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Pathological haematology

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Hematology. --- Pathological haematology --- Hematology

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Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of the blood coagulation system. In most cases, a lack of the ADAMTS13 enzyme leads to an accumulation of ultra-large von Willebrand factor molecules in the plasma which, in turn, initiate the formation of microscopic thromboses in small blood vessels. TTP is a medical emergency. Timely diagnosis and urgent and effective management are vital - mortality in those untreated is in the region of 90%.The understanding of TTP pathogenesis has increased markedly in recent decades. It is now known that TTP is acquired (immunemediated) or congenital, and that the most common type - the acquired form - predominantly affects women in their 40s. It is also clear that the prompt delivery of plasma exchange saves lives. 'Fast Facts: Thrombotic Thrombocytopenic Purpura' sets out, in a clearand accessible format, the steps to suspecting, diagnosing and treating this potentially devastating disease. These steps are complemented by clear descriptions of the disease mechanism and epidemiology. Differential diagnosis, which is of the utmost importance for this disease, is explored in detail.Table of Contents:• Disease overview• Clinical presentation• Differential diagnosis• Laboratory findings and diagnosis• Management

Pathological haematology --- Thromboembolism.

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare fast-growing hematodermic neoplasm that most often occurs in older men. It is notable for its highly aggressive behavior, with cutaneous, lymph node and bone marrow involvement. In the past, BPDCN has been poorly understood, recognized and treated, and consequently has had a poor prognosis. Today, it has been reclassified as a myeloid neoplasm and there is greater understanding of the disease’s clinical features, course and pathology, a new diagnostic test that makes prompt diagnosis possible and a new targeted therapy that, so far, has been shown to at least double survival. The complexity of caring for patients with BPDCN stems from both its rarity and its multiorgan involvement. 'Fast Facts: Blastic Plasmacytoid Dendritic Cell Neoplasm' is designed to bring hematologists, oncologists, dermatologists, pathologists, clinical nurse specialists and trainees in all these fields up to speed on the latest developments, as well as providing the most up-to-date information on first-line chemotherapy, consolidation treatments and stem cell transplantation. It will aid readers of all relevant medical disciplines to implement prompt diagnosis and effective management. Table of Contents: • What is BPDCN? Diagnosis • Management • Unmet needs and future directions

Pathological haematology --- Hematology

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Acute lymphoblastic leukemia (ALL) is the most diagnosed childhood cancer worldwide. Although rare in adults, this aggressive cancer also shows a second smaller incidence peak in later middle age.Modern genetic profiling techniques are providing a better understanding of the molecular biology of ALL, which, in turn, is leading to significant advances in diagnosis, prognostication and therapy selection. Ongoing refinement of risk-adapted frontline treatment protocols, use of novel targeted therapies and better supportive care are improving outcomes for patients with ALL.'Fast Facts: Acute Lymphoblastic Leukemia' covers the epidemiology, etiology, diagnosis and classification of ALL as well as the measurement of minimal residual disease to determine prognosis. It provides guidance on frontline therapies and the treatment of relapsed and refractory ALL, including the use of newer targeted agents and the role of allogeneic stem-cell transplantation. Looking to the future, it also considers the approaches and refinements that are in development for ALL.

Pathological haematology --- Leukemia. --- Lymphocytic leukemia.