Anatomy of Hematologic System

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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.

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The nurse is assisting with the preparation of a blood transfusion for a patient. Which type of fluid should the nurse select to transfuse with the blood?

  • A. 0.9% normal saline
  • B. Dextrose 5% and water
  • C. Dextrose 5% and 0.9% normal saline
  • D. Dextrose 5% and 0.45% normal saline
Correct Answer: A

Rationale: Rationale: A 0.9% normal saline solution is the correct choice for blood transfusion because it is isotonic, which closely matches the osmolarity of blood. This helps prevent hemolysis and maintains the integrity of red blood cells during transfusion. Other choices such as B (dextrose 5% and water), C (dextrose 5% and 0.9% normal saline), and D (dextrose 5% and 0.45% normal saline) are incorrect because they do not provide the necessary isotonic environment for safe blood transfusion, risking hemolysis or other complications.

A nurse is reviewing the medication record of a client who is receiving alteplase following an acute myocardial infarction (MI). Which of the following medications should the nurse expect the client to be taking in addition to the alteplase?

  • A. Protamine
  • B. Desmopressin
  • C. Ferrous sulfate
  • D. Heparin
Correct Answer: D

Rationale: The correct answer is D: Heparin. Following an acute MI, a client receiving alteplase may also be prescribed heparin to prevent re-thrombosis and further clot formation. Alteplase is a thrombolytic agent that works by breaking down clots, while heparin is an anticoagulant that helps prevent new clots from forming. Protamine (choice A) is used to reverse the effects of heparin, not typically administered alongside alteplase. Desmopressin (choice B) is used for conditions like diabetes insipidus, unrelated to MI treatment. Ferrous sulfate (choice C) is an iron supplement, not indicated in this scenario.

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 60-year-old patient with chronic myeloid leukemia will be treated in the home setting and the nurse is preparing appropriate health education. What topic should the nurse emphasize?

  • A. The importance of adhering to the prescribed drug regimen
  • B. The need to ensure that vaccinations are up to date
  • C. The importance of daily physical activity
  • D. The need to avoid shellfish and raw foods
Correct Answer: A

Rationale: The correct answer is A: The importance of adhering to the prescribed drug regimen. This is crucial for managing chronic myeloid leukemia effectively. Non-adherence can lead to disease progression and treatment failure. Option B is important but not as critical as medication adherence. Option C is generally good for overall health but not specific to managing the disease. Option D is irrelevant to the management of chronic myeloid leukemia.

A 13-year-old boy presents to the emergency department with complaints of headache and visual changes. History reveals progressive dyspnea on exertion, generalized fatigue, and increased bruising. His labs are significant for a WBC of 350,000/mcL, of which 80% are reported to be blasts and appear to be myeloblasts without the presence of Auer rods. His hemoglobin is 7.2 g/dL, and his platelets are 18,000/mcL. A CT scan of the head shows a small intracerebral hemorrhage. His coags are normal. Which of the following is the most appropriate therapy?

  • A. Start induction chemotherapy.
  • B. Perform emergent leukapheresis followed the next day by induction chemotherapy.
  • C. Perform emergent leukapheresis plus hydroxyurea.
  • D. Provide emergent cranial radiation plus hydroxyurea followed the next day by induction chemotherapy.
Correct Answer: C

Rationale: The correct answer is C: Perform emergent leukapheresis plus hydroxyurea. In this scenario, the patient presents with symptoms suggestive of acute myeloid leukemia with hyperleukocytosis and intracerebral hemorrhage. The goal of emergent therapy is to rapidly reduce the high blast count to prevent further complications such as leukostasis and hemorrhage. Leukapheresis can provide immediate reduction in the blast count, while hydroxyurea can further decrease the WBC count. Starting induction chemotherapy immediately can lead to tumor lysis syndrome due to rapid cell destruction. Emergent cranial radiation is not the primary intervention for hyperleukocytosis. Performing leukapheresis alone without adjunctive therapy like hydroxyurea may not adequately control the blast count. Thus, the most appropriate initial therapy is emergent leukapheresis plus hydroxyurea to stabilize the patient before initiating induction chemotherapy.

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