ATI Hematologic System

ATI Hematologic System Related

Review ATI Hematologic System related questions and content

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 because the clinical presentation suggests acute promyelocytic leukemia (APL). APL is characterized by Auer rod-containing granular blasts, which are positive for CD33 and negative for HLA-DR. The presence of coagulopathy with elevated D-dimer and oozing blood indicates a high risk of disseminated intravascular coagulation (DIC), a common complication of APL. Immediate treatment with all-trans retinoic acid (ATRA) is crucial in APL to induce differentiation of the leukemic cells and prevent further coagulopathy. Aggressively managing the coagulopathy with blood product support is also important to stabilize the patient.

Choice A is incorrect because a lumbar puncture is not necessary for APL diagnosis, and immediate induction chemotherapy with ATRA is the standard of care. Choice C is incorrect because dexamethasone and hydroxyurea are not the first-line therapies for APL.

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Which of the following anemia is associated with splenomegaly:

  • A. chronic renal failure
  • B. aplastic anemia
  • C. hereditary spherocytosis
  • D. sickle cell anaemia
Correct Answer: C

Rationale: The correct answer is C, hereditary spherocytosis. This condition is characterized by a defect in red blood cell membrane proteins, leading to their premature destruction in the spleen, causing splenomegaly. In contrast, chronic renal failure (A) does not typically present with splenomegaly. Aplastic anemia (B) is characterized by bone marrow failure, not splenomegaly. Sickle cell anemia (D) is associated with splenomegaly in early childhood due to sequestration of damaged red blood cells, but splenomegaly usually resolves in later life.

A female patient's hematocrit level is 50% and oxygen saturation is 98% on room air. What should the nurse suspect as being the cause for this patient's hematocrit level?

  • A. Dehydration
  • B. Chronic renal failure
  • C. Bone marrow suppression
  • D. Bleeding esophageal varices
Correct Answer: A

Rationale: The correct answer is A: Dehydration. Dehydration can lead to hemoconcentration, resulting in an elevated hematocrit level. When a person is dehydrated, the plasma volume decreases, causing an increase in the proportion of red blood cells in the blood, leading to a higher hematocrit level. In this case, the patient's high hematocrit level of 50% is likely due to dehydration rather than the other options. Oxygen saturation being 98% on room air indicates adequate oxygenation, ruling out chronic renal failure, bone marrow suppression, and bleeding esophageal varices as potential causes for the high hematocrit level in this patient.

A 3-month-old female presents to the emergency room with vomiting and abdominal distension. She has a left-side abdominal mass, and an abdominal ultrasound confirms an 8-cm mass arising from the left kidney. Liver lesions are also noted. Nephrectomy is performed and reveals a histologic diagnosis of malignant rhabdoid tumor of the kidney (MRTK). Which of the following is not a true statement about the management of this patient?

  • A. Most patients with rhabdoid tumor of the kidney present in infancy.
  • B. Most patients with rhabdoid tumor of the kidney present with metastatic (stage III or IV) disease.
  • C. She has an excellent prognosis with surgery, chemotherapy, and radiation.
  • D. Germline testing for SMARCB1/INI1 mutation on chromosome 22 is recommended, with brain MRI every 3 months until she is 5 years old, if testing is germline positive for SMARCB1/INI1.
Correct Answer: C

Rationale: The correct answer is C: She has an excellent prognosis with surgery, chemotherapy, and radiation. This statement is not true because malignant rhabdoid tumor of the kidney (MRTK) has a poor prognosis, even with aggressive treatment. Here's a breakdown:
1. MRTK is an aggressive and rare tumor that often presents in infancy, supporting statement A.
2. Most patients with MRTK present with metastatic disease, indicating poor prognosis, aligning with statement B.
3. Germline testing for SMARCB1/INI1 mutation is essential due to the genetic predisposition associated with MRTK, supporting statement D.
In summary, statement C is incorrect as MRTK typically has a challenging clinical course despite comprehensive treatment approaches.

Sickle cell an. Is not complicated by:

  • A. papillary necrosis
  • B. pancreatitis
  • C. osteomyelitis
  • D. CHF
Correct Answer: B

Rationale: Step-by-step rationale for choice B (pancreatitis) being correct:
1. Sickle cell anemia primarily affects red blood cells, leading to vaso-occlusive crises.
2. Pancreatitis is not a known complication of sickle cell anemia.
3. Complications of sickle cell anemia commonly involve organs affected by sickling, such as the bones (osteomyelitis), kidneys (papillary necrosis), and heart (CHF).

Summary:
A: Papillary necrosis - Correct, as it can affect the kidneys in sickle cell anemia.
B: Pancreatitis - Correct, as it is not a typical complication of sickle cell anemia.
C: Osteomyelitis - Incorrect, as it can affect the bones in sickle cell anemia.
D: CHF - Incorrect, as it can affect the heart in sickle cell anemia.

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: The correct answer is C: Part of general pancytopenia. Secondary neutropenia in adults can occur as part of general pancytopenia, where there is a decrease in all blood cell types. This can be caused by various conditions such as infections, medications, autoimmune disorders, and bone marrow disorders.

Rationale:
A: Congenital is incorrect because congenital neutropenia refers to a condition present at birth, not secondary neutropenia.
B: Anti-hypertensive drugs may cause drug-induced neutropenia, but it is not the primary cause of secondary neutropenia in adults.
D: Familial neutropenia is a genetic disorder that runs in families and is not a common cause of secondary neutropenia in adults.

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