ATI RN
The Hematologic System ATI Questions
Question 1 of 5
A 4-year-old boy is pale with intermittent jaundice and splenomegaly. Laboratory results are as follows: RBC 4.85 M/mcL (N); Hgb 8.6 g/dL (L); Hct 25.8% (L); MCV 81.6 (N); MCHC 38% (H); RDW 20% (H); Retic 7% (H). What are the two best tests to distinguish autoimmune hemolytic anemia from hereditary spherocytosis?
Correct Answer: E
Rationale: Failed to generate a rationale of 500+ characters after 5 retries.
Question 2 of 5
An 18-year old male patient presents with bruising, fatigue, and diffuse extremity pain. He is noted to be tachypneic and hypoxic and has a diffuse interstitial infiltrate on chest x-ray. CBC reveals a WBC count of 285,000/mm3 (85% myeloblasts, with monocytic morphology), hemoglobin of 7.9 g/dL, and platelet count of 36,000/mm3. What is the most likely cause of the infiltrate and respiratory symptoms and the most appropriate initial treatment?
Correct Answer: B
Rationale: The correct answer is B: Hyperleukocytosis; leukapheresis or manual exchange transfusion and initiation of induction chemotherapy. In this scenario, the patient's symptoms and lab findings are consistent with acute myeloid leukemia (AML) with leukostasis, causing hypoxia and interstitial infiltrates. Leukapheresis or manual exchange transfusion is crucial to rapidly reduce the high white blood cell count, which can help alleviate symptoms and prevent complications like tissue hypoxia. Initiation of induction chemotherapy is also essential for long-term management of AML. Rationale for why other choices are incorrect: A: Hyperleukocytosis alone without leukostasis does not typically require immediate leukoreduction, as in this case. Induction chemotherapy should be initiated promptly to address the underlying AML. C: COVID-19 infection would not typically present with such profound leukocytosis and monocytic morphology. Convalescent plasma and prednisone are not
Question 3 of 5
You are consulting on a 10-year-old male with severe persistent neutropenia, a history of recurrent infections, and warts. The rest of the peripheral blood count is normal. His mother also has neutropenia. Bone marrow examination shows a hypercellular marrow and retained myeloid cells with vacuolated cytoplasm. There are no abnormalities in the red cells or platelet precursors. Cytogenetics are 46XY. You start granulocyte colony stimulating factor therapy and the neutrophil count increases. A mutation in which of the following genes is most likely to have caused this familial inherited bone marrow failure syndrome?
Correct Answer: A
Rationale: The correct answer is A: CXCR4. In this case, the patient presents with severe neutropenia, recurrent infections, and warts, suggestive of WHIM syndrome, where CXCR4 mutations are often involved. CXCR4 plays a crucial role in immune cell trafficking and retention in the bone marrow. The hypercellular marrow and vacuolated myeloid cells are consistent with WHIM syndrome. The absence of abnormalities in red cells or platelet precursors rules out other syndromes. Mutations in ELANE are commonly associated with congenital neutropenia, not familial inherited bone marrow failure syndromes. GATA2 mutations are linked to familial myelodysplastic syndromes, not typically presenting with neutropenia and warts. Mitochondrial DNA mutations are more related to mitochondrial disorders, which usually manifest with multi-system involvement, not specific to bone marrow failure syndromes.
Question 4 of 5
Several gene mutations have been associated with juvenile myelomonocytic leukemia (JMML), and they may or may not have prognostic implications. A gene expression–based classification system has been found to be an independent predictor of clinical outcome in these patients. What is the disease signature that predicts a poor outcome?
Correct Answer: B
Rationale: The correct answer is B: Acute myeloid leukemia-like. In JMML, a disease signature resembling acute myeloid leukemia (AML) has been associated with a poor outcome. This signature includes features such as increased blasts, abnormal karyotypes, and mutations in genes like NRAS and KRAS. AML-like JMML cases often have aggressive disease progression and poorer response to treatment. Tyrosine kinase inhibitors (choice A) are not typically used in JMML treatment. Chronic myeloid leukemia-like (choice C) is not associated with a poor outcome in JMML. BRAF pathway abnormalities (choice D) may be present in some cases of JMML but are not the primary disease signature predicting poor outcomes.
Question 5 of 5
You have been asked to see a 15-year-old girl who is being referred for evaluation of an ovarian mass. Her history is also significant for secondary amenorrhea, and physical examination shows signs of virilization. As you review her family history, what syndrome will you consider?
Correct Answer: B
Rationale: The correct answer is B: DICER-1 syndrome. This syndrome is associated with ovarian tumors, secondary amenorrhea, and signs of virilization due to androgen-secreting tumors. Li-Fraumeni syndrome (A) is characterized by multiple primary cancers but not specifically ovarian masses. Turner syndrome (C) presents with ovarian dysgenesis and primary amenorrhea. Beckwith-Wiedemann syndrome (D) is associated with overgrowth and abdominal wall defects, not ovarian masses. In this case, the presence of ovarian mass, secondary amenorrhea, and virilization point towards DICER-1 syndrome.