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Taher A: Fast Facts: Thalassemia
Syndromes

Fast Facts: Thalassemia Syndromes

Pyruvate kinase deficiency (PK deficiency) is a rare genetic disease that causes red blood cells to break apart easily (hemolysis). Some patients have no or few symptoms; others have very low numbers of red blood cells (hemolytic anemia) and need treatment with regular blood transfusions. Hemolysis occurs in everyone with PK deficiency. It increases the risk of complications that need monitoring, such as gallstones, iron overload, and low bone density. Treatment with mitapivat, which is taken as a tablet, activates pyruvate kinase and reduces anemia and hemolysis in about half of all adults with PK deficiency. Research into other new treatments, including gene therapy, is promising. Common supportive treatments include blood transfusions, removal of the spleen (splenectomy), and medications to remove excess iron from the blood (chelation therapy).

Fast Facts for Patients: Pyruvate Kinase Deficiency

Thermo-Haemo-Dynamics in Medical Engineering

Essentials

The Essentials is an international, best-selling series of textbooks, all of which are designed to support lecture series or themes on core topics within the health sciences. See www.wiley.com for further details. The definitive introductory textbook on haematology, covering basic science, diagnostic testing, clinical features, and patient management Hoffbrand's Essential Haematology has helped medical students and trainee physicians understand the core principles of clinical and laboratory haematology for more than four decades. Original contributions by leading experts provide authoritative coverage of clinical and laboratory features and management within haematology, including the haematological aspects of systemic diseases, pregnancy, and the neonate. Hundreds of high-quality colour images illustrate various anaemias and white cell disorders, leukaemias, lymphomas and myeloma, bleeding and thrombotic disorders, and other blood diseases. Now in its ninth edition, this classic textbook incorporates current knowledge of the pathogenesis of blood diseases, the 5th WHO (2022) classification of haematological neoplasms, the detection of minimal residual disease, advances in the treatment of benign and neoplastic blood diseases. New sections focus on the haematological consequences of COVID-19 infection and vaccine -induced immune thrombotic thrombocy-topenia (VITT). Additional and expanded chapters describe non-Hodgkin lymphomas, amyloid, and haemophilia. Supported by a companion website with hundreds of MCQs and PowerPoint slides, Hoffbrand's Essential Haematology, Ninth Edition, remains an indispensable resource for trainee haematologists and physicians.

Hoffbrand's Essential Haematology

This case-based book is focused on perioperative pediatric PBM and blood transfusion safety, while addressing a number of patient management issues.&nbsp;Divided into three sections covering all phases of perioperative care, each chapter consists of a stem question, followed by an analysis of the issue, then a discussion of the current evidence in the literature. Rather than being a transfusion-related book written by pathologists and laboratory specialists, chapters are authored by highly specialized clinicians who will write from first-hand experience in addition to an exploration of the current literature. The book features management summaries of topics with scant or only recently available evidence and offers novel content and format in the pediatric patient literature.&nbsp;Perioperative Pediatric Patient Blood Management and Transfusion Safety is aimed for any clinician that treats a child before, during, or after a blood loss surgery.

A Case-Based Review

Perioperative Pediatric Patient Blood Management and Transfusion Safety

This Handbook takes the reader through the entire field of pediatric thrombosis and hemostasis and has been updated since the 2nd edition with considerable new information and elements missing in previous editions. The Handbook begins with an overview of the history of pediatric thrombosis and hemostasis, followed by a concise explanation of the complex pathophysiology of thrombosis and hemostasis. It follows with chapters focused on disorders of hemostasis in children from neonates to adolescents. Subsequent chapters are focused on thrombotic complications in children together with workup and management of thrombophilias in children. New chapters on bleeding in the adolescent girl and on management of children on mechanical circulatory support (ECMO/VAD) have been added. There is also a new chapter focused on hemostatic and thrombotic challenges in children with vascular anomalies, as pediatric hemostasis and thrombosis clinicians are increasingly being asked to manage children with these conditions. This updated Handbook is intended for health care professionals involved in the assessment and care of children with inherited and acquired bleeding and thrombotic disorders to inform and facilitate diagnostic/treatment decisions. This includes general and specialist pediatricians, in particular intensivists, neonatologists, cardiologists/cardiac surgeons, rheumatologists, nephrologists, and hematologists/oncologists, as well as medical trainees, residents, fellows, nurses, nurse practitioners, physician assistants, and pharmacists.

SickKids Handbook of Pediatric Thrombosis and Hemostasis

This comprehensive textbook brings together global experts to deliver an in depth, evidence based guide covering the full spectrum of acquired and inherited marrow failure syndromes. It explores cutting edge advances in diagnostics including next generation sequencing , other genetic studies and the evolving progress in &nbsp;treatment strategies including &nbsp;immunosuppressive therapy, hematopoietic stem cell transplantation, and targeted agents. Special attention is given to age specific management, overlap syndromes and the increasingly recognized inherited bone marrow failure disorders. A dedicated section addresses the unique challenges faced in resource limited settings, offering strategies to improve global access to care. Designed for clinicians, researchers, and allied health professionals, this textbook serves as an essential, practical reference that bridges research and real world practice. It equips readers with the tools to improve outcomes for patients worldwide affected by bone marrow failure.&nbsp; This book is open access.

