Nokea
Presence of an --jaundice --splenomegaly with increase MCH is seen in:
- A. liver cirrhosis
- B. th.major
- C. PNH
- D. herditary spherocytosis
Correct Answer: D
Rationale: The correct answer is D: hereditary spherocytosis. In this condition, there is splenomegaly due to hemolysis, leading to jaundice. The increased MCH (mean corpuscular hemoglobin) is a characteristic finding in hereditary spherocytosis. Liver cirrhosis (choice A) may cause jaundice but is not typically associated with splenomegaly and increased MCH. Thalassemia major (choice B) presents with microcytic anemia and not typically associated with increased MCH. Paroxysmal nocturnal hemoglobinuria (choice C) is known for hemolysis but does not commonly present with splenomegaly and increased MCH.
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A 12-year-old girl presents to your clinic with significant menstrual bleeding at the onset of menarche and is noted to have a hemoglobin of 9.9, although she is not symptomatic from her anemia. Her mother reports that she has a history of epistaxis when she was a child with some episodes lasting 30 minutes and that she also has heavy menstrual bleeding. Which of the following tests will lead to the most likely diagnosis?
- A. Factor XI level
- B. Factor X level
- C. Factor XIII level
- D. Ristoectin cofactor activity
Correct Answer: D
Rationale: The correct answer is D: Ristoectin cofactor activity. The scenario describes a young girl with significant menstrual bleeding and a history of prolonged epistaxis, suggesting a bleeding disorder. Given the clinical presentation, the most likely diagnosis is von Willebrand disease (vWD), the most common inherited bleeding disorder. Ristocetin cofactor activity is a specific test for vWD, as it assesses the ability of von Willebrand factor to bind to platelets in the presence of ristocetin. Factors XI, X, and XIII are not typically associated with vWD, making choices A, B, and C incorrect. Testing for Factor VIII/von Willebrand factor antigen levels and vWF multimer analysis may also be considered to confirm the diagnosis.
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
Non thrombocytopenic purpura is seen in all except:
- A. vasculitis
- B. uraemia
- C. hereditary haemorrhagic telangiectasia
- D. SLE
Correct Answer: D
Rationale: The correct answer is D: SLE. Non-thrombocytopenic purpura refers to purpura without low platelet count. In SLE, thrombocytopenia is common, leading to purpura. A: Vasculitis can cause non-thrombocytopenic purpura due to vessel inflammation. B: Uraemia can lead to non-thrombocytopenic purpura due to vascular damage. C: Hereditary haemorrhagic telangiectasia presents with telangiectasias and recurrent epistaxis, not purpura.
Blinatumomab, a bispecific T-cell engaging molecule, is active against which CD antigen that is expressed on B-lymphoblasts?
- A. CD10
- B. CD15
- C. CD19
- D. CD20
Correct Answer: C
Rationale: Step-by-step rationale:
1. Blinatumomab is a bispecific molecule designed to engage T-cells to target B-lymphoblasts.
2. It targets CD19, a B-cell-specific antigen expressed on B-lymphoblasts.
3. Engagement of T-cells via CD19 leads to targeted killing of B-lymphoblasts.
4. CD10, CD15, and CD20 are not specific to B-cells, making them incorrect choices.
Summary:
The correct answer is C (CD19) because blinatumomab's mechanism of action specifically targets B-lymphoblasts via CD19. Choices A, B, and D are incorrect as they do not have the same specificity for B-cells as CD19 does.
Which of the following is a cause of secondary neutropaenia in adults?
- A. Congenital
- B. Anti-hypertensive drugs
- C. Part of general pancytopaenia
- D. Familial
Correct Answer: C
Rationale: Rationale: Secondary neutropenia in adults is often a part of general pancytopenia, which involves a decrease in all three blood cell types. This can be caused by factors such as bone marrow suppression from chemotherapy, radiation therapy, or certain medications. Neutropenia is not typically congenital or familial in adults, and anti-hypertensive drugs are not commonly known to directly cause secondary neutropenia. Therefore, the correct answer is C as it aligns with the common etiology of secondary neutropenia in adults.