Anatomy of Hematologic System

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You have a new patient consult in clinic this morning. The referral packet includes the newborn screen report, which is flagged abnormal hemoglobinopathy screen, F, A, Bart's, refer to hematology, and a complete blood count done at 4 years of age with a hemoglobin of 10 g/dL and an MCV of 68. The pediatrician has informed the parents the child has some form of alpha thalassemia. The older brother had the same newborn screen results but had a normal complete blood count when checked. The mother wants to know why her second child has an abnormal complete blood count when she and her husband do not have any blood problems. How would you respond to the child's mother?

  • A. The mother and father are both silent carriers and each passed a deleted alpha globin allele to their child. The child inherited a trans-deletion genotype alpha thalassemia trait.
  • B. The mother has cis deletion alpha thalassemia and the father has no alpha globin deletion, giving the child alpha thalassemia trait.
  • C. Neither parent has an alpha globin deletion; this was a new spontaneous mutation causing alpha thalassemia in the child.
  • D. Both parents carry cis deletions in the alpha globin gene cluster.
Correct Answer: A

Rationale: The correct answer is A because both parents are silent carriers, each passing a deleted alpha globin allele to their child, resulting in a trans-deletion genotype alpha thalassemia trait. This explanation aligns with the child's abnormal complete blood count and the family history.

Choice B is incorrect because it states that the mother has cis deletion alpha thalassemia, which is not supported by the information provided.

Choice C is incorrect as it suggests a new spontaneous mutation causing alpha thalassemia in the child, which is not consistent with the genetic inheritance pattern described.

Choice D is incorrect because it claims both parents carry cis deletions in the alpha globin gene cluster, which contradicts the scenario where the parents are silent carriers.

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Plummer Vinson syndrome is not associated with:

  • A. angular stomatitis
  • B. splenomegaly
  • C. clubbing
  • D. post cricoid web
Correct Answer: C

Rationale: The correct answer is C: clubbing. Plummer Vinson syndrome is characterized by the triad of dysphagia, iron deficiency anemia, and esophageal webs. Clubbing is not a typical feature of this syndrome. Angular stomatitis (A), splenomegaly (B), and post cricoid web (D) are all associated with Plummer Vinson syndrome. Angular stomatitis is due to iron deficiency anemia, splenomegaly can be present due to chronic anemia, and post cricoid web is a characteristic finding in the esophagus of patients with this syndrome.

A 14-year-old male patient is diagnosed with very high risk acute lymphoblastic leukemia and is likely going to require an allogeneic hematopoietic stem cell transplant to cure his leukemia. Prior to going to transplant, he is likely to require multiple blood transfusions. Which of the following products or component modifications is the best way to prevent him from developing alloimmunization due to anti-HLA antibodies prior to transplant?

  • A. Frozen RBCs
  • B. Volume-reduced blood products
  • C. Irradiation of all blood products
  • D. Leukoreduced blood products
Correct Answer: D

Rationale: The correct answer is D: Leukoreduced blood products. Leukoreduction removes white blood cells, which are a major source of HLA antigens that can trigger alloimmunization. By using leukoreduced blood products, the risk of developing anti-HLA antibodies is significantly reduced, thus minimizing the potential for alloimmunization prior to transplant.

A: Frozen RBCs - Freezing does not eliminate HLA antigens and does not prevent alloimmunization.
B: Volume-reduced blood products - Reducing volume does not impact HLA antigens and thus doesn't prevent alloimmunization.
C: Irradiation of all blood products - While irradiation prevents graft-versus-host disease, it does not directly prevent the development of anti-HLA antibodies.

Koilonychias is a specific feature of

  • A. Hemolytic anemia
  • B. Sickle cell disease
  • C. Thalassemia major
  • D. Iron deficiency anemia
Correct Answer: D

Rationale: Step-by-step rationale for why the correct answer is D (Iron deficiency anemia):
1. Koilonychias is characterized by spoon-shaped nails, a classic sign of iron deficiency anemia due to changes in nail structure.
2. Iron deficiency leads to decreased production of keratin, causing nail deformities like koilonychias.
3. Hemolytic anemia (choice A) and Sickle cell disease (choice B) are not typically associated with koilonychias.
4. Thalassemia major (choice C) can cause nail changes, but koilonychias is more commonly seen in iron deficiency anemia.

Increase Fe & normal TIBC are found in:

  • A. thalassemia major
  • B. haemosidrosis
  • C. rheumatoid arthritis
  • D. dissiminated malignancy
Correct Answer: C

Rationale: The correct answer is C: rheumatoid arthritis. In rheumatoid arthritis, chronic inflammation can lead to anemia of chronic disease, causing increased levels of serum iron (Fe) due to decreased iron utilization and normal Total Iron Binding Capacity (TIBC). Thalassemia major (A) would show decreased TIBC due to iron overload. Haemosiderosis (B) would show increased Fe and TIBC due to iron overload. Disseminated malignancy (D) may show decreased Fe and TIBC due to anemia of chronic disease or other factors.

Waldeyer's ring does not include:

  • A. faucal tonsils
  • B. submandibular glands
  • C. adenoids
  • D. lingual tonsils
Correct Answer: B

Rationale: The correct answer is B: submandibular glands. Waldeyer's ring consists of a ring of lymphoid tissue in the pharynx, including the palatine tonsils (faucal tonsils), adenoids, and lingual tonsils. Submandibular glands are salivary glands located beneath the mandible and are not part of Waldeyer's ring. Therefore, B is the correct answer. Choices A, C, and D are incorrect because they are all components of Waldeyer's ring.

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