A case for a national registry of red blood cell antibodies.

BACKGROUND AND OBJECTIVES: Red blood cell (RBC) antibody levels diminish over time and negative antibody screen are commonly seen in patients with a history of antibodies. Most hospitals do not have access to a shared registry of antibodies previously detected at other hospitals. MATERIALS AND METHODS: We describe a case where the patient was found to be at high risk of bleeding during liver transplantation. Antibody screen on admission was negative but a history of anti-Jka was identified on reviewing patient's history in local registry of RBC antibodies. The surgery was pushed back to arrange for antigen-negative units. The patient received a total of 16 Jk(a-) RBC units during the admission. RESULTS: No acute or delayed transfusion adverse reactions were seen. However, if the history of anti-Jka identified at another local hospital was not known, approximately three-quarters of the units transfused would have been Jk(a+). Transfusing Jk(a+) units could have potentially exposed the patient to risk of developing an acute and/or delayed haemolytic transfusion reaction which could have led to significant morbidity and perhaps mortality. CONCLUSION: With this case report, we build a case for developing a national registry of RBC antibodies to help improve patient safety and outcomes.

Factor V and VIII deficiency treated with therapeutic plasma exchange prior to redo mitral valve replacement.

A 33-year-old male was admitted to the hospital for a repeat mitral valve replacement. The original surgery, performed in India in 2008 due to rheumatic heart disease, required massive amounts of plasma replacement during and after the surgery. The patient was admitted to our hospital with extremely low Factor V and Factor VIII activities due to a rare combined Factor V and Factor VIII deficiency. His clinical condition on admission was grave due to severe pulmonary hypertension. It was decided to replace the patient's Factor V using therapeutic plasma exchange (TPE) with fresh frozen plasma (FFP) just prior to surgery, and his Factor VIII with Factor VIII concentrate. The patient tolerated the valve replacement surgery very well, without excessive bleeding, and received several more TPE procedures postoperatively. He was successfully made replete with both coagulation factors with little to no bleeding during the procedure and postoperatively. TPE is a promising modality for the treatment of patients with similar factor deficiencies for which a specific factor concentrate is not available, especially those at risk of fluid overload from plasma transfusion. J. Clin. Apheresis 32:196-199, 2017. © 2016 Wiley Periodicals, Inc.

Therapeutic Plasma Exchange for Refractory Hemolysis After Brown Recluse Spider (Loxosceles reclusa) Envenomation.

INTRODUCTION: The brown recluse spider (BRS) (Loxosceles reclusa) envenomation can lead to multiple complications, including hemolysis. We present a case of refractory hemolysis after a BRS bite treated with therapeutic plasma exchange (TPE). CASE REPORT: A 17-year-old female presented with fever, fatigue, and dyspnea. She was diagnosed with sepsis and received intravenous (IV) fluids, inotropic support, and antibiotics. On hospital day 1 she was noted to have skin lesion consistent with a BRS bite and developed hemolysis. Systemic loxoscelism with hemolysis was then suspected and methylprednisolone IV was initiated. She was discharged with a stable HGB on hospital day 3 on oral prednisolone. She was re-admitted 24 h later, with signs of worsening hemolysis. Methylprednisolone was restarted and she was transfused 4 units of packed red blood cells. TPE was initiated due to the refractory hemolysis. Shortly after the TPE session, her clinical and laboratory status improved. She required no further transfusions and was discharged on a steroid taper. DISCUSSION: TPE is an extra-corporeal method to remove substances from the blood by separating plasma from cellular blood components and replacing it with physiologic fluids. TPE has been used for snake envenomation but there are no reports detailing its use for BRS envenomations. Improvement was associated with TPE initiation and may have been due to removal of complement components activated by the spider venom. This report suggests that TPE could be a possible treatment modality for systemic loxoscelism with refractory hemolysis due to BRS envenomation. Further investigation is warranted.

Primary composite lymphoma of the larynx, composed of diffuse large B-cell lymphoma and peripheral T-cell lymphoma, not otherwise specified, presenting as left subglottic tracheal fistula, esophageal diverticulum, and neck abscess.

Primary laryngeal lymphoma occurs very rarely, accounting for far less than 1% of primary malignant laryngeal neoplasms. To the best of our knowledge, primary laryngeal composite lymphoma has not been reported in the literature. Herein, we report the first case of primary laryngeal composite lymphoma composed of diffuse large B-cell lymphoma (DLBCL) and peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), in a 43-year-old man. Of special interest is the patient's unique clinical presentation of left subglottic tracheal fistula, esophageal diverticulum, and neck abscess with no discrete mass identified. We describe the clinical and pathological characteristics of this case and review the literature.