A 7-year-old boy presents with recent onset of vomiting and lethargy. Blood smear shows increased neutrophils with a left shift and 8% abnormal cells. Bone marrow contains 60% of the same cells. Flow cytometry shows that the cells are TdT–, CD10+, CD19+, CD20+, sIg+. What is the most likely diagnosis?

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ATI Hematologic System Test Questions

Question 1 of 5

A 7-year-old boy presents with recent onset of vomiting and lethargy. Blood smear shows increased neutrophils with a left shift and 8% abnormal cells. Bone marrow contains 60% of the same cells. Flow cytometry shows that the cells are TdT–, CD10+, CD19+, CD20+, sIg+. What is the most likely diagnosis?

Correct Answer: A

Rationale: The correct answer is A: Burkitt leukemia/lymphoma. This diagnosis is supported by the presence of abnormal cells that are CD10+, CD19+, CD20+, sIg+, which are characteristic of Burkitt lymphoma. The absence of TdT– indicates that it is not T-cell ALL. B-cell ALL would typically show TdT positivity. Hematogones are immature B-cell precursors commonly seen in the bone marrow of healthy children and do not typically present with the described clinical picture. The high percentage of abnormal cells in both blood and bone marrow, along with the specific immunophenotype, point towards Burkitt leukemia/lymphoma.

Question 2 of 5

A study is designed to investigate the rates of central line–associated blood stream infections among pediatric hematology/oncology patients. Three common central line types (totally implanted catheter [port], peripherally inserted central catheter [PICC], and tunneled externalized catheter [TEC]) were included in the study. What data structure is central line type?

Correct Answer: C

Rationale: The correct answer is C: Nominal. Central line type is a nominal data structure because it represents categories without any inherent order or ranking. In this study, the central line types (port, PICC, TEC) are distinct categories with no numerical value or order. It's important to distinguish between the different types of central lines, but they cannot be ordered in a meaningful sequence, making them nominal. Incorrect answers: A: Continuous - Central line type does not have a continuous range of values, it is categorical. B: Dichotomous - Dichotomous data structure has only two categories, while central line type has more than two distinct categories. D: Ordinal - Ordinal data structure involves categories with a clear order or ranking, which is not applicable to central line types as they do not have a natural order.

Question 3 of 5

You receive a phone call from a community pediatrician who is caring for a 2-year-old toddler with a cancer predisposition syndrome. The pediatrician describes a child at the 95th percentile for height and weight with a history of corrective oral surgery to reduce a large tongue and a history of an omphalocele in infancy. The pediatrician is currently performing ultrasound of the abdomen and laboratory evaluation for this patient every 3 months. Which tumor is this patient most at risk of developing?

Correct Answer: D

Rationale: The correct answer is D: Nephroblastoma (Wilms tumor). This patient likely has Beckwith-Wiedemann syndrome given the features of overgrowth, macroglossia, and omphalocele. Beckwith-Wiedemann syndrome is associated with an increased risk of Wilms tumor. Wilms tumor is the most common renal malignancy in childhood. Monitoring for Wilms tumor with ultrasound and laboratory evaluations is appropriate due to the increased risk in this patient population. Choice A: Pleuropulmonary blastoma is a rare lung tumor more commonly seen in children under 2 years old. It is not typically associated with Beckwith-Wiedemann syndrome. Choice B: Hepatocellular carcinoma is a primary liver cancer more commonly seen in adults, not children with Beckwith-Wiedemann syndrome. Choice C: Cystic nephroma is a benign kidney tumor typically seen in young children, but it is not associated with Beckwith-Wiedemann syndrome nor is it malignant

Question 4 of 5

A 2-month-old infant is brought to your clinic with an extensive scaly rash on the scalp, which has been biopsied and shown to be Langerhans cell histiocytosis (LCH). You want to determine whether this patient has skin-only LCH or involvement of any of the 'high-risk' organs. The child has a normal CBC; normal liver enzymes and bilirubin; and a normal skeletal survey, skull films, and chest X ray. What other screening test will be important for finding involvement of a high-risk organ?

Correct Answer: D

Rationale: The correct answer is D: Serum albumin and total protein. In Langerhans cell histiocytosis (LCH), involvement of high-risk organs like the liver, spleen, and bone marrow can occur. Serum albumin and total protein levels can indicate liver involvement as hypoalbuminemia and hypoproteinemia can be seen in liver dysfunction. The other choices, A (Reticulocyte count), B (Erythrocyte sedimentation rate), and C (Alkaline phosphatase) are not specific for evaluating high-risk organ involvement in LCH. Reticulocyte count is related to red blood cell production, ESR is a nonspecific marker of inflammation, and alkaline phosphatase is commonly elevated in various conditions, not specific for high-risk organ involvement in LCH.

Question 5 of 5

A 9-month-old boy has been referred to you for the evaluation of an enlarged abdomen. Imaging studies show a large liver mass (PRETEXT III). Alfa-fetoprotein is 98 ng/mL, and a CT scan of the lungs show bilateral lung metastases. A needle biopsy is performed, and you are planning to review the specimen with the pathologist. Which of the following diagnoses are you suspecting?

Correct Answer: D

Rationale: The correct answer is D: Small cell undifferentiated hepatoblastoma. In hepatoblastoma, the presence of lung metastases indicates a high-risk tumor. The small cell undifferentiated subtype is more aggressive with a poorer prognosis compared to other subtypes. The AFP level is lower than typically seen in hepatoblastoma, but still within the range for this diagnosis. Pure fetal histology hepatoblastoma (choice A) is less common and usually associated with a better prognosis. Embryonal sarcoma of the liver (choice B) is a distinct entity with different histological features. Fibrolamellar hepatocellular carcinoma (choice C) typically occurs in older children and has a different imaging appearance.

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