The amyloidoses are disorders of secondary structure in which a soluble protein secreted from a cell forms insoluble, fibrillar tissue deposits, leading to organ dysfunction. The site and rate of deposition determine the clinical presentation. All amyloid deposits contain a single major fibrillar component and minor nonfibrillar components. To date, 24 different fibril proteins have been isolated from different forms of human amyloidosis; one is immunoglobulin light chain.
Light-chain amyloidosis (AL) is caused by a monoclonal plasma cell disorder in which the secreted immunoglobulin, either because of its amino acid sequence or some other structural feature, is predisposed to fibrillogenesis under physiologic conditions. AL is characterized by fatigue, weight loss, purpura, heart failure, proteinuria, renal failure, gastrointestinal dysfunction, neuropathy, and various other symptoms, depending upon the organ(s) involved.
Diagnosis of amyloidosis (of any type) is made by biopsy of an affected organ or subcutaneous fat aspiration followed by Congo red staining. In the face of similar clinical features, distinguishing AL from the other systemic amyloidoses, in which AL-specific treatment would be inappropriate, is critical. Chemotherapy reduces the size of the plasma cell clone producing the amyloidogenic light chain and prolongs survival.
Source:
Williams Hematology has guided generations of clinicians, biomedical researchers, and trainees in many disciplines through the origins, pathophysiological mechanisms, and management of benign and malignant disorders of blood cells and coagulation proteins. The book begins with the evaluation of the patient and progresses to the molecular and cellular underpinnings of normal and pathological hematology. Subsequent sections present disorders of the erythrocyte, granulocytes and monocytes, lymphocytes and plasma cells, malignant myeloid and lymphoid diseases, hemostasis and thrombosis, and transfusion medicine.
Featuring a brand-new full-color presentation, the Eighth Edition of Williams Hematology is extensively revised to reflect the latest views of experts in the field. Two new chapters examine the importance of genomics in hematology and cell therapy for tissue replacement, whereas other chapters have been revised to incorporate the newest clinical approaches to the leukemias and lymphomas, and the application of targeted therapy in the treatment of hematologic disorders.
New to this Edition:
Light-chain amyloidosis (AL) is caused by a monoclonal plasma cell disorder in which the secreted immunoglobulin, either because of its amino acid sequence or some other structural feature, is predisposed to fibrillogenesis under physiologic conditions. AL is characterized by fatigue, weight loss, purpura, heart failure, proteinuria, renal failure, gastrointestinal dysfunction, neuropathy, and various other symptoms, depending upon the organ(s) involved.
Diagnosis of amyloidosis (of any type) is made by biopsy of an affected organ or subcutaneous fat aspiration followed by Congo red staining. In the face of similar clinical features, distinguishing AL from the other systemic amyloidoses, in which AL-specific treatment would be inappropriate, is critical. Chemotherapy reduces the size of the plasma cell clone producing the amyloidogenic light chain and prolongs survival.
Source:
Williams Hematology has guided generations of clinicians, biomedical researchers, and trainees in many disciplines through the origins, pathophysiological mechanisms, and management of benign and malignant disorders of blood cells and coagulation proteins. The book begins with the evaluation of the patient and progresses to the molecular and cellular underpinnings of normal and pathological hematology. Subsequent sections present disorders of the erythrocyte, granulocytes and monocytes, lymphocytes and plasma cells, malignant myeloid and lymphoid diseases, hemostasis and thrombosis, and transfusion medicine.
Featuring a brand-new full-color presentation, the Eighth Edition of Williams Hematology is extensively revised to reflect the latest views of experts in the field. Two new chapters examine the importance of genomics in hematology and cell therapy for tissue replacement, whereas other chapters have been revised to incorporate the newest clinical approaches to the leukemias and lymphomas, and the application of targeted therapy in the treatment of hematologic disorders.
New to this Edition:
- Full-color presentation conveniently integrates images of blood and tissue findings where they are cited in the text
- Two additional chapters, “Genomics and Epigenetics,” and “Regenerative Medicine: Principles of Multipotential Cell Therapy for Tissue Replacement,” offer insights into the application of genomic and monoclonal therapies to hematology and emphasize their role in improving diagnostic and therapeutic methods
- Expanded coverage of non-Hodgkin lymphoma includes separate chapters on its constituent diseases, providing an in-depth assessment of the diagnosis and management of these disorders
- A comprehensive discussion of erythrocytosis and thrombocytosis reviews reactive and malignant forms of myeloproliferation, encompassing new genetic advances in these disorders along with more targeted therapeutic approaches
- Companion CD includes a large selection of morphologies, illustrations, and drawings from the book, in a presentation-ready PowerPoint format