RBC Diseases - Module 1

A 6-year-old girl is brought into the emergency room after

an automobile accident. Physical examination shows bleeding

from multiple wounds, and a CBC reveals a normocytic,

normochromic anaemia. Which of the following indices is most

helpful in defining this patient’s anaemia as normocytic?

• Haematocrit

• Haemoglobin

• Red blood cell count

• Mean corpuscular haemoglobin concentration

• Mean cell volume

Which group is at highest risk for developing iron deficiency anaemia?

• Post-menopausal women

• Children over 10 years

• Pregnant women

• Elderly men

Another name for sickle cell is:

• Acanthocyte

• Drepanocyte

• Echinocyte

• Dacrocyte

• Spherocyte

Anaemia of chronic disease is characterised by:

• Increased erythropoietin activity

• Decreased iron stores

• Elevated hepcidin levels

• Increased ferritin with low iron

In hereditary spherocytosis, haemolysis is;

• Predominantly extravascular

• Predominantly intravascular

• Both intravascular and extravascular

• Contained within the bone marrow

Aplastic anaemia most likely results from:

• Genetic predisposition

• Immune-mediated stem cell destruction

• Globin-chain imbalance

• Iron sequestration

A 33-year-old man presents with episodes of dark-colored urine in the morning and increasing fatigue. He also reports shortness of breath on exertion and has had two episodes of deep vein thrombosis in the past year. Laboratory evaluation reveals:

• Haemoglobin: 9.2 g/dL

• Lactate dehydrogenase (LDH): elevated

• Haptoglobin: undetectable

• Direct Coombs test: negative

• Flow cytometry: decreased expression of CD55 and CD59 on red blood cells

Which of the following best explains the pathophysiology of this patient’s condition?

• Autoantibody-mediated destruction of RBCs

• Impaired synthesis of haemoglobin chains

• Defective cytoskeletal proteins in red cell membrane

• Complement-mediated lysis due to deficiency of GPI-anchored proteins

• Deficiency of glucose-6-phosphate dehydrogenase

Hepcidin downregulates iron absorption by:

• Stimulating transferrin production

• Inhibiting ferroportin

• Enhancing DMT-1 expression

• Increasing ferritin synthesis and storage

• Enhancing Vitamin C activity

Which of the following best describes mean corpuscular haemoglobin concentration (MCHC)?

• The average amount of haemoglobin per red blood cell

• The percentage of red blood cells in a given volume of whole blood

• The concentration of haemoglobin in a given volume of packed red blood cells

• The total mass of haemoglobin in the blood divided by the number of red blood cells

• The average size of red blood cells

Which of the following best describes the primary pathophysiological mechanism of sickle cell disease?

• Autoimmune destruction of red blood cells

• Defective haem synthesis due to iron deficiency

• Abnormal globin chain production due to a beta-globin gene mutation

• Impaired DNA synthesis due to folate defciency

• Decreased HbF production