Le6 REVIEW HEMATOLOGY Flashcards

1
Q

A 25-year-old woman presents with easy bruising and prolonged bleeding after minor cuts. Laboratory findings show normal or slightly prolonged aPTT, decreased VWF antigen, decreased VWF activity, and decreased factor VIII activity. Multimer analysis reveals a normal distribution but decreased quantity. What is the most likely type of von Willebrand Disease?

A. Type 1
B. Type 2A
C. Type 2B
D. Type 3

A

A. Type 1

🧠 High-Yield Rationale:

Type 1 VWD is the most common and mildest form of the disease.
It is due to a quantitative deficiency of von Willebrand factor (VWF), leading to decreased VWF antigen and activity but a normal multimer distribution.
Patients often have mild bleeding tendencies, such as easy bruising, prolonged bleeding after cuts, and menorrhagia.
aPTT is normal or slightly increased, and factor VIII activity is mildly reduced.

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2
Q

A 6-year-old child with a history of severe bleeding episodes presents to the clinic. Laboratory results show a markedly prolonged aPTT, very low VWF antigen, undetectable VWF activity, and severely reduced factor VIII activity. Multimer analysis reveals an absence of multimers. What is the most likely type of von Willebrand Disease?

A. Type 1
B. Type 2B
C. Type 2N
D. Type 3

A

D. Type 3

🧠 High-Yield Rationale:

Type 3 VWD is the most severe form and results from a complete absence of VWF.
aPTT is significantly prolonged due to the absence of factor VIII stabilization.
VWF antigen and activity are undetectable.
Multimers are absent on analysis.
Patients present early in life with severe bleeding, including hemarthroses, spontaneous mucosal bleeding, and post-surgical hemorrhage.

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3
Q

A 45-year-old woman presents with fatigue, pallor, and spoon-shaped nails. Laboratory results show ↓ serum iron, ↑ TIBC, ↓ transferrin saturation, ↓ ferritin, and ↑ soluble transferrin receptor. What is the most likely diagnosis?

A. Anemia of chronic disease
B. Acute phase reaction
C. Iron deficiency anemia
D. Iron overload

A

C. Iron deficiency anemia

High TIBC and low ferritin confirm IDA!

🧠 High-Yield Rationale:
- Iron deficiency anemia (IDA) is characterized by low iron stores, leading to:
- ↓ Serum iron (due to depletion)
- ↑ TIBC (transferrin) (compensatory increase to bind more iron)
- ↓ Transferrin saturation (less iron available for transport)
- ↓ Ferritin (low iron storage)
- ↑ Soluble transferrin receptor (increased demand for iron in erythropoiesis)
- Common symptoms: Fatigue, pallor, pica (craving for non-food items), koilonychia (spoon nails).

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4
Q

A 60-year-old male with rheumatoid arthritis presents with mild anemia. Lab results show ↓ serum iron, ↓ TIBC, ↓ transferrin saturation, and normal ferritin levels. What is the most likely diagnosis?

A. Iron deficiency anemia
B. Anemia of chronic disease
C. Acute phase reaction
D. Iron overload

A

B. Anemia of chronic disease

Low TIBC + High/Normal Ferritin = ACD!

🧠 High-Yield Rationale:
- Anemia of chronic disease (ACD) occurs due to inflammatory cytokines (e.g., IL-6) causing:
- ↓ Serum iron (trapped in macrophages)
- ↓ TIBC (transferrin production decreases as part of inflammatory response)
- ↓ Transferrin saturation (less circulating iron)
- Normal or ↑ Ferritin (iron is sequestered in storage but unavailable for use)
- Key distinction from IDA: Ferritin is normal or elevated, whereas IDA has low ferritin.

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5
Q

A 55-year-old patient presents with fever and elevated inflammatory markers. Lab results show ↓ serum iron, ↓ TIBC, ↓ transferrin saturation, and ↑ ferritin. What is the most likely cause?

