Nokea
You are seeing a 12-year-old boy in the survivorship program who presented at 2 years old with a desmoplastic nodular medulloblastoma. You note the child recently underwent germline genetic testing and was found to have nevoid basal cell carcinoma syndrome. In which gene is the child most likely to have a pathogenic variant?
- A. PTEN
- B. CDKN2A
- C. SUFU
- D. SMARCB1
Correct Answer: C
Rationale: Rationale:
1. Nevoid basal cell carcinoma syndrome is also known as Gorlin syndrome, caused by mutations in SUFU.
2. SUFU gene regulates the Hedgehog signaling pathway, implicated in medulloblastoma development.
3. Desmoplastic nodular medulloblastoma is commonly seen in Gorlin syndrome.
4. PTEN is associated with Cowden syndrome, CDKN2A with melanoma, and SMARCB1 with rhabdoid tumors, not typically seen in Gorlin syndrome.
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When assessing a newly admitted patient, the nurse notes pallor of the skin and nail beds. The nurse should ensure that which laboratory test has been ordered?
- A. Platelet count
- B. Neutrophil count
- C. White blood cell count
- D. Hemoglobin (Hgb) level
Correct Answer: D
Rationale: The correct answer is D: Hemoglobin (Hgb) level. Pallor of the skin and nail beds indicates possible anemia, which is a condition characterized by low levels of hemoglobin. Thus, checking the hemoglobin level is crucial to confirm the suspicion of anemia. Platelet count (choice A) assesses for clotting ability, neutrophil count (choice B) and white blood cell count (choice C) are indicators of infection or inflammation, which are not directly related to pallor. Hemoglobin level directly correlates with the observed symptom of pallor, making it the most appropriate laboratory test to order.
A 77-year-old male is admitted to a unit with a suspected diagnosis of acute myeloid leukemia (AML). When planning this patient's care, the nurse should be aware of what epidemiologic fact?
- A. Early diagnosis is associated with good outcomes.
- B. Five-year survival for older adults is approximately 50%.
- C. Five-year survival for patients over 75 years old is less than 2%.
- D. Survival rates are wholly dependent on the patient's pre-illness level of health.
Correct Answer: C
Rationale: The correct answer is C: Five-year survival for patients over 75 years old is less than 2%. This is because older age is a significant negative prognostic factor in acute myeloid leukemia (AML). As individuals age, their overall health and ability to tolerate aggressive treatments decline, leading to poorer outcomes. The survival rate of less than 2% for patients over 75 years old reflects the challenges of treating AML in this age group.
Choice A is incorrect because early diagnosis does not necessarily guarantee good outcomes in AML, especially in older adults where other factors play a significant role. Choice B is incorrect as the five-year survival rate of 50% does not apply to older adults with AML. Choice D is incorrect as survival rates in AML are influenced by various factors beyond just the patient's pre-illness health status.
Bone marrow responds to iron therapy by increasing erythropoietic activity. Which of the following in bone marrow would most likely indicate erythropoiesis?
- A. Myelocytes
- B. Reticulocytes
- C. Ring sideroblasts
- D. Target cells
Correct Answer: B
Rationale: Rationale:
1. Bone marrow increases erythropoiesis in response to iron therapy.
2. Reticulocytes are immature red blood cells derived from erythroblasts, indicating active erythropoiesis.
3. Myelocytes are precursors of granulocytes, not involved in erythropoiesis.
4. Ring sideroblasts are abnormal erythroblasts with iron granules, not indicative of active erythropoiesis.
5. Target cells are red blood cells with central "target-like" appearance due to excess membrane, not directly related to erythropoiesis.
The typical Reed-Sternberg cells are either infrequent or absent. Instead, lymphocytic and histiocytic cells or 'popcorn cells' are seen within a background of inflammatory cells, which are predominantly benign lymphocytes. Which type of Hodgkin's lymphoma best suit the description?
- A. Nodular sclerosis
- B. Mixed cellularity
- C. Lymphocyte depleted
- D. Lymphocyte predominance
Correct Answer: D
Rationale: The correct answer is D: Lymphocyte predominance. In this type of Hodgkin's lymphoma, the typical Reed-Sternberg cells are absent or rare. Instead, popcorn cells (lymphocytic and histiocytic cells) are seen among benign lymphocytes. This is characteristic of lymphocyte predominance subtype.
A: Nodular sclerosis - characterized by lacunar Reed-Sternberg cells in a background of collagen bands.
B: Mixed cellularity - characterized by numerous Reed-Sternberg cells, eosinophils, plasma cells, and histiocytes in a background of mixed inflammatory cells.
C: Lymphocyte depleted - characterized by few Reed-Sternberg cells in a background of fibrosis and few inflammatory cells.
Therefore, based on the description provided, choice D is the most appropriate answer.
You are seeing a 12-year-old boy in the survivorship program who presented at 2 years old with a desmoplastic nodular medulloblastoma. You note the child recently underwent germline genetic testing and was found to have nevoid basal cell carcinoma syndrome. In which gene is the child most likely to have a pathogenic variant?
- A. PTEN
- B. CDKN2A
- C. SUFU
- D. SMARCB1
Correct Answer: C
Rationale: Rationale: The correct answer is C: SUFU. Nevoid basal cell carcinoma syndrome, also known as Gorlin syndrome, is associated with pathogenic variants in the SUFU gene. SUFU is a tumor suppressor gene involved in the sonic hedgehog signaling pathway, which plays a role in medulloblastoma and basal cell carcinoma development. PTEN (A) is associated with Cowden syndrome, CDKN2A (B) with familial atypical multiple mole melanoma syndrome, and SMARCB1 (D) with rhabdoid tumor predisposition syndrome, but not specifically linked to nevoid basal cell carcinoma syndrome.