Hematology

• Upon completion of this problem, students should be able to describe how the structure of hemoglobin impacts on its role in oxygen transport. They should also be able to explain the genetics of autosomal recessive conditions.
• Upon completion of this problem, students should be able to explain the mechanisms of anemia.
• Upon completion of this problem, students should be able to describe hematopoiesis.
• Upon completion of this problem, students should be able to describe coagulation and mechanisms of thrombosis.
• Upon completion of this problem, students should be able to describe the role of coagulation factors in secondary hemostasis. Students should be able to assess the risk to family members of an individual with an X-linked condition.
• Upon completion of this problem, students should be able to identify and describe mechanisms of compensation and eventual failure of cardiovascular responses to sudden volume loss. Additionally, students should be able to connect and describe the consequences to and responses of other organ systems (renal, hematologic) to acute volume loss and hemodynamic compromise
• Upon completion of this case, students will be able to describe the role of tumour suppressor genes in the generation of cancers, and be able to identify the elements of pediatric oncology that are different from adult oncology.
• Upon completion of this problem, students will be able to describe the normal immune system response to infection as well as how chemotherapy can cause myelosuppression.
• Upon completion of this problem, students should be able to explain the role of platelets in hemostasis and thrombosis.
• Upon completion of this problem, students should be able to describe the role of Von Willebrand Factor in hemostasis and its function in relation to the coagulation cascade. The student should be able to describe causes of variable expression of Von Willebrand disease.
• Describe the role of tumour suppressor genes and oncogenes in carcinogenesis.
• Describe the two-hit hypothesis.
• Describe how genetic mutations can impact cell cycle regulation.
• Identify key principles in managing childhood tumours compared to adults.
• Explain the pathophysiology of chemotherapy-induced myelosuppression.
• Broadly differentiate between acute leukemias (ALL vs AML).
• Describe normal hematopoiesis (red blood cells, white blood cells, platelets).
• Describe the cellular components of a normal peripheral blood smear (complete blood count).
• Describe normal bone marrow appearance and cell composition.
• Define pancytopenia and develop a conceptual diagnostic approach to pancytopenia.
• Describe the dietary requirements for normal RBC production.
• Explain the role of iron in red blood cell production and how it changes with age.
• Explain the hemodynamic response to chronic anemia.
• Develop a conceptual approach to diagnosis of anemia, including selection of laboratory tests.
• Describe normal hemoglobin structure.
• Explain the role of hemoglobin in gas exchange and oxygen delivery.
• Describe the development of hemoglobin during embryonic, fetal, infancy and adult periods. Be able to differentiate between the different kinds of hemoglobin and briefly describe how hemoglobin electrophoresis can be used to identify the varying hemoglobins, normal and pathologic.
• Explain the mechanisms of destruction of red blood cells and hemoglobin.
• Explain the concept of quantitative versus qualitative platelet disorders.
• Develop a conceptual approach to diagnosis of thrombocytopenia.
• Describe thrombopoiesis and normal platelet physiology.
• Compare and contrast the clinical presentation of bleeding disorders caused by abnormalities of primary and secondary hemostasis.
• Explain the role of Factor VIII and other coagulation factors in normal blood coagulation.
• Develop a conceptual approach to diagnosis of bleeding disorders.
• Describe the coagulation cascade.
• Recognize the role of platelets and coagulation factors in the process of thrombosis.
• Identify the major risk factors predisposing to thrombosis.
• Explain the role of the natural anticoagulants and the fibrinolytic system.
• Describe the impact of pulmonary embolism on oxygenation and the cardiovascular system.
• Describe how the concept of resource stewardship applies to ordering diagnostic tests for pulmonary embolism.
• Describe the structures of the chest wall and the ventilatory/circulatory effect of fluid in the pleural space.
• Explain the clinical signs and symptoms associated with (acute) blood loss and shock (i.e., tachypnea, tachycardia, hypotension) and describe why they occur.
• Describe the complications (both acute and delayed) of blood transfusion.
• Describe the effects of volume loss and hypotension on renal function and explain the timing of subsequent changes.
• Describe the factors affecting red blood cell production.
• Describe treatment of iron deficiency anemia.
• Describe the difference between different kinds of hemoglobin and describe how hemoglobin electrophoresis is used to differentiate them.
• Describe the features of autosomal recessive inheritance and differentiate from autosomal dominant inheritance.
• Describe the genetic inheritance of genetic disorders of hemoglobin, with emphasis on sickle cell disease and thalassemia.
• Describe the worldwide epidemiology of sickle cell disease and thalassemia.
• Describe the medical complications of sickle cell disease and thalassemia, and develop a conceptual framework for disease modifying treatments.
• Describe the roles of von Willebrand factor.
• Develop an approach to coagulation testing: PT, PTT, fibrinogen, (basic) factor studies, (basic) vWF studies.
• Differentiate Type I, II, III vWD, both from a molecular and functional perspective.
• Differentiate plasma vs cryoprecipitate.
• Describe the features of X-linked inheritance and assess the risk to other family members.
• Describe the features of X linked inheritance and compare with the typical features of autosomal recessive and autosomal dominant inheritance patterns.
• Understand where coagulation factors are generated.
• Describe the mechanism of action of different drugs that will impact/modify the coagulation: heparin, warfarin, direct thrombin inhibitors, anti-Xa
• Describe the laboratory principles of safely transfusing blood (blood grouping, antibody screening, crossmatch).
• Describe the acid-base changes that can occur with both respiratory and renal compromise.
• Describe the response of the bone marrow to sudden blood loss.
• Define palliative and end-of-life care.
• Reflect and question our own thoughts, feelings and emotions around death, dying and bereavement.
• Explore issues of end-of-life care and the palliative approach to care.
• Review how the immune system responds to infection and explain how myelosuppression impacts the immune response.
• Upon completion of this problem, students will be able to describe the role of the lymphocyte in the immune system, develop an introductory approach to the manifestations, diagnosis, and complications of lymphoma, and will be able to describe a palliative approach to care.
• Explain what a lymphocyte is and its role in the immune system.
• Describe the different components of the lymphoid system: Waldeyer’s ring, lymph nodes, liver, spleen.
• Develop an approach to the diagnosis and pre-treatment workup of lymphoma.
• Develop a framework for categorizing and staging lymphoma.
• Identify the common clinical manifestations of hematological malignancy.
• Describe the management of tumour lysis syndrome including indications for dialysis.
• Explain the pathophysiology of thrombotic microangiopathies and specifically how platelets get consumed in the context of development of microthrombi.
• Identify the risk of contracting blood-borne infections from blood products in Canada, including some of the historical context around blood donation.
• Understand how nutritional deficiencies and antimetabolites might affect hematopoiesis and the clinical consequences.
• Develop a framework for approach to macrocytic anemias.