Nonmalignant hematology

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Nonmalignant Hematology Expert Clinical Review: Questions and Answers Syed A Abutalib Jean M Connors Margaret V Ragni Editors 123 Nonmalignant Hematology Syed A Abutalib • Jean M Connors Margaret V Ragni Editors Nonmalignant Hematology Expert Clinical Review: Questions and Answers Editors Syed A Abutalib Cancer Treatment Centers of America Department of Hematology and Bone Marrow Transplantion Zion Illinois USA Margaret V Ragni Department of Medicine University of Pittsburgh Medical Center Pittsburgh Pennsylvania USA Jean M Connors Dana-Farber Cancer Institute Brigham and Women’s Hospital Boston Massachusetts USA ISBN 978-3-319-30350-5 ISBN 978-3-319-30352-9 DOI 10.1007/978-3-319-30352-9 (eBook) Library of Congress Control Number: 2016946230 © Springer International Publishing Switzerland 2016 This work is subject to copyright All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed The use of general descriptive names, registered names, trademarks, service marks, etc in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made Printed on acid-free paper This Springer imprint is published by Springer Nature The registered company is Springer International Publishing AG Switzerland I dedicate this book to my everlasting thirst for acquiring medical knowledge, my valued mentors who I strive to emulate in their compassion and advocacy on behalf of the patient, and my junior colleagues who continue to teach and remind me that there is always more to learn, and sincerest thanks to my family for their support, most especially my daughter who is the love of my life Syed A Abutalib For my parents, for imparting that a balanced life is essential, for my husband and children who let me know when work is consuming the balance; and for my mentors and colleagues–I thank you for keeping me in the game Jean M Connors To my husband Fred and children, Christopher and Caroline, for their inspiration and support, and to my mentors, especially my teachers, colleagues, fellows, students, and patients, who made this endeavor possible Margaret V Ragni Preface The field of “benign” or “nonmalignant” hematology encompasses a wide and diverse range of inherited and acquired disorders, including abnormalities in the number or function of white cells, red cells, and platelets, as well as coagulation disorders that can result in bleeding or clotting Significant advances in technology development now allow rapid diagnosis using sophisticated clinical tests, while a parallel development in treatments has occurred that can alter the course and outcome of many of the nonmalignant hematologic disorders Therapies that target the precise pathophysiologic mechanism at the level of the cell, protein, or nuclear material have developed at a rapid pace in the last decade Assimilating and applying these new diagnostic and therapeutic modalities to daily patient care can be challenging Our book provides a concise case-based approach to the diagnosis and management of the many disorders faced by hematologists in academic and community-based practice Readers will become familiar with both the basics and nuances in care in a case-based format written by experts in the field Physicians in training, and physicians in any discipline wishing to increase their knowledge in this subspecialty area, will find the question and answer format practical and informative, with direct relevance to daily practice Readers will find that Nonmalignant Hematology: Expert Clinical Perspective has clear takeaway points that are invaluable for physicians in any specialty faced with patients with hematologic disorders It is hoped that professionals reading this book will find the content of value in their own interactions with their patients Zion, IL, USA Boston, MA, USA Pittsburgh, PA, USA Syed A Abutalib, MD Jean M Connors, MD Margaret V Ragni, MD, MPH vii Contents Red Blood Cells Evaluation of Anemia in Children and Adults Peter W Marks Iron Homeostasis and the Pathophysiology and Management of Iron Deficiency 13 Gordon D McLaren, Roman L Kleynberg, and Gregory J Anderson Porphyrias: Diagnosis and Management 23 Peter V Tishler Disorders of Hemoglobin Synthesis: Pathophysiology and Diagnostic Evaluation 29 Elena Cassinerio, Giovanna Graziadei, and Maria Domenica Cappellini Management of Thalassemias 39 Sherif M Badawy and Alexis A Thompson Allogeneic Hematopoietic Cell Transplant in β-Thalassemia Major 53 Syed A Abutalib Sickle Cell Disease: Prevention of Complications 63 Enrico Maria Novelli Sickle Cell Disease: Management of Complications 75 Michael Winstead and Elliott Vichinsky Allogeneic Hematopoietic Cell Transplant in Sickle Cell Disease 89 Santosh L Saraf Anemia of Inflammation 97 Robert T Means Jr Iron Overload: Diagnosis, Complications, and Management 103 Pierre Brissot Megaloblastic and Nutritional Anemias 113 Sally P Stabler ix Human Blood Antigens and Antibodies: Diagnostic and Therapeutic Implications positive for IgG only and an eluate showed antiJka Additionally, as part of the workup for the anti-Jka, the reference lab performed a red cell genotype Question What is the most likely cause of the signs and symptoms that