Select a Community
Are you sure you want to trigger topic in your Anconeus AI algorithm?
You are done for today with this topic.
Would you like to start learning session with this topic items scheduled for future?
Anemia of chronic disease
26%
13/50
Infection with Ebstein-Barr virus
18%
9/50
Mutation of ankyrin
32%
16/50
Rheumatoid arthritis
Substitution of glutamic acid with valine at the beta chain
6%
3/50
Select Answer to see Preferred Response
This patient’s profession (e.g., kindergarten teacher at risk for parovirus B19 infection), symptoms of anemia (e.g., extreme fatigue, pallor, and palpitations), and past symptoms (e.g., joint pain and viral-like illness) suggest aplastic crisis secondary to parovirus B19 infection in the setting of hereditary spherocytosis. Hereditary spherocytosis is due to mutations of ankyrin and spectrin. Hereditary spherocytosis is due to defects in proteins that interact with the red blood cell (RBC) membrane skeleton and plasma membrane (e.g., ankyrin, band 3, protein 4.2, and spectrin). Defects in these proteins lead to small, round RBCs that are prematurely removed by the spleen, thus leading to splenomegaly and extravascular hemolysis. Laboratory findings will demonstrate increased mean corpuscular hemoglobin concentration and positive osmotic fragility test. As patients have increased bone-marrow stress, infection with parovirus B19 leads to aplastic crisis or reticulocytopenia. Treatment is with blood transfusion. Incorrect Answers: Answer 1: Anemia of chronic disease is incorrect as this patient does not have any obvious chronic disease. Though her joint pain may suggest rheumatoid arthritis (RA), infection with parovirus b19 may also present similarly. In addition, joint pains secondary to rheumatoid arthritis would not self-resolve. Answer 2: Infection with Ebstein-Barr virus (EBV) is probable as she had viral-like symptoms and splenomegaly. However, she lacks other symptoms (e.g., lymphadenopathy, pharyngitis, or fever). Her symptoms of anemia are also not explained by EBV infection. Answer 4: Rhuematoid arthritis is an autoimmune condition that presents with symmetric joint pain/stiffness that is often worse in the morning. The patient reports similar symptoms in the past that self-resolved, making RA less likely. It is worth nothing that parovirus B19 infection may present similarly. Answer 5: Substitution of glutamic acid with valine at the beta chain is the pathogenesis of sickle cell disease, which can also present with aplastic crisis in the setting of parovirus B19 infection. However, sickle cell patients would likely have an extensive past medical history at the age of 29. Bullet Summary: Hereditary spherocytosis is due to genetic mutations in red blood cell proteins such as ankyrin and spectrin.
2.2
(26)
Please Login to add comment