Anatomy of Hematologic System

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A 2-year-old girl has a diagnosis of overall stage IV favorable histology Wilms' tumor with pulmonary metastases and local stage III disease due to finding positive lymph nodes. After she completes 6 weeks of vincristine/dactinomycin/doxorubicin (DD4A) chemotherapy, restaging shows complete resolution of some but not all lung nodules. Tumor genetic testing reveals combined loss of heterozygosity for 1p and 16q. Which of the following would be the most appropriate treatment plan?

  • A. Continue chemotherapy with vincristine, doxorubicin, and dactinomycin to complete 25 weeks of therapy. Administer radiation to lungs and flank.
  • B. Continue chemotherapy with vincristine, doxorubicin and dactinomycin to complete 25 weeks of therapy. Radiation to flank only. No lung radiation.
  • C. Continue chemotherapy with vincristine, doxorubicin and dactinomycin, add cyclophosphamide and etoposide to complete 33 weeks of therapy. Radiation to flank only. No lung radiation.
  • D. Continue chemotherapy with vincristine, doxorubicin and dactinomycin, add cyclophosphamide and etoposide to complete 33 weeks of therapy. Radiation to lungs and flank.
Correct Answer: D

Rationale: The correct answer is D because the addition of cyclophosphamide and etoposide to the chemotherapy regimen is indicated for patients with combined loss of heterozygosity for 1p and 16q in Wilms' tumor. This genetic abnormality is associated with a higher risk of relapse and poorer outcomes. The extended duration of chemotherapy (33 weeks) is necessary to target any remaining tumor cells and reduce the risk of recurrence. Additionally, the decision to administer radiation to both the lungs and flank is appropriate given the incomplete resolution of some lung nodules after initial chemotherapy. Lung radiation helps to target any remaining metastatic lesions, while flank radiation targets the primary tumor site and positive lymph nodes. This comprehensive treatment approach aims to maximize the chances of long-term disease control and survival for the patient.

Choice A is incorrect because it lacks the addition of cyclophosphamide and etoposide, which are needed for Wilms' tumor with the specific genetic abnormality mentioned.

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A patient shows prolonged prothrombin time and activated partial thromboplastin time. Blood test otherwise is normal. Which of the following is the most probably diagnosis for this patient's condition?

  • A. Hemophilia A
  • B. Thrombocytopenia
  • C. Von Willebrand factor disease
  • D. Vitamin K deficiency
Correct Answer: D

Rationale: The correct answer is D: Vitamin K deficiency. Prolonged prothrombin time and activated partial thromboplastin time indicate impaired blood clotting, which is characteristic of vitamin K deficiency. Vitamin K is essential for synthesizing clotting factors II, VII, IX, and X. Hemophilia A (choice A) is a genetic disorder resulting in deficiency of clotting factor VIII, not vitamin K-dependent factors. Thrombocytopenia (choice B) is a condition characterized by low platelet count, not affecting clotting factor synthesis. Von Willebrand factor disease (choice C) involves a deficiency or dysfunction of von Willebrand factor, not related to vitamin K deficiency.

Which of the following is false regarding Burkitt's lymphoma?

  • A. Associated with translocation of c-myc gene on chromosome 18
  • B. Usually presents with abdominal involvement in HIV patients
  • C. Common in Africa children due to Epstein Barr infection after malaria exposure.
  • D. Histologically characterized by a 'starry sky' appearance
Correct Answer: A

Rationale: The correct answer is A because Burkitt's lymphoma is associated with translocation of c-myc gene on chromosome 8, not chromosome 18. This translocation leads to uncontrolled cell proliferation. Choice B is incorrect because Burkitt's lymphoma commonly presents with jaw or facial bone involvement in HIV patients. Choice C is incorrect as the association between Epstein Barr infection and malaria exposure in African children increases the risk of Burkitt's lymphoma. Choice D is incorrect because Burkitt's lymphoma is histologically characterized by a 'starry sky' appearance due to macrophages engulfing apoptotic tumor cells, not due to the presence of the c-myc translocation.

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 young child with consanguineous parents has developmental delay and a history of multiple recurrent bacterial infections and short stature. He presents to the emergency department following trauma and requires a blood transfusion. Blood work identifies leukocytosis, neutrophilia, and the Bombay blood group (absent H antigen as well as absent A and B antigens). What is this patient's diagnosis?

  • A. Chediak-Higashi syndrome
  • B. Leukocyte adhesion deficiency (LAD) Type II
  • C. CD18 deficiency
  • D. Griscelli syndrome
Correct Answer: B

Rationale: The correct answer is B: Leukocyte adhesion deficiency (LAD) Type II. This patient's symptoms of recurrent bacterial infections, leukocytosis, neutrophilia, short stature, and Bombay blood group (lack of H antigen) are characteristic of LAD Type II. In LAD Type II, there is a defect in fucose metabolism, leading to impaired leukocyte adhesion and migration, causing immune dysfunction. Chediak-Higashi syndrome (A) presents with oculocutaneous albinism, recurrent infections, and giant granules in leukocytes. CD18 deficiency (C) is a type of LAD characterized by mutations in the CD18 gene causing impaired integrin function. Griscelli syndrome (D) presents with silvery hair, immunodeficiency, and neurological deficits, not matching this patient's symptoms.

A nurse reviews the laboratory data for an older patient. The nurse would be most concerned about which finding?

  • A. Hematocrit of 35%
  • B. Hemoglobin of 11.8 g/dL
  • C. Platelet count of 400000/L
  • D. White blood cell (WBC) count of 2800/L
Correct Answer: D

Rationale: The correct answer is D: White blood cell (WBC) count of 2800/L. A low WBC count (leukopenia) in an older patient can indicate an impaired immune system, making them vulnerable to infections. This is concerning as older adults have a weakened immune response. A: Hematocrit of 35% is within the normal range. B: Hemoglobin of 11.8 g/dL is slightly low but not a major concern as long as it's not significantly lower. C: Platelet count of 400000/L is within the normal range and not typically a cause for concern.

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