ATI Hematologic System

ATI Hematologic System Related

Review ATI Hematologic System related questions and content

A 14-year-old Syrian male with beta thalassemia major has relocated to your community as a refugee. He has been receiving chronic transfusion therapy in Turkey for the past 3 years. On his first visit, you notice that his height is below the fifth percentile. He has skin discoloration and hepatosplenomegaly. His mother reports they have not had regular access to chelation therapy. Laboratory testing shows a serum ferritin of 6,200 ng/mL. A cardiac MRI shows grossly normal cardiac function but a T2* value of 9 ms. What is the most likely cause of his short stature?

  • A. Lack of regular blood transfusion causing growth failure
  • B. Cirrhosis and liver failure
  • C. Ineffective erythropoiesis and chronic anemia
  • D. Growth hormone deficiency due to iron deposition in the pituitary
Correct Answer: D

Rationale: The correct answer is D: Growth hormone deficiency due to iron deposition in the pituitary. Iron deposition in the pituitary gland can lead to impaired production and secretion of growth hormone, resulting in short stature. This is a known complication of beta thalassemia major. The patient's elevated serum ferritin levels and T2* value of 9 ms indicate iron overload, which can cause damage to organs including the pituitary gland. Choices A, B, and C are incorrect because the patient's growth failure is primarily due to the impact of iron deposition on the pituitary gland rather than lack of transfusion, cirrhosis, or ineffective erythropoiesis. Regular chelation therapy is essential to prevent iron overload and its complications in patients with beta thalassemia major.

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You examine a 10-year-old boy with severe aplastic anemia. He has no dysmorphic features and is at the 50th percentile for height and weight. Family history includes a sister with aplastic anemia unresponsive to anti-human thymocyte globulin (ATG) and cyclosporine who died early in the course of an unrelated donor hematopoietic stem cell transplant complicated by severe mucositis and transplant-related organ toxicities. There are no other siblings. A cousin died of acute myeloid leukemia at age 5 years. A peripheral blood sample test for Fanconi anemia is negative with no increased chromosomal breaks in response to diepoxylbutane or mitomycin C. Which of the following is the most important next step in management?

  • A. Administer ATG and cyclosporine.
  • B. Search for a donor for matched unrelated transplant.
  • C. Send a bone marrow aspirate for Fanconi anemia testing.
  • D. Send a skin fibroblast culture for Fanconi anemia testing.
Correct Answer: D

Rationale: The correct answer is D: Send a skin fibroblast culture for Fanconi anemia testing. This is the most important next step in management because the patient's family history, particularly the sister's unresponsiveness to ATG and cyclosporine and the cousin's history of leukemia, raises suspicion for Fanconi anemia. Testing for Fanconi anemia is crucial as it is an inherited bone marrow failure syndrome that predisposes individuals to aplastic anemia and leukemia. Skin fibroblast culture is the preferred test for diagnosing Fanconi anemia as it can detect chromosomal abnormalities indicative of the disease. Options A and B are not appropriate as the patient's sister did not respond to ATG and cyclosporine, and searching for a donor for transplant without confirming the underlying cause of aplastic anemia could lead to transplant failure. Option C is also not the best choice as the peripheral blood sample test for Fanconi anemia was already negative, and a skin fib

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: Rationale:
1. Blinatumomab is designed to engage T cells to target CD19 antigen on B-lymphoblasts.
2. CD19 is a specific marker expressed on B cells, making it a target for Blinatumomab.
3. CD10 is not targeted by Blinatumomab and is commonly found on early B-cell precursors.
4. CD15 and CD20 are not targeted by Blinatumomab and have different functions unrelated to B-lymphoblasts.

Summary:
- CD19 is the correct answer as it is the specific target for Blinatumomab on B-lymphoblasts.
- CD10, CD15, and CD20 are incorrect as they are not the targeted antigens for Blinatumomab and have different roles in immune function.

A 15-year-old female presents with 1 month of fatigue and 3 days of chest pain and shortness of breath. Her physical exam is unremarkable. A chest x-ray shows a large mediastinal mass that is greater than 33% of the diameter of her chest cavity. A biopsy shows nodular sclerosing, classic Hodgkin lymphoma (cHL). Metastatic workup at diagnosis, including CT scan of neck, chest, abdomen, and pelvis and PET scan, shows no other site of disease. According to the Ann Arbor staging system, the patient has which stage of cHL?

  • A. Stage I
  • B. Stage II
  • C. Stage III
  • D. Stage IV
Correct Answer: A

Rationale: The correct answer is A: Stage I. In the Ann Arbor staging system for Hodgkin lymphoma, Stage I indicates involvement of a single lymph node region or a single extralymphatic organ or site. In this case, the patient's disease is limited to the mediastinum without involvement of other lymph nodes or organs. The large mediastinal mass is considered as a single site of disease. The absence of disease involvement in other areas based on the metastatic workup supports the classification as Stage I. The other choices (B, C, D) are incorrect because they represent more extensive disease involving multiple lymph node regions or extralymphatic organs, which is not the case in this patient.

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.

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.

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