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Molecular pathology

1 Compare and contrast DNA and RNA as targets for molecular testing. DNA is double-stranded with A-T and C-G pairs of deoxyribonucleotides; it includes exons, introns, and intergenic regions; all nucleated normal cells have the full DNA complement; and DNA is relatively stable. RNA is usually single stranded using uracil in place of thymine; ubiquitous ribonucleases make RNA unstable outside of living cells requiring rapid/immediate sample…

Pediatric solid tumors pathology

1 Histology is one of the most important factors that play a role in risk stratification of neuroblastomas. What features differentiate between favorable and unfavorable histology, and how do they relate to patient age? Neuroblastoma (NB) is the most prevalent pediatric sarcoma characterized by a significant heterogeneity affecting the tumor behavior and prognosis. The tumor originates from a subset of nerve crest cells programmed to differentiate…

Neoplastic hematopathology

1 What is B-cell acute lymphoblastic leukemia, and how is it diagnosed? B-cell acute lymphoblastic leukemia (B-ALL) is a neoplasm of B lineage lymphoid progenitors (B lymphoblasts). It includes multiple distinct subtypes characterized by constellations of genetic alterations, including aneuploidy, chromosomal rearrangements, DNA copy number alterations, and sequence mutations. The bone marrow (BM) usually shows extensive involvement by lymphoblasts at initial presentation. Rarely the marrow can…

Principles of infectious disease and management of febrile neutropenia

1 What are typical infectious complications in pediatric oncology patients? Typical infectious complications include bacterial sepsis, systemic or organ-specific infections caused by either primary or reactivated viruses (e.g., cytomegalovirus [CMV], Epstein-Barr virus [EBV]), respiratory viruses (e.g., influenza, respiratory syncytial virus [RSV]), and invasive fungal infections (e.g., Candida , Aspergillus sp.) 2 How does bacterial infectious risk vary across the spectrum of oncology patients? The risk of…

Pediatric radiation oncology

1 How does radiation therapy work? In the realm of external beam radiation (the most commonly used radiotherapy), a linear accelerator produces a beam of high-energy electrons, which hits a metal target at the head of the machine. Upon striking the target, electron energies are subsequently converted to x-ray (photon) energies. Filters and multileaf collimators (MLCs) are placed distal to the target in the head of…

Transfusion medicine

1 Describe the collection processes, storage conditions, clinical indications, and pediatric dosing for red blood cells, fresh frozen plasma, platelets, and cryoprecipitate (see Table 18.1 ). Red blood cells (RBCs): Indication: Increase oxygen-carrying capacity Collection: Apheresis or centrifugation of whole blood Storage: 1°C to 6°C Dosing: 10 to 15 mL/kg Table 18.1 Blood Products With their Pediatric Dosing, Indications, Processing, and Storage Component Dosage for patients…

Clinical emergencies in children with cancer

1 What is tumor lysis syndrome? Tumor lysis syndrome (TLS) is defined by the metabolic derangements that result from massive release of intracellular contents into the systemic circulation secondary to the initiation of cytotoxic therapy. The laboratory findings of TLS include hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia, and an increasing serum creatinine; clinically, TLS may cause renal failure, cardiac arrhythmias, seizures, or sudden death. Serum lactate dehydrogenase (LDH)…

Health equity and disparities in pediatric and adolescent/young adult oncology

1 What are health disparities? In discussions about cancer-related outcomes, the word “disparity” is often used interchangeably with other words like “difference” or “inequity.” But disparities are not simply “any” differences in health. Rather, disparities, as defined by the World Health Organization (WHO), are avoidable differences in health arising from the social and economic conditions that determine an individual’s risk of illness, and the actions taken…

Predisposition syndromes

1 What percent of childhood cancer is attributable to inherited mutations? In a recent study of 1120 children and adolescents with cancer, germline mutations in cancer-predisposing genes were identified in 8.5% of the cases. With the exception of this small percentage of cases attributable to hereditary cancer syndromes or genetic syndromes such as Down syndrome, the etiology of most childhood cancers is unknown. Importantly family history…

Precision medicine and systems biology

1 What is precision medicine, and how does it relate to pediatric oncology? Precision medicine refers to approaching the treatment and prevention of disease by tailoring patients’ care based on their individual variability in genes, environment, and lifestyle. Oncology has been at the forefront of applying precision medicine principles to the care of patients supported by a rapid expansion in genomic technologies and large-scale databases along…

