Nokea
Splenectomy is curative in:
- A. G6PD def.
- B. ITP
- C. thalassemia
- D. hereditary spherocytosis
Correct Answer: D
Rationale: The correct answer is D: hereditary spherocytosis. Splenectomy is curative in hereditary spherocytosis because the spleen is responsible for the destruction of the abnormal spherocytes in this condition. Removing the spleen eliminates the primary site of red blood cell destruction, leading to an improvement in anemia and other symptoms.
Incorrect choices:
A: Splenectomy is contraindicated in G6PD deficiency as it can worsen hemolysis by increasing the workload on other organs.
B: Splenectomy can be beneficial in ITP to increase platelet counts but is not curative as the underlying autoimmune process persists.
C: Splenectomy is not curative in thalassemia as it does not address the underlying genetic defect causing abnormal hemoglobin synthesis.
You may also like to solve these questions
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. The patient's presentation is consistent with acute promyelocytic leukemia (APL), indicated by the presence of Auer rod-containing blasts, very bright CD33 expression, and coagulopathy (elevated INR, low fibrinogen, elevated D-dimer). APL is associated with a specific genetic abnormality involving the PML-RARA fusion gene. ATRA is the mainstay of induction therapy for APL as it induces differentiation of leukemic promyelocytes. Aggressive management of coagulopathy is crucial to prevent life-threatening bleeding complications.
Incorrect answers:
A: Performing a lumbar puncture is not necessary at this stage as the patient's symptoms and laboratory findings point towards APL, not central nervous system involvement.
C: Dexamethasone and hydroxyurea are not
A 9-year-old boy is being treated for standard-risk acute lymphoblastic leukemia. His treatment protocol calls for administration of intravenous methotrexate and intramuscular L-asparaginase during interim maintenance chemotherapy. What is the most appropriate sequence of drug administration?
- A. Administer L-asparaginase during the methotrexate infusion.
- B. Administer L-asparaginase immediately after the methotrexate infusion.
- C. Administer both drugs at the same time to maximize synergistic activity.
- D. Administer methotrexate 24 hours after the asparaginase.
Correct Answer: B
Rationale: The correct answer is B: Administer L-asparaginase immediately after the methotrexate infusion. This is the most appropriate sequence because methotrexate can impair the activity of L-asparaginase if administered together. By waiting to administer L-asparaginase after the methotrexate infusion, the efficacy of both drugs is maximized without interference. Administering L-asparaginase during the methotrexate infusion (Choice A) would lead to decreased effectiveness of L-asparaginase. Administering both drugs at the same time (Choice C) is not recommended due to potential drug interactions. Administering methotrexate 24 hours after the asparaginase (Choice D) does not optimize the synergistic effects of the drugs during interim maintenance chemotherapy.
Basophilic stippling is classically seen in:
- A. CML
- B. myelosclerosis
- C. chronic lead poisoning
- D. iron def anemia
Correct Answer: C
Rationale: Basophilic stippling refers to the presence of blue-staining granules in red blood cells, which is a classic finding in chronic lead poisoning due to the inhibition of heme synthesis. In CML, the primary feature is the presence of the Philadelphia chromosome. Myelosclerosis is characterized by bone marrow fibrosis, not basophilic stippling. Iron deficiency anemia typically presents with microcytic hypochromic red blood cells, not basophilic stippling. Therefore, the correct answer is C: chronic lead poisoning.
The main vector transmitting malaria in Sabah is Anopheles balabacensis. This vector breeds in
- A. Artificial containers in urban centres
- B. Drains
- C. Temporary collecting of water in jungles
- D. Rock pools in control areas
Correct Answer: C
Rationale: The correct answer is C: Temporary collecting of water in jungles. Anopheles balabacensis is a forest-dwelling mosquito species commonly found in jungle environments. They prefer to breed in temporary water collections such as rainwater puddles, small ponds, or stream edges within the jungle. Breeding in urban areas, drains, or rock pools would not be typical for this species based on its natural habitat preference. Therefore, the most suitable breeding site for this vector in Sabah would be temporary water collections in jungles.
A 2-month-old infant is brought to your clinic with an extensive scaly rash on the scalp, which has been biopsied and shown to be Langerhans cell histiocytosis (LCH). You want to determine whether this patient has skin-only LCH or involvement of any of the 'high-risk' organs. The child has a normal CBC; normal liver enzymes and bilirubin; and a normal skeletal survey, skull films, and chest X ray. What other screening test will be important for finding involvement of a high-risk organ?
- A. Reticulocyte count
- B. Erythrocyte sedimentation rate
- C. Alkaline phosphatase
- D. Serum albumin and total protein
Correct Answer: D
Rationale: The correct answer is D: Serum albumin and total protein. In Langerhans cell histiocytosis (LCH), involvement of high-risk organs like the liver, spleen, and bone marrow can occur. Serum albumin and total protein levels can indicate liver involvement as hypoalbuminemia and hypoproteinemia can be seen in liver dysfunction. The other choices, A (Reticulocyte count), B (Erythrocyte sedimentation rate), and C (Alkaline phosphatase) are not specific for evaluating high-risk organ involvement in LCH. Reticulocyte count is related to red blood cell production, ESR is a nonspecific marker of inflammation, and alkaline phosphatase is commonly elevated in various conditions, not specific for high-risk organ involvement in LCH.