made her visit her pediatrician following the transfusion? A Intravascular hemolysis due to naturally occurring RBC antibody B Extravascular hemolysis due to an anamnestic RBC antibody C Bacterial contamination of the transfused product resulting in a septic transfusion reaction D Mechanical hemolysis as a result of transfusing blood and antibiotics through the same IV E Drug-induced hemolytic anemia due to antibiotics Expert Perspective: Hemolytic transfusion reactions (HTRs) are adverse events caused by antibodies binding to their target on transfused RBCs and causing their destruction Acute hemolytic reactions (AHTR) occur within 24 h of the transfusion and are caused by preformed antibodies that bind to and destroy the transfused antibodies almost immediately after they have been transfused (Davenport 2012) Data from the United Kingdom’s serious hazards of transfusion (SHOT) biovigilance database from 2005 to 2010 (reviewed in Popovsky Transfusion reactions 2012) showed that nearly 80 % of AHTRs reported were caused by antibodies to antigens other than those in the ABO system Delayed hemolytic reactions (DHTR) occur >24 h following the transfusion and are typically caused by anamnestic antibody reactions, i.e., an RBC antibody whose titer had decreased below the level of detection of the method used to perform the antibody screen, can increase following a second exposure to RBCs containing that antigen This increase can occur over days or weeks and lead to the destruction of any remaining transfused cells, as occurred to the patient in this case study Occasionally, new alloantibodies formed in a primary immune response to an RBC antigen can lead to a DHTR 701 Alloimmunization mitigation in the transfusion management of SCD is quite variable among institutions The prevalence of RBC alloimmunization after at least one red cell transfusion ranges from to 30 % (Vichinsky et al 1990; Miller et al 2013) At the author’s institution, the pattern of RBC antigens (i.e., those that are present and absent) in a SCD patient is determined on their first encounter This pattern of antigens is known as an RBC phenotype (Harm et al 2014) SCD patients who have not produced an RBC antibody receive RBCs matched to their Rh and Kell phenotypes Once a patient becomes alloimmunized, antigen-negative red cells are provided, and every effort is made to match the donor’s RBC phenotype to that of the recipients in order to minimize exposure to foreign antigens Matching donor and recipient RBC phenotypes has been shown to reduce alloimmunization (Lasalle-Williams et al 2011), and using genetic techniques to facilitate donor and recipient matching is becoming more mainstream (Wilkinson et al 2012; Klapper et al 2010) Case 3: Review of TransfusionRelated Acute Lung Injury (TRALI) and Transfusion-Associated Circulatory Overload (TACO) A sixty-five-year-old male with a history of hypertension controlled with medications, angioplasty for coronary artery disease, and arthritis presents for a hip replacement surgery His daily medications include low-dose aspirin, beta blocker, and a statin He is a nonsmoker He is blood type A+ with a negative antibody screen His surgery was largely uncomplicated; however, his postoperative platelet count was 197 × 109/L, and his surgeon wondered if his platelets were not adequately functional Due to ongoing oozing from the wound, the physician decided to transfuse one unit of apheresis platelets Thirty minutes into the transfusion, the patient became hypoxic and dyspneic His O2 saturation dropped to 90 % on room air The transfusion was stopped and a chest X-ray was performed The chest X-ray showed bilateral pulmonary edema C.A Sheppard et al 702 Furosemide was immediately administered; however there was no clinical improvement in his respiratory function The patient continued to decompensate and was finally intubated and mechanically ventilated The 36 h following the transfusion, the CXR showed a decrease in edema and the patient was able to be extubated uneventfully Question Based on these signs and symptoms, which is the most likely clinical diagnosis? A B C D Transfusion-associated cardiovascular overload Transfusion-related acute lung injury Allergic reaction Acute hemolytic reaction Question Which of the following donors has the highest risk for causing TRALI? A A female with one previous pregnancy B A male with a previous blood transfusion history C A man or female with a previous organ transplant D A multiparous female Question Prior to the implementation of donor TRALI risk mitigation strategies, which blood component was most commonly implicated in causing TRALI? A B C D E Plasma Cryoprecipitate Whole blood-derived platelets Packed red blood cell Whole blood Expert Perspective: TRALI is an adverse event caused by the transfusion of bioactivators present in blood components and manifested by noncardiogenic pulmonary edema resulting in dyspnea and typically profound hypoxia Its incidence has recently been reported to be 1:12,000 blood components (Toy et al 2012) TRALI is the leading cause of transfusion-related mortality reported to the FDA (72/190 (38 %) reported fatalities to FDA from 2009 to 2013) It is a clinicopathological diagnosis