Textbook of Bone Marrow Failure

This book is mainly about solving clinical problems in the application of medical apheresis.&nbsp;Unlike many other books on apheresis, it is kept short and concise. Many pictures, tables and workflow diagrams help in understanding the solutions presented.The aim is to guide professionals in transfusion medicine, pathology, haematology - oncology, neurology, rheumatology, nephrology and transplant medicine through the process of treatment and decision-making in the clinic and in their own practice. The book provides practical knowledge that can be immediately applied in patient care to optimise the apheresis treamtent.&nbsp;

Problem Solving in Apheresis Medicine

Die Immunthrombozytopenie (abgekürzt ITP) ist eine seltene Erkrankung, bei der es im menschlichen Körper zu wenig Blutplättchen gibt. Diese Broschüre erklärt, was ITP ist, wie diese chronische Krankheit das Leben beeinflusst und welche Behandlungen es gibt. Außerdem erfahren Sie, was Sie selbst tun können, um Ihre Lebensqualität zu verbessern und ein weitgehend normales Leben zu führen.

 Der Ratgeber wurde gemeinsam mit Herrn Prof. Dr. Axel Matzdorff und einer Gruppe von Patient*innen entwickelt.

Fast Facts für Patient*innen: Leben mit Immunthrombozytopenie

Diseases of the hematopoietic system often manifest themselves in the skin, and some manifestations are so typical that the dermatologist may be the first physician to detect the underlying blood disorder. More commonly, however, skin symptoms arise in the context of a known hematopoietic disease. This book reviews skin signs and symptoms that develop secondarily to underlying malignant or benign diseases of the hematopoietic system, and mainly built as a clinical text to support the physician in making a correct diagnosis and understanding the significance of skin signs for the prognosis and management of the hematological disease. The malignant hematological diseases comprise approximately 80% of the book, which reflects the spectrum of patients in hematology departments, as well as the experience of the authors. In addition to its pragmatic structure that enables the reader to find relevant information, tables are included for differential diagnoses and cross-referencing the skin sign with hematological diagnoses and vice versa. For each chapter, ample background information is provided to convey dermatologists the essence of hematological disorders. Likewise, to enhance understanding among hematologists, dermatological jargon is avoided and skin signs described in understandable terms, underscoring the patterns of the lesions and key features allowing the non-dermatologist clinician to differentiate one skin condition from another.This book is primarily for the physicians who see adult patients; to achieve consistency and readability, the work is meant as a monograph written by specialists in the field, who collaborate with one another on a daily basis. While not aiming to be an exhaustive reference work, it is built as an intelligible handbook that for the first time covers the cross-section of dermatology and hematology. The audience comprises dermatologists working in an academic or hospital setting, as well as hematologists and internists who manage patients with blood diseases in their daily clinical practice.

A Dermatologic Correlation of Hematologic Disorders

Diseases of Blood and Skin

This five-volume series provides clinicians with a detailed, up-to-date, well-referenced overview of the latest developments in this rapidly expanding field. It serves as an authoritative guide to the diagnosis and treatment of platelet disorders.The primary audience for the series is clinicians, residents and fellows in internal medicine, cardiology and haematology, as well as clinicians (e.g. neurologists, nephrologists, rheumatologists) and basic scientists who may be concerned with disorders and cellular mechanisms involving platelets.

Volumes 1-5

Platelets in Thrombotic and Non-Thrombotic Disorders

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This "transversal" book is a valuable resource not only for hematologists but also for clinicians who may encounter acquired bleeding situations that they must manage, even if they lack access to a bleeding disorder specialist. The discussed conditions are relatively prevalent and can lead to frequent bleeding complications of varying severity, such as bleeding during anticoagulant therapy. The book also covers the significance of disseminated intravascular coagulation, another common complication that varies in severity based on the underlying condition that triggered it.
This book fills a void by addressing these common complications in a single resource that will prove beneficial to medical professionals in training and specialists in various medical fields who treat patients with specific clinical condition.
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How to Manage Acquired Bleeding Disorders

Die zielführende Diagnostik bei hämatologischen Erkrankungen beginnt beim zytomorphologischen Befund am Mikroskop. Er wird in diesem Buch mit über 800 exzellenten Bildern von Blut- und Knochenmarkausstrichen für häufige und seltene hämatologische Erkrankungen gezeigt. Die Befunde können auch als Vergleichsbilder für die eigene Diagnostik verwendet werden. Davon ausgehend werden die Indikation für weiterführende immunphänotypische, histologische und insbesondere zytogenetische und molekulargenetische Untersuchungen und deren Stellenwert beschrieben.In der 4. Auflage ist die neue WHO-Klassifikation der Hämatolymphoiden Tumoren von 2022 berücksichtigt. Neue Entitäten sind beschrieben und mit neuem Bildmaterial illustriert, speziell im Hinblick auf die Korrelation zwischen Phänotyp und Genotyp. Vermehrt wird auch auf aktuelle spezifische Therapien mit molekularem Bezug aus der Diagnostik verwiesen.Aus dem Inhalt: Störungen der Erythropoese - Reaktive Blut- und Knochenmarkveränderungen - Angeborene Anomalien der Granulozytopoese - Benigne Veränderungen der Lymphozyten - Aplastische Anämien (Panmyelopathien) - Speicherkrankheiten - Hämophagozytische Lymphohistiozytose (HLH) - Myeloproliferative Neoplasien (MPN) - Myeloische und lymphatische Neoplasien mit Eosinophilie und Anomalien von PDGFRA, PDGFRB, FGFR1 oder mit PCM1-JAK2 - Myelodysplastische/myeloproliferative Neoplasien (MDS/MPN) - Myelodysplastische Neoplasien (MDS) - Myeloische Neoplasien mit Keimbahn-Prädisposition - Akute Leukämien - Lymphatische Neoplasien - Dendritische Zellneoplasien - Tumoraspirate bei KnochenmarkbefallDer Autor: Prof. Dr. Dr. Torsten Haferlach, MLL Münchner Leukämielabor GmbH, München&nbsp;

Atlas und diagnostisches Handbuch

Hämatologische Erkrankungen

Fast Facts: Thalassemia Syndromes

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