Anatomy of Hematologic System

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A 2-year-old girl has a diagnosis of overall stage IV favorable histology Wilms' tumor with pulmonary metastases and local stage III disease due to finding positive lymph nodes. After she completes 6 weeks of vincristine/dactinomycin/doxorubicin (DD4A) chemotherapy, restaging shows complete resolution of some but not all lung nodules. Tumor genetic testing reveals combined loss of heterozygosity for 1p and 16q. Which of the following would be the most appropriate treatment plan?

  • A. Continue chemotherapy with vincristine, doxorubicin, and dactinomycin to complete 25 weeks of therapy. Administer radiation to lungs and flank.
  • B. Continue chemotherapy with vincristine, doxorubicin and dactinomycin to complete 25 weeks of therapy. Radiation to flank only. No lung radiation.
  • C. Continue chemotherapy with vincristine, doxorubicin and dactinomycin, add cyclophosphamide and etoposide to complete 33 weeks of therapy. Radiation to flank only. No lung radiation.
  • D. Continue chemotherapy with vincristine, doxorubicin and dactinomycin, add cyclophosphamide and etoposide to complete 33 weeks of therapy. Radiation to lungs and flank.
Correct Answer: D

Rationale: The correct answer is D because the patient has residual lung nodules after initial chemotherapy, indicating a need for intensified treatment. Adding cyclophosphamide and etoposide to the chemotherapy regimen can improve outcomes in patients with combined loss of heterozygosity for 1p and 16q. Radiation to both the lungs and flank targets all remaining disease sites effectively.

Choice A is incorrect because it does not address the need for intensified treatment with additional agents or radiation to the lungs, where residual disease remains.

Choice B is incorrect as it only includes radiation to the flank and not the lungs, which are still harboring disease.

Choice C is incorrect because although it adds cyclophosphamide and etoposide, it does not include radiation to the lungs, which is necessary for comprehensive disease control.

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The nurse is assisting with the preparation of a blood transfusion for a patient. Which type of fluid should the nurse select to transfuse with the blood?

  • A. 0.9% normal saline
  • B. Dextrose 5% and water
  • C. Dextrose 5% and 0.9% normal saline
  • D. Dextrose 5% and 0.45% normal saline
Correct Answer: A

Rationale: Rationale: A 0.9% normal saline solution is the correct choice for blood transfusion because it is isotonic, which closely matches the osmolarity of blood. This helps prevent hemolysis and maintains the integrity of red blood cells during transfusion. Other choices such as B (dextrose 5% and water), C (dextrose 5% and 0.9% normal saline), and D (dextrose 5% and 0.45% normal saline) are incorrect because they do not provide the necessary isotonic environment for safe blood transfusion, risking hemolysis or other complications.

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. Individuals with DICER1 syndrome are at increased risk for developing various tumors, including papillary thyroid cancer. This is due to the role of DICER1 gene in regulating cell growth and division. The other choices, B (medullary thyroid cancer), C (pheochromocytoma), and D (renal cell carcinoma), are not typically associated with DICER1 syndrome. Medullary thyroid cancer is commonly linked to RET gene mutations, pheochromocytoma to genes such as SDHB, SDHD, and VHL, and renal cell carcinoma to mutations in genes like VHL and MET.

Which statement is correct regarding lymphocyte counts in infants versus adults?

  • A. NK-cell numbers are lowest at birth and increase with age.
  • B. B-cell numbers are highest at birth and decline with age.
  • C. T-cell numbers in infants are higher than in adults.
  • D. Infants have low lymphocyte counts that increase with age.
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.

The nurse examines the lymph nodes of a patient during a physical assessment. Which assessment finding would be of most concern to the nurse?

  • A. A 2-cm nontender supraclavicular node
  • B. A 1-cm mobile and nontender axillary node
  • C. An inability to palpate any superficial lymph nodes
  • D. Firm inguinal nodes in a patient with an infected foot
Correct Answer: A

Rationale: The correct answer is A. A 2-cm nontender supraclavicular node is of most concern as supraclavicular nodes are not normally palpable, and their enlargement can indicate underlying malignancy, such as metastatic cancer.
B: A 1-cm mobile and nontender axillary node is within normal limits.
C: Inability to palpate superficial lymph nodes may be due to factors like obesity or normal variation.
D: Firm inguinal nodes in a patient with an infected foot are expected as a local response to infection.

A 17-year-old patient is referred to you for a platelet count of 1,200,000/mm3. On history, she notes that she often has numbness and tingling in her hands and feet and has frequent epistaxis. She is otherwise well-appearing and has no recent infections. On her exam, you note splenomegaly. What do you expect to see on further evaluation?

  • A. Elevated C-reactive protein
  • B. Low ferritin
  • C. A hypocellular bone marrow
  • D. Low von Willebrand factor activity
Correct Answer: D

Rationale: The correct answer is D: Low von Willebrand factor activity. This patient likely has Essential Thrombocythemia, a disorder characterized by excessive production of platelets by the bone marrow. This condition can lead to abnormal bleeding and clotting. The patient's symptoms of numbness, tingling, epistaxis, and splenomegaly are consistent with this diagnosis. Low von Willebrand factor activity is expected in Essential Thrombocythemia due to platelet dysfunction. Elevated C-reactive protein, low ferritin, and a hypocellular bone marrow are not typically associated with Essential Thrombocythemia and are less likely to be seen in this patient.

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