A patient without previous acute lung injury who develops bilateral pulmonary edema within h of transfusion and has no signs of cardiogenic edema (i.e., left atrial hypertension, a newly increased BNP, and/or a lack of response to appropriate doses of diuretics) might be experiencing a TRALI The exact etiological mechanism by which TRALI occurs is unknown However, HLA antibodies that are found in high plasma volume blood products are commonly implicated as the causative agent in TRALI cases (Middelburg et al 2008; Bux and Sachs 2007) Multiparous females have a much higher prevalence of HLA antibodies than male donors or nulliparous females (Triulzi et al 2009) Thus the current TRALI mitigation strategy, which was recommended by the AABB in 2007 and mandated in April 2014, involves identifying multiparous females and excluding them from donating high plasma volume products either on spec or if they are shown to have HLA antibodies This strategy has been successful in reducing reported TRALI cases (Eder et al 2013; Müller et al 2015) The second most commonly reported cause of transfusion-related fatality to the FDA from 2009 to 2013 was TACO comprising 24 % of cases TACO, while appearing clinically similar to TRALI, is primarily a result of circulatory overload due to a rate of infusion and/or a quantity of transfusion that overwhelms the recipient’s ability to compensate for the increased intravascular volume There is no minimum volume of transfused blood products that can lead to TACO; even younger patients who receive modest amounts of blood products can experience this reaction if they have other risk factors or are transfused rapidly Recent prospective studies have demonstrated that the prevalence of TACO is much higher than historically reported (1:68–356 units of plasma transfused) and is frequently underreported (Narick et al 2012; Rana et al 2006) Other predictors may include a history of CHF, preexisting left ventricular dysfunction (Li et al 2011) and renal failure, in particular if the recipient requires dialysis Signs of left heart strain and volume overload may help differentiate TRALI from TACO, and an elevated B-type naturitic peptide can indicate patients both at risk of TACO Human Blood Antigens and Antibodies: Diagnostic and Therapeutic Implications and those who have experienced this reaction An improvement in the recipient’s breathing following diuretic use, administered cautiously in a patient with unstable blood pressure, can be both diagnostic and therapeutic Case 4: Review of Red Blood Cell age and Clinical Outcome Question A 57-year-old male with CAD presents today for his CABG surgery The anesthesiologist is consenting him for the procedure and possible blood transfusion when the patient asks, “Is blood transfusion safe?” The patient states that he is aware that infectious disease risks such as hepatitis C and HIV have been greatly minimized but remembers hearing something about the deleterious effects of “old blood.” Is there a clinically important difference between the effect of shorter storage age RBCs vs longer storage age RBC on clinical outcome and mortality risk in premature neonates, cardiac surgery, and ICU patients? Expert Perspective: Some observational studies have suggested that patients who received “older” RBCs, typically >14 days old, experienced more adverse events and increased mortality compared to patients transfused with fresher RBCs Recently three randomized, controlled clinical trials that analyzed the frequency of adverse events in patients transfused with fresh vs older RBCs have concluded These studies include the red cell storage duration study (RECESS) in cardiac surgery patients, the age of red blood cells in premature infants (ARIPI), and the age of blood study (ABLE) in ICU patients RECESS is a US, multicenter, partially blinded, randomized clinical trial (RCT) which sought to evaluate clinical outcomes in patients receiving RBCS stored for either ≤10 days or ≥21 days while undergoing complex cardiac surgery The primary outcome was a change in multiple organ dysfunction score (MODS, a predictor of mortality) starting before surgery and going through day 7, death, or discharge There were 703 1098 evaluable patients The study found that RBC storage duration was not significantly associated with a 7-day change in MODS, serious adverse events, or 28-day all-cause mortality (Steiner et al 2015) ARIPI is a Canadian, multicentered, randomized study of premature neonates (Fergusson et al 2012) that evaluated the impact of the transfusion of RBCs that were
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Xem thêm: Nonmalignant hematology , Nonmalignant hematology , Case 4. Undiagnosed Congenital Causes of Anemia in Adulthood, Case 2. Diagnosis and Management of a Neonate with Bleeding, Case 1. Review of the Complications and Management of Type 1 VWD, Case 2 and 3. Review of the Complications and Management of Non-DDAVP Responsive VWD, Case 3. Warfarin Monitoring in Special Circumstances, “I Have a History of TTP or AHUS. Can I Become Pregnant?”, Surgical Technique (Comerota et al. 2012) With Mayo Clinical Institutional Practice Modifications

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