Nokea
Hepato-splenomegaly with lymphadenopathy occur in all except:
- A. ALL
- B. lymphoma
- C. CML
- D. dissiminated TB
Correct Answer: C
Rationale: Step 1: Hepato-splenomegaly with lymphadenopathy is commonly seen in hematologic malignancies.
Step 2: Chronic Myeloid Leukemia (CML) primarily presents with splenomegaly, but not lymphadenopathy.
Step 3: Acute Lymphoblastic Leukemia (ALL), lymphoma, and disseminated TB can all present with these findings.
Step 4: Therefore, the correct answer is C - CML, as it does not typically present with lymphadenopathy. Other choices are incorrect as they can present with these symptoms.
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A 5-year-old boy presents with a 3-week history of his right eye “not moving to the right side.†The remainder of his neurologic exam is normal with the exception of a weak gag. MRI of the brain reveals a diffusely infiltrative mass in the pons. If this patient were to undergo a stereotactic biopsy, what would be the most likely molecular finding?
- A. BRAFKIAA1549 fusion
- B. Trisomy 21
- C. H3 K27M mutation
- D. RELA fusion
Correct Answer: C
Rationale: The correct answer is C: H3 K27M mutation. In children with diffuse midline gliomas, the most common molecular finding is the H3 K27M mutation. This mutation is associated with a worse prognosis and is commonly found in tumors located in the pons, which is consistent with the MRI findings in this case.
Choice A (BRAFKIAA1549 fusion) is incorrect as this fusion is typically associated with pilocytic astrocytomas, which are low-grade tumors and not typically found in the pons.
Choice B (Trisomy 21) is incorrect as it is a chromosomal abnormality associated with Down syndrome, not a molecular finding in brain tumors.
Choice D (RELA fusion) is incorrect as this fusion is typically found in ependymomas, which are not typically located in the pons.
Cooley's anemia is:
- A. Sickle cell an.
- B. thalassemia major
- C. high ESR
- D. aplastic an.
Correct Answer: B
Rationale: Cooley's anemia is another term for thalassemia major, a genetic disorder characterized by abnormal hemoglobin production leading to severe anemia. The correct answer is B because Cooley's anemia specifically refers to thalassemia major. Sickle cell anemia (A) is a different genetic disorder caused by abnormal hemoglobin shape. High ESR (C) is a nonspecific marker of inflammation and does not directly relate to Cooley's anemia. Aplastic anemia (D) is a condition where the bone marrow does not produce enough blood cells, not related to Cooley's anemia.
A 20-month-old otherwise healthy male presents late for his 18-month well child check. During his first year of life, he took iron-fortified formula and had a point-of-care hemoglobin (Hgb) of 12 g/dL at his 1-year well child check. His mother reports that he is a picky eater but loves milk and has recently become obsessive about chewing the corners of his cardboard books. Physical examination is normal except for a flow murmur. Which combination of laboratory test results listed below would most likely characterize this patient?
- A. Hgb 8.7 g/dL, mean corpuscular volume (MCV) 60 fL, serum ferritin 2 ng/mL
- B. Hgb 12.0 g/dL, MCV 80 fL, serum ferritin 30 ng/mL
- C. Hgb 9.2 g/dL, MCV 60 fL, serum ferritin 30 ng/mL
- D. Hgb 11.2 g/dL, MCV 90 fL, serum ferritin 7 ng/mL
Correct Answer: A
Rationale: The correct answer is A because it indicates iron deficiency anemia. The low Hgb of 8.7 g/dL is below normal range for his age. The MCV of 60 fL is low, indicating microcytic anemia which is characteristic of iron deficiency. The serum ferritin level of 2 ng/mL is very low, supporting the diagnosis.
Choice B is incorrect as the Hgb and MCV are within normal range, and the ferritin level is not indicative of iron deficiency. Choice C has a low Hgb but normal MCV and ferritin level. Choice D has a normal Hgb and low ferritin, but the MCV is high, inconsistent with iron deficiency anemia.
A 4-year-old girl with a history of recurrent epistaxis and easy bruising is referred to you for evaluation. She is found to have a prolonged PTT and a factor VIII level that is less than 1%. Both parents have a history of excessive bleeding. She is admitted with a severe episode of epistaxis, and your colleague orders 40 IU/kg of recombinant factor VIII. Her epistaxis resolves initially but within an hour starts again at the same severity as before. What is the best next step?
- A. Infuse a von Willebrand factor concentrate.
- B. Give another dose of recombinant factor VIII concentrate.
- C. Call otorhinolaryngology to pack her nose.
- D. Check for a factor VIII inhibitor.
Correct Answer: A
Rationale: The correct answer is A: Infuse a von Willebrand factor concentrate. In this scenario, the 4-year-old girl has a history of recurrent epistaxis and easy bruising, indicative of a bleeding disorder. The prolonged PTT and factor VIII level less than 1% suggest a deficiency in von Willebrand factor (VWF) or factor VIII. When the initial dose of recombinant factor VIII did not completely resolve the epistaxis, it indicates a possible deficiency in VWF activity. Therefore, the best next step is to infuse a von Willebrand factor concentrate to address the VWF deficiency, which should help control the bleeding.
Summary:
- Option B (Give another dose of recombinant factor VIII concentrate) is not the best choice because the initial dose did not fully resolve the bleeding, indicating a different factor may be deficient.
- Option C (Call otorhinolaryngology to pack her nose) addresses the symptom but does not
The nurse examines the lymph nodes of a patient during a physical assessment. Which assessment finding would be of most concern to the nurse?
- A. A 2-cm nontender supraclavicular node
- B. A 1-cm mobile and nontender axillary node
- C. An inability to palpate any superficial lymph nodes
- D. Firm inguinal nodes in a patient with an infected foot
Correct Answer: A
Rationale: The correct answer is A: A 2-cm nontender supraclavicular node. This finding is concerning because supraclavicular nodes are not normally palpable. Enlarged supraclavicular nodes can indicate metastatic cancer. B is incorrect because mobile and nontender axillary nodes are usually benign. C is incorrect as the inability to palpate superficial nodes may be normal. D is incorrect as firm inguinal nodes could indicate a localized infection or inflammation.