A. Iron deficiency anemia
B. Anemia of chronic disease
C. Acute phase reaction
D. Iron overload

A

C. Acute phase reaction

Looks like ACD, but more transient!

(LOOK AT FERRITIN)

🧠 High-Yield Rationale:
- Inflammation (e.g., infection, trauma, malignancy) causes acute phase reactants like ferritin to increase, leading to:
- ↓ Serum iron (iron is sequestered to reduce bacterial access)
- ↓ TIBC (transferrin decreases due to inflammation)
- ↓ Transferrin saturation (low available iron)
- ↑ Ferritin (inflammatory response stores more iron intracellularly)
- Key distinction from ACD: Both have low serum iron and TIBC, but acute phase reactions are temporary and resolve once the inflammation subsides.

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6
Q

A 35-year-old male with hereditary hemochromatosis presents with skin hyperpigmentation, diabetes, and joint pain. Lab results show ↑ serum iron, N/↓ TIBC, ↑ transferrin saturation, and ↑ ferritin. What is the most likely diagnosis?

A. Iron deficiency anemia
B. Anemia of chronic disease
C. Acute phase reaction
D. Iron overload

A

D. Iron overload

High ferritin + High transferrin saturation = Iron overload!

🧠 High-Yield Rationale:
- Iron overload (e.g., hereditary hemochromatosis) results in excess iron absorption and deposition in organs, causing:
- ↑ Serum iron (iron accumulation)
- N/↓ TIBC (iron saturation is already high, so no compensatory increase)
- ↑ Transferrin saturation (>50% suggests iron overload)
- ↑ Ferritin (reflecting high iron storage)
- Classic symptoms: “Bronze diabetes” (skin pigmentation + diabetes), joint pain, liver disease, heart failure.
- Management: Phlebotomy to remove excess iron.

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7
Q

A 14-year-old Hispanic male presents with fever, pallor, and hepatosplenomegaly. His laboratory findings show a WBC count of 120 × 10⁹/L, T-cell lineage ALL, and CNS involvement. Cytogenetics reveals a BCR-ABL1 (Philadelphia chromosome-positive) mutation. What is the most appropriate next step in treatment planning?

A. Standard chemotherapy alone
B. Close observation and supportive care
C. Allogenic stem cell transplant (SCT) in first complete remission
D. Low-intensity chemotherapy due to high risk of toxicity

A

C. Allogenic stem cell transplant (SCT) in first complete remission
High-Yield Rationale:
* High-risk factors:
* Age ≥10 years
* Male sex
* Hispanic ethnicity
* CNS involvement
* High WBC count (≥100 × 10%L in T-cell ALL)
* T-cell lineage (worse prognosis than B-cell ALL)
* Philadelphia chromosome-positive (BCR-ABL1 mutation)
* High-risk patients are candidates for allogenic SCT in first remission to prevent relapse.

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8
Q

A 9-year-old boy with ALL undergoes induction chemotherapy. His MRD at the end of induction therapy is 2 × 10⁻² nucleated cells. Based on MRD stratification, what does this indicate?

A. Low-risk disease, no additional treatment needed
B. High-risk disease, poor prognosis
C. Standard risk, continue with maintenance therapy
D. Favorable prognosis due to young age

A

B. High-risk disease, poor prognosis

🧠 High-Yield Rationale:

Minimal residual disease (MRD) at the end of induction therapy is the strongest predictor of relapse.
Persistence of MRD ≥10⁻³ is associated with a poor prognosis.
Patients with high MRD after induction require intensified therapy, including SCT consideration.
MRD is a stronger prognostic indicator than age or WBC count alone.

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9
Q

A 7-year-old child with B-cell ALL completes induction chemotherapy. Bone marrow examination shows <5% blast cells but MRD is 0.02% (2 leukemia cells in 10,000 BM cells). Which of the following best describes this patient’s response?

