Anatomy of Hematologic System

Anatomy of Hematologic System Related

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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: Rationale for correct answer A:
1. Both parents must be carriers for the child to have alpha thalassemia.
2. The child has an abnormal complete blood count, indicating a hemoglobinopathy.
3. The child inherited a trans-deletion genotype alpha thalassemia trait from both parents.
4. Silent carriers have no symptoms but can pass on the trait.

Summary for incorrect choices:
B: In cis deletion, both parents would have to pass on the trait, which is not the case here.
C: Spontaneous mutation is unlikely for alpha thalassemia.
D: Both parents having cis deletions would result in a more severe form of alpha thalassemia.

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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 iron deficiency anemia, dysphagia, and esophageal webs. Clubbing is not a typical feature of Plummer Vinson syndrome. Angular stomatitis (A), splenomegaly (B), and post cricoid web (D) are commonly associated with Plummer Vinson syndrome due to chronic iron deficiency anemia. Clubbing is more commonly seen in conditions such as chronic respiratory or cardiac diseases, not in Plummer Vinson syndrome.

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 laboratory finding of granulocytosis

  • A. Complete absence of peripheral blood granulocytes and their precursors in the bone marrow
  • B. Acute bacterial infections
  • C. Myeloproliferative disorders
  • D. Increased numbers of granulocytes in blood and bone marrow
Correct Answer: D

Rationale: The correct answer is D because granulocytosis refers to an increased number of granulocytes in both blood and bone marrow. This finding is commonly associated with conditions such as infections, inflammation, and certain hematologic disorders. Other choices are incorrect because A describes a condition of granulocytopenia, B is associated with acute bacterial infections but not specific to granulocytosis, and C refers to myeloproliferative disorders which may involve other cell types besides granulocytes.

A 10-year-old girl is a long-term survivor of type II pleuropulmonary blastoma (PPB). You suspect she has a cancer predisposition syndrome and perform genetic testing, which confirms she has DICER1 syndrome. Which other cancer is she predisposed to?

  • A. Papillary thyroid cancer
  • B. Medullary thyroid cancer
  • C. Pheochromocytoma
  • D. Renal cell carcinoma
Correct Answer: A

Rationale: The correct answer is A: Papillary thyroid cancer. DICER1 syndrome is associated with an increased risk of developing various tumors, including papillary thyroid cancer. This is due to the role of DICER1 gene mutation in dysregulating cell growth and division. Other choices like B: Medullary thyroid cancer, C: Pheochromocytoma, and D: Renal cell carcinoma are not typically associated with DICER1 syndrome. Medullary thyroid cancer is more commonly linked to RET gene mutations, pheochromocytoma to RET or SDHB/C/D gene mutations, and renal cell carcinoma to VHL gene mutations. Therefore, based on the genetic mechanism and known associations, papillary thyroid cancer is the most likely cancer this patient is predisposed to.

The nurse is documenting findings after completing data collection with a patient. What term should the nurse use to document a large area of discoloration from hemorrhage under the skin?

  • A. Pallor
  • B. Rubor
  • C. Petechiae
  • D. Ecchymosis
Correct Answer: D

Rationale: The correct term to document a large area of discoloration from hemorrhage under the skin is ecchymosis. Ecchymosis refers to a bruise caused by the extravasation of blood into the subcutaneous tissues. Pallor (A) is paleness, rubor (B) is redness, and petechiae (C) are tiny, pinpoint-sized red or purple spots on the skin due to bleeding. Ecchymosis is the most appropriate term for describing a large area of discoloration from hemorrhage under the skin as it specifically denotes a bruise resulting from blood leakage into tissues.

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