Hematology Oncology Take Home Quiz Answers 1. In which forms of anemia can basophilic stippling be seen? More common in severe lead intoxication, but can also be seen in thalassemia or Fe deficiency. 2. What is the most common form of genetically determined hemolytic anemia in: A. White population: Hereditary sherocytosis B. Black males: G6PD deficiency (In contrast to Zitelli, uptodate mentions that while pyruvate kinase deficiency is less common, it may be more severe and comes to clinical attention more commonly than G6PD.) 3. Describe the consequences of an infant or toddler consuming cow’s milk as the primary source of nutrition. The iron in cow’s milk is not very bioavailable. If intake is excessive, the patient is drinking milk in lieu of other iron-rich foods. Severe iron deficiency anemia occurs with associated signs/symptoms. Also, if there is any degree of intestinal sensitivity to the cow’s milk, chronic inflammation leads to protein-losing enteropathy. This may lead to anasarca. 4. Compare and contrast Diamond Blackfan Anemia and Transient Erythroblastopenia of Childhood: DBA TEC Age Infancy 1-3y/o Inherited? Inherited? Acquired Antecedent illness No Viral illness Abnormal facies/anomalies Yes: 25-50% No RBC Adenosine Deaminase High Normal MCV High Normal (?high in recovery) Hgb F High Normal (?high in recovery 5. Give the name of the hematological syndrome which may be associated with a giant hemangioma. List the constellation of associated findings. Kasabach-Merritt syndrome: consumptive coagulopathy, Hypofibrinogenemia, elevated degredation products, microangiopathic fragmentation of red cells, thrombocytopenia. 6. Compare and contrast the clinical presentation of a child with a disorder of platelets vs a child with a coagulopathy. Disorder of platelets – mucocutaneous bleeding or purpura, petechiae or ecchymoses Coagulopathy – bleeding into deep tissue or joints Both may present with hematuria, guaiac-positive stools, menorrhagia or bleeding in the CNS 7. What are the accepted treatments for the following? A. Immune Thrombocytopenia – IVIG, Anti-D antibody B. Isoimmune thrombocytopenia in the newborn – washed maternal platelets and IVIG C. Hemophilia A or B – Factor replacement D. Von Willebrand Disease – DDAVP, von Willebrand factor replacement through transfused donor blood products 8. Describe the bone marrow findings in the following conditions. A. Immune thrombocytopenia – normal to increased numbers of megakaryocytes and no evidence of abnormal cells B. Aplastic anemia – stromal marrow cells with no hematopoietic elements C. Leukemia – marrow replacement with blast cells and “crowding out” of normal marrow cells D. Hemophagocytic lymphohistiocytosis – hemophagocytosis may be visualized 9. Name the physical exam findings and 2 paraneoplastic syndromes that may be found in a patient with neuroblastoma. Describe the findings in the 2 paraneoplastic syndromes. Physical Exam Findings – Abdominal distention/mass, FTT, weight loss, anorexia, UTI from obstruction, Horner’s syndrome, Paralysis and/or weakness (Invasion of spinal cord or peripheral nerves), cerebellar ataxia, nodular skin lesion, raccoon eyes, heterochromia (occasionally) Paraneoplastic syndromes – 1. opsoclonus-myoclonus describes random eye movements and myoclonic jerking. 2. Kerner-Morrison syndrome – intractable secretory diarrhea, hypokalemia, dehydration. 10. Describe the bone changes in the following. A. ALL – metaphyseal lucencies, growth arrest lines, osteopenia, lytic bone lesions B. Osteosarcoma – metaphyseal tumor involving the bones exhibiting the most rapid growth in adolescence (femur, tibia, humerus) C. Ewing sarcoma – diaphyseal but may also arise from soft tissue