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A 3-month-old female presents to the emergency room with vomiting and abdominal distension. She has a left-side abdominal mass, and an abdominal ultrasound confirms an 8-cm mass arising from the left kidney. Liver lesions are also noted. Nephrectomy is performed and reveals a histologic diagnosis of malignant rhabdoid tumor of the kidney (MRTK). Which of the following is not a true statement about the management of this patient?
- A. Most patients with rhabdoid tumor of the kidney present in infancy.
- B. Most patients with rhabdoid tumor of the kidney present with metastatic (stage III or IV) disease.
- C. She has an excellent prognosis with surgery, chemotherapy, and radiation.
- D. Germline testing for SMARCB1/INI1 mutation on chromosome 22 is recommended, with brain MRI every 3 months until she is 5 years old, if testing is germline positive for SMARCB1/INI1.
Correct Answer: C
Rationale: The correct answer is C: She has an excellent prognosis with surgery, chemotherapy, and radiation. This statement is not true because malignant rhabdoid tumor of the kidney (MRTK) has a poor prognosis, even with aggressive treatment. Here's a breakdown:
1. MRTK is an aggressive and rare tumor that often presents in infancy, supporting statement A.
2. Most patients with MRTK present with metastatic disease, indicating poor prognosis, aligning with statement B.
3. Germline testing for SMARCB1/INI1 mutation is essential due to the genetic predisposition associated with MRTK, supporting statement D.
In summary, statement C is incorrect as MRTK typically has a challenging clinical course despite comprehensive treatment approaches.
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A 13-year-old Hispanic girl is found to have a WBC count of 6,500/mm3 with 40% Auer rod–containing granular blasts that, by flow cytometry, express very bright CD33 but are negative for human leukocyte antigen–DR isotype (HLA-DR). She is oozing blood around her peripheral IV site. Coagulation studies reveal an international normalized ratio (INR) of 3.4, a fibrinogen of 170, and a markedly elevated D-dimer. Marrow aspirate shows nearly 90% blasts with a similar morphology. You send the marrow to the fluorescence in situ hybridization (FISH) lab and request STAT testing for the most likely recurrent genetic abnormality based on the clinical presentation. How do you plan to initiate therapy?
- A. Perform a lumbar puncture to determine leukemic involvement, then proceed with induction chemotherapy.
- B. Begin therapy with all-trans retinoic acid (ATRA) immediately while aggressively managing coagulopathy with blood product support.
- C. Start dexamethasone and hydroxyurea immediately while aggressively managing coagulopathy with blood product support.
- D. Start induction chemotherapy, obtain HLA typing, and start a donor search because of the poor prognosis associated with this leukemic phenotype.
Correct Answer: B
Rationale: The correct answer is B: Begin therapy with all-trans retinoic acid (ATRA) immediately while aggressively managing coagulopathy with blood product support. In this scenario, the patient presents with acute promyelocytic leukemia (APL), characterized by the presence of Auer rod-containing blasts expressing CD33 and negative for HLA-DR. The presence of coagulopathy with elevated INR, D-dimer, and oozing blood suggests disseminated intravascular coagulation (DIC), a common complication in APL. Immediate treatment with ATRA is crucial to differentiate and mitigate the risk of DIC worsening. ATRA induces differentiation of APL blasts, resolving the coagulopathy. Aggressive management of coagulopathy with blood product support is essential to prevent bleeding complications. Lumbar puncture (choice A), dexamethasone and hydroxyurea (choice C), and starting a donor search (choice D) are not indicated as the
A nurse is caring for a client who is about to begin taking folic acid to treat megaloblastic anemia. The nurse should monitor which of the following laboratory values to determine therapeutic effectiveness?
- A. Amylase level
- B. Reticulocyte count
- C. C-reactive protein
- D. Creatinine clearance
Correct Answer: B
Rationale: The correct answer is B: Reticulocyte count. Reticulocytes are immature red blood cells. In megaloblastic anemia, there is a decrease in red blood cell production due to a deficiency in folic acid. Monitoring reticulocyte count helps determine if the folic acid treatment is increasing red blood cell production.
A: Amylase level is not relevant to monitoring the effectiveness of folic acid in treating megaloblastic anemia.
C: C-reactive protein is a marker for inflammation and not specific to monitoring anemia treatment.
D: Creatinine clearance is used to assess kidney function, not the effectiveness of folic acid in treating anemia.
An oncology nurse is caring for a patient with multiple myeloma who is experiencing bone destruction. When reviewing the patient's most recent blood tests, the nurse should anticipate what imbalance?
- A. Hypercalcemia
- B. Hyperproteinemia
- C. Elevated serum viscosity
- D. Elevated RBC count
Correct Answer: A
Rationale: The correct answer is A: Hypercalcemia. In multiple myeloma, bone destruction releases calcium into the bloodstream, leading to hypercalcemia. Elevated serum viscosity (C) is not typically associated with multiple myeloma. Hyperproteinemia (B) may be present due to increased production of abnormal proteins, but it does not directly cause bone destruction. Elevated RBC count (D) is not a common finding in multiple myeloma and is not directly related to bone destruction.
You have a new 7-year-old female patient with a WBC count of 6,000/mm3, hemoglobin of 7.2 g/dL, and platelet count of 30,000/mm3. A bone marrow aspirate reveals 14% blasts with a monocytic morphologic appearance that are surface marker positive for CD33. You receive a call from the fluorescence in situ hybridization (FISH) lab that the bone marrow is positive for KMT2A rearrangement in 68% of cells. Your staff asks whether this represents a diagnosis of acute leukemia in the current classification scheme for this type of hematologic malignancy. What would you say?
- A. No, because for a diagnosis of acute leukemia you must have 30% or more blasts in the marrow.
- B. No, because for a diagnosis of acute leukemia you must have 20% or more blasts in the marrow.
- C. No, because the cytogenetics do not include +21, monosomy 7, or trisomy 8.
- D. Yes, because the FISH is positive for KMT2A rearrangement.
Correct Answer: D
Rationale: The correct answer is D: Yes, because the FISH is positive for KMT2A rearrangement. The presence of KMT2A rearrangement in 68% of cells indicates a specific genetic abnormality associated with acute leukemia. This abnormality is a known marker for acute myeloid leukemia (AML) with recurrent genetic abnormalities. The percentage of blasts in the bone marrow (14%) may not meet the traditional criteria for a diagnosis of acute leukemia based on blast percentage alone, but the presence of KMT2A rearrangement overrides this requirement in this case. Choices A and B are incorrect because blast percentage alone is not the sole determinant of a diagnosis of acute leukemia when specific genetic abnormalities are present. Choice C is incorrect because while the absence of specific cytogenetic abnormalities may be relevant for some cases, the presence of KMT2A rearrangement is sufficient to support a diagnosis of acute leukemia in this context.
A nurse is caring for a client who recently started alteplase therapy. The nurse should monitor the client for which of the following adverse effects?
- A. Bronchodilation
- B. Headache
- C. Edema
- D. Hypertension
Correct Answer: B
Rationale: The correct answer is B: Headache. Alteplase is a thrombolytic medication that can increase the risk of bleeding, including intracranial bleeding, leading to headaches. Monitoring for headaches is crucial to detect any signs of bleeding. Bronchodilation (A) is not a common adverse effect of alteplase. Edema (C) and hypertension (D) are also not typically associated with alteplase therapy. Monitoring for headache is essential for early detection of potentially life-threatening complications.