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Tumors of the Central Nervous System

Central nervous system (CNS) tumors can involve the brain or the spinal cord, may be benign or malignant, and may be primary or metastatic. In the United States, about 80,000 new primary brain tumors and other nervous system tumors are diagnosed annually, and about one-third of primary CNS tumors are malignant. In contrast, intracranial metastases are five times more common than primary brain tumors. Over 100…

Amyloidosis

The amyloidoses are protein-misfolding diseases caused by a precursor protein that undergoes a conformational change, which in turn triggers the formation of amyloid fibrils that damage different organs. Amyloidosis can be either localized or systemic. In systemic amyloidosis ( Table 174-1 ), amyloid deposition occurs at sites distant from where the precursor protein is formed. In localized amyloidosis, the deposits form exclusively where the precursor protein…

Plasma Cell Disorders

Definition Plasma cell disorders are neoplastic or potentially neoplastic diseases associated with the clonal proliferation of immunoglobulin-secreting plasma cells ( Table 173-1 ). These disorders are characterized by the secretion of electrophoretically and immunologically homogeneous (monoclonal) proteins that represent intact or incomplete immunoglobulin molecules. Monoclonal proteins are commonly referred to as M proteins, myeloma proteins, or paraproteins. TABLE 173-1 PLASMA CELL PROLIFERATIVE DISORDERS I. Premalignant monoclonal…

Hodgkin Lymphoma

Definition Hodgkin lymphoma, one of the B-cell lymphomas, consists of two major types: classic Hodgkin lymphoma, with a characteristic neoplastic cell, the Hodgkin-Reed-Sternberg cell; and the much less common (~10% of cases) nodular lymphocyte-predominant Hodgkin lymphoma, with a characteristic predominance of small lymphocytes. Both types have a distinct natural history, and most important, an excellent response to treatment, with the large majority of patients being cured.…

Non-Hodgkin Lymphomas

Definition Lymphomas are solid tumors of the immune system. Increasing knowledge of the biology of the immune system has led to a corresponding increase in the understanding of these malignancies. In addition to better systems of classification and clinical evaluation, this new knowledge has led to the development of new therapies. Beneficial treatment is available for essentially every patient with non-Hodgkin lymphoma. The overall survival of…

CHRONIC Myeloid Leukemia

Definition Chronic myeloid leukemia (CML) is a hematopoietic stem cell disease caused by the reciprocal translocation of gene sequences from the breakpoint cluster region ( BCR ) gene on chromosome 22 with the tyrosine kinase sequences of the Abelson ( ABL ) gene on chromosome 9. On a cytogenetic level, this results in an elongated chromosome 9 and a shortened chromosome 22, the latter known as…

Chronic Lymphocytic Leukemia

Definition Chronic lymphocytic leukemia is a mature B-cell malignancy in which the neoplastic cells share an immunoglobulin gene rearrangement. The disease involves the blood, bone marrow, lymph nodes, spleen, liver, and often other organs at the time of presentation, but clinical manifestations range from asymptomatic disease that may not require therapy for decades to rapidly progressive disease that soon can be life-threatening. Recent therapeutic advances using…

Cancer Biology and Genetics

Introduction Cancer is a collection of many different diseases and is often not uniformly categorized by even the tissue of origin. For example, hormone receptor–positive breast cancers are distinct in biology, prognosis, and treatment compared with hormone receptor–negative breast malignancies. A unifying feature shared by all human cancers is that they have a genetic basis, even though familial cancer syndromes are relatively uncommon compared with sporadic…

Epidemiology of Cancer

Cancer Burden and Trends Global Statistics Cancer is a major public health concern worldwide. The World Health Organization (WHO) estimates that cancer is the first or second leading cause of death before age 70 years in the majority of countries. In 2020, 19.3 million new cases of cancer were diagnosed globally, and almost 10 million deaths were attributable to cancer ( Fig. 165-1 ). Among women,…

Approach to the Patient with Cancer

Introduction to the Cancer Patient Conveying or receiving an initial diagnosis of cancer, or the knowledge that cancer has recurred, is among the most difficult of human enterprises, and no amount of either specialized training or forewarning can adequately assuage the intensity of the emotions associated with these encounters. Patients often experience a storm of feelings that may limit useful discussion immediately following the receipt of…

Hematopoietic Cell Transplantation

Hematopoietic cell transplantation is a procedure by which hematopoietic stem and progenitor cells are infused intravenously to restore hematopoiesis and immune function following a chemotherapy conditioning regimen, with or without radiation therapy, that ablates the endogenous hematopoietic and immune system. Stem cells used for hematopoietic cell transplantation are of hematopoietic origin, in contrast to the more primitive pluripotent stem cells (i.e., embryonic stem cells) that are…

Transfusion Medicine

Blood transfusion can be lifesaving and is currently the safest it has ever been. However, blood transfusion is associated with multiple adverse outcomes, including both noninfectious and infectious complications. The benefits and risks of transfusion can be considered for each patient using evidence-based, multidisciplinary approaches to reduce unnecessary transfusions and improve patients’ outcomes. As a result, the number of units of blood transfused has declined substantially…

Disseminated Intravascular Coagulation and BLEEDING IN LIVER FAILURE

Disseminated Intravascular Coagulation Definition Disseminated intravascular coagulation (DIC), also referred to as consumptive coagulopathy or defibrination, is caused by a wide variety of serious disorders ( Table 161-1 ). In most patients, the underlying process dominates the clinical picture, but in some cases (e.g., occult malignant neoplasm, envenomation), DIC may be the initial or predominant manifestation of the disorder. DIC never occurs in isolation, without an…

Coagulation Factor Deficiencies

Coagulation Deficiencies Severe coagulation deficiencies, or coagulopathies, are typically characterized by spontaneous or provoked bleeding, such as during surgery or trauma, and may result in life- or limb-threatening complications. By contrast, moderate and mild coagulopathies may remain clinically silent until they are detected coincidentally on routine laboratory screening tests (e.g., prothrombin time [PT], activated partial thromboplastin time [aPTT]), or when these tests are ordered to evaluate…

Parenting and Parenting Behavior

For additional material related to the content of this chapter, please see Chapters 6 , 12 , 20 , and 21 . Vignette Parents and their newborns continued to trickle into the community center meeting room for the Parents of Newborns group. As they waited for the meeting to start, Sarah began chatting with Jen, who was sitting next to her. “I’m so looking forward to…

Understanding Child Development and Behavior in the Context of Family Systems

For additional material related to the content of this chapter, please see Chapter 10 , 13 , 81 , 97 , and 117 . Vignette The Martin Family Karen and Scott married in their late 40 s, after each ended their first marriages. Both brought biological children to their blended family: Karen’s daughters (Emily and Sara) and Scott’s sons (Brett and Justin) all were older adolescents…

End-of-Life, Death, and Bereavement

For additional material related to the content of this chapter, please see Chapters 59 and 63 . Vignette Emmanuel is a 14-year-old boy born with epidermolysis bullosa (EB), a rare genetic blistering skin disease, who develops acute worsening fatigue, shortness of breath, and severe cachexia with loss of 18% of his body weight. He is diagnosed with heart failure and a large left ventricular thrombus and…

Transition to Adulthood

For additional material related to the content of this chapter, please see Chapters 9 and 105 . Vignette Evelyn is a 17-year-old girl with attention-deficit/hyperactivity disorder (ADHD) who is beginning her senior and final year of high school. She is experiencing a significant amount of stress as she thinks about all the decisions that she must make this year. Her parents would like her to attend…