The Hematologic System ATI

The Hematologic System ATI Related

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A 16-year-old female patient with severe factor XI deficiency presents with acute appendicitis and requires urgent surgery. You are called by the surgeon, who wants to know what, if any, blood products or treatments are required to reduce the risk of perioperative bleeding. The patient weighs 62 kg. What should you tell him to administer?

  • A. Cryoprecipitate (five units), which will likely raise her factor XI level to 20%
  • B. Factor XI concentrate (20 units/kg), which will raise her factor XI level to 20%
  • C. Fresh frozen plasma (20 mL/kg), which will raise her factor XI level to 20%
  • D. Prothrombin complex concentrate (40 units/kg), which will raise her factor XI level to 20%
Correct Answer: C

Rationale: The correct answer is C: Fresh frozen plasma (20 mL/kg), which will raise her factor XI level to 20%. Fresh frozen plasma contains various clotting factors, including factor XI. In a patient with severe factor XI deficiency, administering fresh frozen plasma can increase factor XI levels and help reduce the risk of perioperative bleeding. The dose of 20 mL/kg is appropriate for this patient's weight of 62 kg. Cryoprecipitate (choice A) may contain factor XI but is not the optimal choice for raising factor XI levels specifically. Factor XI concentrate (choice B) would be ideal but is not commonly available, making fresh frozen plasma a more practical option. Prothrombin complex concentrate (choice D) primarily contains factors II, VII, IX, and X, but not factor XI, so it would not effectively raise factor XI levels in this patient.

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A 4-year-old child with acute lymphoblastic leukemia is receiving high-dose methotrexate during interim maintenance. He receives ondansetron and арrepitant during his stay, which control his nausea and vomiting well. These medications work by inhibiting signaling in which part of the brain?

  • A. Vestibular system
  • B. Cerebral cortex
  • C. Hypothalamus
  • D. Vomiting center
Correct Answer: E

Rationale: The correct answer is D: Vomiting center. Ondansetron and aprepitant are antiemetic drugs that work by targeting the vomiting center in the brainstem. The vomiting center coordinates the vomiting reflex in response to various stimuli, including chemotherapy drugs like methotrexate. By inhibiting signaling in the vomiting center, these medications effectively prevent nausea and vomiting. The other choices (A: Vestibular system, B: Cerebral cortex, C: Hypothalamus) are not directly involved in the control of nausea and vomiting in response to chemotherapy.

Peripheral bl. Picture is most useful in:

  • A. NHL
  • B. multiple myeloma
  • C. myelodysplastic syndrome
  • D. CML
Correct Answer: D

Rationale: The correct answer is D: CML. Peripheral blood picture is most useful in CML because it typically shows increased white blood cell count with left shift, basophilia, and presence of the Philadelphia chromosome. In contrast, A (NHL) primarily involves lymph nodes, B (multiple myeloma) shows monoclonal protein in serum/bone marrow, and C (myelodysplastic syndrome) presents with cytopenias and dysplastic changes in blood cells. Therefore, based on the specific findings seen in CML on peripheral blood, it is the most appropriate choice.

Which of the following is a key feature of Factor XIII?

  • A. Its half-life is about 10 days.
  • B. It is an important activator of thrombin.
  • C. Its levels are normal in newborns.
  • D. It is part of the contact activation system.
Correct Answer: A

Rationale: Factor XIII has a long half-life of about 10 days, allowing it to play a crucial role in stabilizing fibrin clots. This extended lifespan contributes to the maintenance of clot integrity over time. The other choices are incorrect because Factor XIII is not an activator of thrombin (B), its levels are low in newborns and gradually increase (C), and it is not part of the contact activation system (D).

Henoch Schonlein purpura is not associated with:

  • A. thrombocytopenia
  • B. palpable purpura
  • C. intussusception
  • D. acute diffuse glomerulonephritis
Correct Answer: A

Rationale: Henoch Schonlein purpura is characterized by palpable purpura, abdominal pain, arthritis, and acute diffuse glomerulonephritis. Thrombocytopenia is not typically associated with Henoch Schonlein purpura, making choice A correct. Thrombocytopenia is more commonly seen in conditions like immune thrombocytopenic purpura. Choices B, C, and D are incorrect as palpable purpura, intussusception, and acute diffuse glomerulonephritis are commonly associated with Henoch Schonlein purpura.

An 8-year-old girl presents with National Cancer Institute (NCI) Standard Risk acute pre-B-cell acute lymphoblastic leukemia. Her family history is significant for her mother having been diagnosed with breast cancer at age 34 years and a maternal uncle who developed osteosarcoma as a teenager. What cytogenetic abnormality is most likely to be detected in this patient?

  • A. t(1;19)
  • B. CRLF2 rearrangement with a JAK2 mutation
  • C. KMT24 rearrangement
  • D. Hypodiploidy with a modal chromosome number of 34
Correct Answer: D

Rationale: The correct answer is D, Hypodiploidy with a modal chromosome number of 34. In pediatric pre-B-cell acute lymphoblastic leukemia, hypodiploidy with fewer than 44 chromosomes is associated with a poor prognosis. This cytogenetic abnormality is commonly seen in cases of NCI Standard Risk acute pre-B-cell ALL.

A: t(1;19) is typically associated with T-cell ALL, not pre-B-cell ALL.
B: CRLF2 rearrangement with a JAK2 mutation is more commonly seen in high-risk B-cell ALL.
C: KMT24 rearrangement is not a recognized cytogenetic abnormality in ALL.

In summary, the presence of hypodiploidy with a modal chromosome number of 34 is the most likely cytogenetic abnormality in this patient based on her clinical presentation and family history.

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