Page 259 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

13 Bone Marrow Failure Syndromes

CHAPTER

KEY TERMS

aplastic anem ia dyserythropoietic anem ia pancytopenia

congenital erythroblastopenia pathophysiology
constitutional aplastic anem ia Fanconi’s anem ia telom eres

Diam ond-Blackfan anem ia iatrogenic

LEARNING OUTCOMES

Bone marrow failure syndromes ■ Describe the laboratory features of familial aplastic anemia.

■ Describe general characteristics of bone marrow syndromes. ■ Describe characteristics of dyskeratosis congenita.

■ Name a variety of diagnoses associated with cytopenias with hypo- Bone marrow failures involving a single-cell
cellular bone marrow. lineage

■ De ne the term pancytopenia.
■ Name three examples of pure red cell aplasia.
Acquired aplastic anemia Compare acute, acquired, and congenital examples of red cell

■ Describe the major characteristics of acquired aplastic anemia. aplasia.

■ De ne the term iatrogenic. Describe the characteristics of Diamond-Blackfan syndrome, includ-

■ List at least three iatrogenic substances that can cause acquired ing the nature of the defect.
aplastic anemia. Name four types of congenital dyserythropoietic anemia.

■ Name at least four types of viral infection that have been associated Describe the characteristics of congenital dyserythropoietic
with acquired aplastic anemia. anemia.

■ Brie y describe how the immune process causes acquired aplastic ■ Explain the laboratory ndings in congenital dyserythropoietic
anemia. anemia.

Explain the three phases of development of acquired aplastic ■ Describe the characteristics of severe congenital neutropenia and

anemia. cyclic neutropenia.

■ Describe the clinical features of acquired aplastic anemia. ■ Explain the characteristics of Shwachman-Diamond syndrome.

■ Discuss the laboratory ndings in acquired aplastic anemia. ■ Describe characteristics of congenital amegakaryocytic

■ Explain the impact of telomere on hematopoietic failure. thrombocytopenia.

■ Explain how the laboratory ndings in acquired aplastic anemia ■ Compare three types of congenital amegakaryocytic
manifest themselves after acute radiation exposure. thrombocytopenia.

Discuss the role of bone marrow transplantation and other modes of Case studies

treatment of acquired aplastic anemia.
Analyze the patient history, clinical signs and symptoms, and labo-

Constitutional bone marrow failure syndromes ratory data for the stated case studies, answer the related critical

associated w ith aplastic anemia thinking questions, and conclude the most likely diagnosis.

Describe the mode of inheritance of Fanconi’s anemia.

■ Explain the clinical signs and symptoms of Fanconi’s anemia. NOTE:
■ Name one treatment for Fanconi’s anemia. ■ indicates MLT and MLS core content

Compare the relationship between familial aplastic anemia and indicates MLT (optional) and MLS advanced content

Fanconi’s anemia.

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