Nokea
A 12-year-old patient has been referred to you following complete resection with clean margins of a high-grade malignant peripheral nerve sheath tumor of the shoulder region. The tumor measured approximately 4 cm in greatest dimension. A CT scan of the chest and a bone scan were within normal limits. The patient does not have evidence of neurofibromatosis type 1 (NF1). Which of the following treatment approaches would you recommend?
- A. Chemotherapy with doxorubicin and ifosfamide
- B. Radiotherapy
- C. Chemotherapy with doxorubicin and ifosfamide plus radiotherapy
- D. Observation
Correct Answer: D
Rationale: The correct answer is D: Observation. Given the complete resection with clean margins, normal chest CT, bone scan, and absence of NF1, observation is appropriate. Chemotherapy or radiotherapy may be considered in the presence of residual disease, positive margins, or metastasis. Chemotherapy with doxorubicin and ifosfamide may cause unnecessary toxicity without clear benefit in this scenario. Radiotherapy may be reserved for cases with high-risk features. Combining chemotherapy and radiotherapy may lead to increased toxicity without proven benefit. Observation allows monitoring for disease recurrence while minimizing unnecessary treatment side effects.
You may also like to solve these questions
The nurse is documenting findings after completing data collection with a patient. What term should the nurse use to document a large area of discoloration from hemorrhage under the skin?
- A. Pallor
- B. Rubor
- C. Petechiae
- D. Ecchymosis
Correct Answer: D
Rationale: The correct term to document a large area of discoloration from hemorrhage under the skin is ecchymosis. Ecchymosis refers to a bruise caused by the extravasation of blood into the subcutaneous tissues. Pallor (A) is paleness, rubor (B) is redness, and petechiae (C) are tiny, pinpoint-sized red or purple spots on the skin due to bleeding. Ecchymosis is the most appropriate term for describing a large area of discoloration from hemorrhage under the skin as it specifically denotes a bruise resulting from blood leakage into tissues.
A patient shows prolonged prothrombin time and activated partial thromboplastin time. Blood test otherwise is normal. Which of the following is the most probably diagnosis for this patient's condition?
- A. Hemophilia A
- B. Thrombocytopenia
- C. Von Willebrand factor disease
- D. Vitamin K deficiency
Correct Answer: D
Rationale: The correct answer is D: Vitamin K deficiency. Prolonged prothrombin time and activated partial thromboplastin time indicate impaired blood clotting, which is a characteristic of Vitamin K deficiency. Vitamin K is essential for the synthesis of clotting factors II, VII, IX, and X. Hemophilia A (Choice A) is a genetic disorder resulting in a deficiency of clotting factor VIII, leading to prolonged activated partial thromboplastin time only. Thrombocytopenia (Choice B) is a condition characterized by low platelet count, which can lead to bleeding but does not affect clotting factors. Von Willebrand disease (Choice C) is a genetic disorder affecting von Willebrand factor and platelet function, not clotting factors.
Increase Fe & normal TIBC are found in:
- A. thalassemia major
- B. haemosidrosis
- C. rheumatoid arthritis
- D. dissiminated malignancy
Correct Answer: C
Rationale: The correct answer is C: rheumatoid arthritis. In rheumatoid arthritis, there is chronic inflammation leading to elevated levels of ferritin (Fe) due to increased iron storage and a normal total iron-binding capacity (TIBC). This is because despite the increased iron stores, the body is unable to utilize it fully due to chronic inflammation.
Incorrect choices:
A: Thalassemia major would typically present with decreased TIBC due to increased iron absorption to compensate for the chronic anemia.
B: Haemosiderosis would show elevated Fe but a decreased TIBC due to iron overload.
D: Disseminated malignancy may show elevated Fe levels but typically with a decreased TIBC due to anemia of chronic disease.
A young child with consanguineous parents has developmental delay and a history of multiple recurrent bacterial infections and short stature. He presents to the emergency department following trauma and requires a blood transfusion. Blood work identifies leukocytosis, neutrophilia, and the Bombay blood group (absent H antigen as well as absent A and B antigens). What is this patient's diagnosis?
- A. Chediak-Higashi syndrome
- B. Leukocyte adhesion deficiency (LAD) Type II
- C. CD18 deficiency
- D. Griscelli syndrome
Correct Answer: B
Rationale: The correct answer is B: Leukocyte adhesion deficiency (LAD) Type II. This patient's symptoms of recurrent bacterial infections, leukocytosis, neutrophilia, short stature, and Bombay blood group (lack of H antigen) are characteristic of LAD Type II. In LAD Type II, there is a defect in fucose metabolism, leading to impaired leukocyte adhesion and migration, causing immune dysfunction. Chediak-Higashi syndrome (A) presents with oculocutaneous albinism, recurrent infections, and giant granules in leukocytes. CD18 deficiency (C) is a type of LAD characterized by mutations in the CD18 gene causing impaired integrin function. Griscelli syndrome (D) presents with silvery hair, immunodeficiency, and neurological deficits, not matching this patient's symptoms.
You have a new patient consult in clinic this morning. The referral packet includes the newborn screen report, which is flagged abnormal hemoglobinopathy screen, F, A, Bart's, refer to hematology, and a complete blood count done at 4 years of age with a hemoglobin of 10 g/dL and an MCV of 68. The pediatrician has informed the parents the child has some form of alpha thalassemia. The older brother had the same newborn screen results but had a normal complete blood count when checked. The mother wants to know why her second child has an abnormal complete blood count when she and her husband do not have any blood problems. How would you respond to the child's mother?
- A. The mother and father are both silent carriers and each passed a deleted alpha globin allele to their child. The child inherited a trans-deletion genotype alpha thalassemia trait.
- B. The mother has cis deletion alpha thalassemia and the father has no alpha globin deletion, giving the child alpha thalassemia trait.
- C. Neither parent has an alpha globin deletion; this was a new spontaneous mutation causing alpha thalassemia in the child.
- D. Both parents carry cis deletions in the alpha globin gene cluster.
Correct Answer: A
Rationale: Rationale for correct answer A:
1. Both parents must be carriers for the child to have alpha thalassemia.
2. The child has an abnormal complete blood count, indicating a hemoglobinopathy.
3. The child inherited a trans-deletion genotype alpha thalassemia trait from both parents.
4. Silent carriers have no symptoms but can pass on the trait.
Summary for incorrect choices:
B: In cis deletion, both parents would have to pass on the trait, which is not the case here.
C: Spontaneous mutation is unlikely for alpha thalassemia.
D: Both parents having cis deletions would result in a more severe form of alpha thalassemia.