An infant is born with a 7 cm × 6 cm lesion over the upper extremity from the elbow to the shoulder. The lesion is indurated and purpuric, with some petechiae around the edges. No other areas of petechiae are noted on the skin. The infant is doing well without other systemic problems. i were 9 and 9. You are called by the pediatric nurse practitioner to the NICU. What is the most appropriate next step?

Correct Answer: D

Rationale: The correct next step is D: Obtain labs, including a CBC with platelet count and fibrinogen. This is the most appropriate action because the infant presents with an indurated and purpuric lesion with petechiae, suggesting a possible coagulation disorder. Labs can help assess for any underlying hematologic abnormalities. Choice A is incorrect because it is important to investigate further given the concerning presentation. Choice B is not the best next step as an ultrasound would not provide information on the hematologic status. Choice C is also not the best option as an MRI is not typically used to assess coagulation disorders.

Correct Answer: B

Rationale: Correct Answer: B Rationale: 1. Autologous hematopoietic stem cell transplantation should be used when it can provide a meaningful survival benefit over chemotherapy. 2. The most common cancers it is used for include neuroblastoma, responsive brain tumors in young children to avoid/minimize early radiation therapy, and relapsed lymphoma. 3. This choice aligns with the current guidelines and evidence-based practice in utilizing autologous stem cell transplantation for specific cancer types to improve outcomes. Summary of Other Choices: A: Incorrect. It is not necessary to use autologous stem cell transplantation solely for high-dose therapy. The listed cancers are not the most common indications for this procedure. C: Incorrect. The statement about the graft-versus-tumor effect is misleading, and the listed cancers are not the most common ones treated with autologous stem cell transplantation. D: Incorrect. Autologous stem cell transplantation is not used to shorten treatment or avoid extensive chemotherapy. The listed cancers

Correct Answer: B

Rationale: The correct answer is B: Leukocyte adhesion deficiency (LAD) Type II. This diagnosis is supported by the patient's history of recurrent bacterial infections, leukocytosis, neutrophilia, and the Bombay blood group. LAD Type II is characterized by a defect in the CD18 integrin molecules, leading to impaired leukocyte adhesion and migration to sites of infection. The Bombay blood group is due to the absence of H antigen, which is also seen in LAD Type II. A: Chediak-Higashi syndrome is characterized by recurrent infections, albinism, and peripheral neuropathy, not leukocyte adhesion deficiency. C: CD18 deficiency is actually another term for LAD Type I, not LAD Type II. D: Griscelli syndrome presents with silvery hair and immunodeficiency, but does not involve leukocyte adhesion deficiency.

Correct Answer: C

Rationale: The correct answer is C because T-cell numbers in infants are indeed higher than in adults. This is due to the active development of the immune system in infants, leading to a higher proportion of T-cells. A is incorrect because NK-cell numbers in infants are actually higher at birth and decrease with age. B is incorrect because B-cell numbers are lower at birth and increase with age. D is incorrect because infants do not have low lymphocyte counts; their immune system is actively developing, leading to higher lymphocyte counts compared to adults.

Correct Answer: D

Rationale: The correct answer is D because the addition of cyclophosphamide and etoposide to the chemotherapy regimen is indicated for patients with combined loss of heterozygosity for 1p and 16q in Wilms' tumor. This genetic abnormality is associated with a higher risk of relapse and poorer outcomes. The extended duration of chemotherapy (33 weeks) is necessary to target any remaining tumor cells and reduce the risk of recurrence. Additionally, the decision to administer radiation to both the lungs and flank is appropriate given the incomplete resolution of some lung nodules after initial chemotherapy. Lung radiation helps to target any remaining metastatic lesions, while flank radiation targets the primary tumor site and positive lymph nodes. This comprehensive treatment approach aims to maximize the chances of long-term disease control and survival for the patient. Choice A is incorrect because it lacks the addition of cyclophosphamide and etoposide, which are needed for Wilms' tumor with the specific genetic abnormality mentioned.