Principles of immunotherapy

1 What is immunotherapy? Immunotherapy is a form of cancer therapy that uses the innate or adaptive immune system of a patient to eradicate malignant cells. Although some variations of this therapy directly stimulate or disinhibit the patient’s own immune system, other variations of this therapy use immune cells manipulated ex vivo and introduce or reintroduce the cells back to the patient. 2 How does the…

Principles of chemotherapy

1 What is the single most important determinant when choosing a treatment using anticancer drugs (chemotherapy) and what is the concept of “risk stratification”? Before embarking on a treatment plan that involves the use of anticancer drugs, every effort should be made to ascertain an accurate histologic and molecular diagnosis. This involves pathologic examination and molecular testing of tumor tissue after surgical excision or a well-planned…

Epidemiology

1 Internationally there are approximately 300,000 cases of child and adolescent cancer diagnosed annually. What variables exist in childhood cancer incidence and outcome when comparing high-income countries with low-income countries? The difference in high-income versus low-income countries resides in the inability of so-called “third-world countries” to make a proper, timely diagnosis; obstacles also exist in accessing care, abandonment of treatment, lack of supportive care, and death…

Hematological manifestations of systemic disease

1 Describe the systemic diseases that have hematological manifestations. Almost all systemic conditions can result in some degree of derangement of the hematological system. Conditions associated with inflammation can cause increase in platelets, which are an acute phase reactant, as well as anemia, typically because of impaired iron metabolism. Other disruptions in nutrients critical to hematologic system can also cause derangements. In other cases, particular organ…

Bone marrow failure syndromes

1 Describe the three main pathophysiological mechanisms of aplastic anemia. a. Direct damage to bone marrow i. Typically iatrogenic because of cytotoxic drugs/chemotherapy, radiation therapy, and other myelosuppressive medications. Has also been seen with environmental toxins, including benzene (workplace toxin), now more common in low-income countries. ii. Effects are dose-dependent and often associated with spontaneous recovery. b. Immune-mediated i. Acquired aplastic anemia, when severe and acute,…

Disorders of platelets

1 What is pseudothrombocytopenia? Pseudothrombocytopenia, or spurious thrombocytopenia, is a falsely low automated platelet count that occurs secondary to in vitro platelet clumping. This is generally an incidental finding in an otherwise asymptomatic patient and can be confirmed by observation of platelet clumping on the peripheral smear. Pseudothrombocytopenia can be caused by a poorly collected specimen that has clotted, the presence of ethylenediaminetetra-acetic acid (EDTA)–dependent antibodies…

Granulocytes

1 What are the signs and symptoms suggestive of a defect in neutrophil number or function? The skin and mucous membranes are the first line of defense against infection. Once these barriers are broken, the white blood cells (WBCs), specifically neutrophils, come into play. When neutrophil counts are low or defective, the most common sites of infection are the skin and mucous membranes. Common presentations include…

Nutritional anemia

1 What is nutritional anemia? Anemia develops when a pathological decrease in the circulating red blood cell (RBC) mass reduces the oxygen-carrying capacity of blood below the physiologic requirements of the body. Anemia is clinically assessed by measuring the hemoglobin (Hb) concentration, hematocrit (packed cell volume), or RBC count in the peripheral blood. Nutritional anemia develops as a result of a deficiency of one or more…

Thalassemias

1 What is thalassemia? Normal hemoglobin (Hb) is a heterodimer of two globins produced as a result of expression of genes from the alpha (α)-globin gene cluster on chromosome 16 and two globins produced as a result of expression of genes from the beta (β)-globin gene cluster on chromosome 11. Thalassemia refers to a group of congenital disorders of globin synthesis in which there is decreased…

Intrinsic and extrinsic red blood cell defects

1 What is hemolytic anemia? Hemolytic anemia involves disorders originating from either inside the red blood cell (RBC; intrinsic) or outside of the RBC (extrinsic) that cause fragility and premature breakage and clearance of RBC. 2 What is the typical presentation for hemolytic anemia? History and examination findings Fatigue, pallor Splenomegaly Dark (tea/cola) urine Laboratory findings Low hemoglobin (Hb) Elevated reticulocyte count Elevated (indirect) bilirubin Elevated…