Nokea
A 50-year-old male who had frequent infections of his upper respiratory tract manifested with subcutaneous petechiae over his arms and legs. Investigations revealed low hemoglobin and a dry tap marrow. The most likely condition the patient is suffering from
- A. Iron deficiency anemia
- B. Megaloblastic anemia
- C. Hemolytic anemia
- D. Aplastic anemia
Correct Answer: D
Rationale: The correct answer is D: Aplastic anemia. Aplastic anemia is characterized by pancytopenia, which includes low hemoglobin levels leading to anemia. The dry tap marrow indicates bone marrow failure, which is a hallmark of aplastic anemia. The subcutaneous petechiae are due to thrombocytopenia, another feature of this condition. Iron deficiency anemia (A) typically presents with microcytic hypochromic red blood cells and is not associated with bone marrow failure. Megaloblastic anemia (B) is characterized by macrocytic red blood cells and is usually due to deficiencies in vitamin B12 or folate. Hemolytic anemia (C) involves the premature destruction of red blood cells and is not typically associated with bone marrow failure.
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A 2-month-old girl is found to have a small, hard mass on her scalp. The mass increases in size over the next 4 weeks. A biopsy is performed that confirms a diagnosis of embryonal rhabdomyosarcoma. You initiate chemotherapy with vincristine, dactinomycin, and cyclophosphamide. The child presents to clinic for day 1 of cycle 3 of chemotherapy, and the mass on her scalp is smaller. She is afebrile, absolute neutrophil count is 1,405 cells/mcL, platelet count is 154,000/mcL, and total bilirubin is 0.8 mg/dL. Her mother reports she looks very tired because her eyelids have been 'very droopy,' and she thinks she has a sore throat because her cry is hoarse. Her last bowel movement was 2 days ago. What is the most appropriate chemotherapy plan?
- A. Continue vincristine, dactinomycin, and cyclophosphamide at full dosage.
- B. Do not administer any chemotherapy; rhabdomyosarcoma is progressing and she needs different therapy.
- C. Administer dactinomycin and cyclophosphamide but hold the vincristine and reevaluate weekly. If the ptosis and hoarse cry resolve, vincristine can be resumed with a dose reduction and, if tolerated, re-escalated to the full dose in the future.
- D. Administer dactinomycin and cyclophosphamide but discontinue vincristine permanently.
Correct Answer: C
Rationale: The correct answer is C: Administer dactinomycin and cyclophosphamide but hold the vincristine and reevaluate weekly. If the ptosis and hoarse cry resolve, vincristine can be resumed with a dose reduction and, if tolerated, re-escalated to the full dose in the future.
Rationale:
1. Ptosis and hoarse cry are symptoms of vincristine-induced neurotoxicity.
2. Holding vincristine allows for resolution of these side effects.
3. Reevaluating weekly ensures close monitoring of symptoms.
4. If symptoms resolve, vincristine can be cautiously reintroduced with a dose reduction to prevent further neurotoxicity.
Summary:
A: Continuing all chemotherapy may worsen neurotoxicity.
B: Withholding all chemotherapy is not appropriate if two agents are effective.
D: Discontinuing vincristine permanently may limit treatment options.
A 4-year-old boy is pale with intermittent jaundice and splenomegaly. Laboratory results are as follows: RBC 4.85 M/mcL (N); Hgb 8.6 g/dL (L); Hct 25.8% (L); MCV 81.6 (N); MCHC 38% (H); RDW 20% (H); Retic 7% (H). What are the two best tests to distinguish autoimmune hemolytic anemia from hereditary spherocytosis?
- A. Free erythrocyte protoporphyrin and IgG levels
- B. Hemoglobin electrophoresis and direct antiglobulin test (DAT)
- C. Lactate dehydrogenase (LDH) and modified Russell viper venom test
- D. Red cell distribution width (RDW) and mean corpuscular hemoglobin concentration (MCHC)
Correct Answer: E
Rationale: As the correct answer is not provided, I'll provide a step-by-step rationale for each choice:
A: Free erythrocyte protoporphyrin and IgG levels are not specific tests for distinguishing between autoimmune hemolytic anemia and hereditary spherocytosis.
B: Hemoglobin electrophoresis and direct antiglobulin test (DAT) can help differentiate between these two conditions as autoimmune hemolytic anemia is associated with a positive DAT, while hereditary spherocytosis typically has a negative DAT.
C: Lactate dehydrogenase (LDH) and modified Russell viper venom test are not specific tests for distinguishing between autoimmune hemolytic anemia and hereditary spherocytosis.
D: Red cell distribution width (RDW) and mean corpuscular hemoglobin concentration (MCHC) are not typically used to distinguish between autoimmune hemolytic anemia and hereditary spherocytosis.
Therefore, the best tests
A patient with advanced leukemia is responding poorly to treatment. The nurse finds the patient tearful and trying to express his feelings, but he is clearly having difficulty. What is the nurse's most appropriate action?
- A. Tell him that you will give him privacy and leave the room.
- B. Offer to call pastoral care.
- C. Ask if he would like you to sit with him while he collects his thoughts.
- D. Tell him that you can understand how he's feeling.
Correct Answer: C
Rationale: The correct answer is C: Ask if he would like you to sit with him while he collects his thoughts. This is the most appropriate action because it shows empathy and support for the patient's emotional distress. By offering to sit with the patient, the nurse acknowledges the patient's feelings and provides a comforting presence. This can help the patient feel understood and supported during a difficult time.
Choices A and D are incorrect because they do not actively offer emotional support or show empathy towards the patient. Choice A may make the patient feel abandoned, and choice D may come across as insincere or dismissive of the patient's emotions.
Choice B, offering to call pastoral care, is also not the most appropriate action in this scenario because the patient may need immediate emotional support from the nurse. Pastoral care can be considered later if the patient expresses a desire for spiritual or religious support.
Several gene mutations have been associated with juvenile myelomonocytic leukemia (JMML), and they may or may not have prognostic implications. A gene expression–based classification system has been found to be an independent predictor of clinical outcome in these patients. What is the disease signature that predicts a poor outcome?
- A. Tyrosine kinase inhibitors
- B. Acute myeloid leukemia–like
- C. Chronic myeloid leukemia-like
- D. BRAF pathway abnormalities
Correct Answer: B
Rationale: The correct answer is B: Acute myeloid leukemia-like. In JMML, a disease signature resembling acute myeloid leukemia (AML) has been associated with a poor outcome. This signature includes features such as increased blasts, abnormal karyotypes, and mutations in genes like NRAS and KRAS. AML-like JMML cases often have aggressive disease progression and poorer response to treatment. Tyrosine kinase inhibitors (choice A) are not typically used in JMML treatment. Chronic myeloid leukemia-like (choice C) is not associated with a poor outcome in JMML. BRAF pathway abnormalities (choice D) may be present in some cases of JMML but are not the primary disease signature predicting poor outcomes.
Incorrect about pernicious an:
- A. hyperchlorhydria
- B. premature graying of hair
- C. anti intrinsic factor antibody in 60% of pts.
- D. gastric polyp may occur
Correct Answer: A
Rationale: The correct answer is A: hyperchlorhydria. Pernicious anemia is characterized by vitamin B12 deficiency due to impaired absorption, not excess stomach acid. Premature graying of hair is unrelated. Presence of anti-intrinsic factor antibody and risk of gastric polyps are associated with pernicious anemia, but not hyperchlorhydria. Therefore, choice A is the correct answer as it does not align with the typical symptoms and characteristics of pernicious anemia.