youngest patient in the literature to date. Our patient's diagnosis is rare in occurrence, location, and age of onset.