Nokea
Splenectomy is contraindicated in:
- A. pyruvate kinase def.
- B. ITP
- C. BM failure
- D. angiogenic myeloid metaplasia
Correct Answer: C
Rationale: Splenectomy is contraindicated in bone marrow (BM) failure because the spleen plays a crucial role in hematopoiesis and immune function. Removing the spleen can worsen BM failure by disrupting hematopoiesis and exacerbating the existing condition. In pyruvate kinase deficiency, splenectomy can improve symptoms by reducing hemolysis. In immune thrombocytopenic purpura (ITP), splenectomy may be considered as a treatment option to increase platelet count. Angiogenic myeloid metaplasia does not have a direct contraindication for splenectomy, as the primary concern is the underlying disease pathology, not the surgical intervention.
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The patient is a 2-month-old boy who presented with a skin abscess and is febrile. On exam, he is noted to have silvery hair and hypopigmented skin. A CBC shows a leukocyte count of 3.4 K/mcL with 10% neutrophils. What does the abnormality on the peripheral smear suggest?
- A. Abnormal lysosomal biogenesis
- B. Abnormal ribosome function
- C. Abnormal phagocytosis of opsonized particles
- D. Abnormal mitochondrial activity
Correct Answer: A
Rationale: The correct answer is A: Abnormal lysosomal biogenesis. The patient's presentation of silvery hair and hypopigmented skin suggests a lysosomal storage disorder like Chediak-Higashi syndrome. The peripheral smear showing 10% neutrophils with a low leukocyte count indicates impaired neutrophil function due to abnormal lysosomal biogenesis. This affects phagocytosis and intracellular killing of pathogens, leading to recurrent infections and abscess formation.
Incorrect choices:
B: Abnormal ribosome function - not related to the patient's presentation.
C: Abnormal phagocytosis of opsonized particles - the primary issue is with lysosomal biogenesis, not phagocytosis.
D: Abnormal mitochondrial activity - does not explain the silvery hair and hypopigmented skin seen in Chediak-Higashi syndrome.
You are consulted on a 4-year-old girl who is newly diagnosed with standard-risk pre-B acute lymphoblastic leukemia. After reviewing her previous complete blood examinations, you note she has had a platelet count ranging from 80,000 to 100,000 cells/mcL over the past 2 years. Her father mentions that he has also been told he has mild thrombocytopenia. You suspect the child may have a cancer predisposition syndrome. Which sample should you send for analysis, and which gene is most likely implicated?
- A. Skin fibroblasts to evaluate the RUNX1 gene
- B. Skin fibroblasts to evaluate the ETV6 gene
- C. Buccal swab to evaluate the RUNX1 gene
- D. Buccal swab to evaluate the ETV6 gene
Correct Answer: B
Rationale: The correct answer is B: Skin fibroblasts to evaluate the ETV6 gene.
1. ETV6 gene mutations are associated with inherited thrombocytopenia, making it a likely candidate in this case.
2. Skin fibroblasts are needed for genetic analysis as they provide a reliable sample for detecting genetic mutations.
3. RUNX1 gene mutations are also linked to thrombocytopenia but are more commonly associated with familial platelet disorder with predisposition to acute myeloid leukemia.
4. Buccal swabs are not ideal for evaluating genetic mutations related to thrombocytopenia as they may not provide sufficient genetic material for accurate testing.
Which of the following is the most suitable anti malarial drug for pregnant women?
- A. Atovaquone and proguanil
- B. Quinine and doxycycline
- C. Quinine and clindamycin
- D. Primaquine
Correct Answer: C
Rationale: The correct answer is C: Quinine and clindamycin. Quinine is considered safe for use in pregnancy to treat malaria, as it has been used for many years without significant adverse effects. Clindamycin is also safe for use in pregnancy and can be used in combination with quinine to treat malaria. Atovaquone and proguanil (choice A) are not recommended for use in pregnancy due to limited safety data. Quinine and doxycycline (choice B) is not suitable for pregnant women as doxycycline is contraindicated during pregnancy. Primaquine (choice D) is not recommended for use during pregnancy as it can cause harm to the fetus.
Busulfan therapy lead to all except:
- A. hyperpigmentation
- B. pulm .Fibrosis
- C. optic neuritis
- D. BM suppression
Correct Answer: C
Rationale: The correct answer is C: Optic neuritis. Busulfan therapy is known to cause adverse effects like hyperpigmentation, pulmonary fibrosis, and bone marrow suppression due to its cytotoxic nature. However, optic neuritis is not a common side effect associated with busulfan. Optic neuritis typically occurs due to inflammation of the optic nerve and is more commonly linked to conditions like multiple sclerosis. Therefore, in the context of busulfan therapy, optic neuritis is not a expected side effect, making it the correct answer.
A female infant is diagnosed with hemophagocytic lymphohistiocytosis (HLH) not associated with an Epstein-Barr virus (EBV) infection. In taking the family history, you learn that another female infant died of HLH 2 years ago. Also, a newborn female child died of an unknown disease 4 years prior and was said have been bleeding profusely, jaundiced, and had a distended abdomen. When counseling the family about the genetics of HLH, how will you explain it?
- A. It is an X-linked syndrome
- B. It is an autosomal recessive syndrome
- C. It is a dominant inheritance syndrome
- D. It is an autosomal recessive syndrome with incomplete penetrance
Correct Answer: B
Rationale: The correct answer is B: It is an autosomal recessive syndrome. HLH is typically inherited in an autosomal recessive manner, meaning that two copies of the affected gene are needed to manifest the disease. In this case, the family history indicates that multiple female infants were affected, suggesting a recessive pattern. Choice A is incorrect as HLH is not an X-linked syndrome, indicated by the affected female infants. Choice C is incorrect since dominant inheritance would not result in multiple affected female infants. Choice D is also incorrect as autosomal recessive syndromes do not typically exhibit incomplete penetrance.