A. Complete hematologic remission (CHR)
B. Complete molecular remission (MRD negativity)
C. Molecular failure/MRD positivity
D. Hematologic relapse

A

C. Molecular failure/MRD positivity

🧠 High-Yield Rationale:
- Complete hematologic remission (CHR) = <5% blast cells in BM. ✅ (Patient meets this criterion)
- MRD negativity = ≤0.01% (1 leukemia cell in 10,000 BM cells). ❌ (This patient has 0.02% MRD, which is above the threshold for negativity)
- Molecular failure/MRD positivity = CHR but MRD >0.01% ✅ (This matches the patient’s status)
- Hematologic relapse = >5% blast cells in BM/blood ❌ (Patient has <5%, so this is incorrect)

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10
Q

A 12-year-old boy with T-cell ALL undergoes treatment and achieves complete hematologic remission. His MRD is undetectable at the end of consolidation therapy. What is the best classification of his response?

A. Complete hematologic remission (CHR)
B. Complete molecular remission/MRD negativity
C. Molecular relapse/MRD positivity
D. Hematologic relapse

A

B. Complete molecular remission/MRD negativity

🧠 High-Yield Rationale:
- MRD negativity is the best predictor of long-term remission.
- Complete hematologic remission (CHR) alone means <5% blast cells in BM, but MRD negativity is stricter (≤0.01% leukemia cells).
- Undetectable MRD indicates complete molecular remission.

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11
Q

A 9-year-old girl with ALL initially achieves MRD negativity but later develops MRD positivity (0.015%), despite still having <5% blast cells in BM. What is the correct classification?

A. Complete molecular remission
B. Molecular relapse/MRD positivity
C. Hematologic relapse
D. Complete hematologic remission (CHR)

A

B. Molecular relapse/MRD positivity

🧠 High-Yield Rationale:
- Molecular relapse/MRD positivity occurs when a patient previously had MRD negativity but now has detectable leukemia cells, even if blast count remains <5% in BM.
- Hematologic relapse requires >5% blast cells, which this patient does not have.

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12
Q

A 6-year-old boy with ALL is being monitored after induction chemotherapy. His latest bone marrow biopsy shows 6% blast cells in BM. What is the most accurate classification?

A. Molecular failure/MRD positivity
B. Complete hematologic remission (CHR)
C. Molecular relapse/MRD positivity
D. Hematologic relapse

A

D. Hematologic relapse

🧠 High-Yield Rationale:
- Hematologic relapse = >5% blast cells in BM or blood.
- This patient has 6% blasts, confirming relapse.

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13
Q

A 10-year-old girl with B-cell ALL has completed treatment and achieved complete remission. However, a recent bone marrow assessment shows 2% blast cells, and MRD is 0.03%. What is her classification?

A. Complete molecular remission
B. Molecular failure/MRD positivity
C. Hematologic relapse
D. Complete hematologic remission

A

B. Molecular failure/MRD positivity

🧠 High-Yield Rationale:
- <5% blast cells = NOT a hematologic relapse.
- MRD >0.01% = Molecular failure.

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14
Q
  1. Which subtype of B-ALL is associated with Burkitt’s lymphoma and expresses surface IgM?
    A. Common ALL
    B. Pro B-ALL
    C. Pre-B ALL
    D. Mature B-ALL
A

D. Mature B-ALL
Rationale: Mature B-ALL expresses surface IgM (sIgM) and is linked to Burkitt’s lymphoma.

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15
Q
  1. What is the most common subtype of B-lineage Acute Lymphoblastic Leukemia (B-ALL)?
    A. Pro B-ALL
    B. Common ALL
    C. Pre-B ALL
    D. Mature B-ALL
A

B. Common ALL
Rationale: Common ALL accounts for 49% of cases, making it the most prevalent subtype of B-ALL.

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16
Q
  1. Which of the following best describes the immunophenotype of Common ALL?
    A. CD10-, TdT+, myeloid marker+
    B. CD10+, without markers of mature B cells (cytoplasmic or surface membrane Ig)
    C. CD10±, cyIg+
    D. CD10±, sIg+
A

B. CD10+, without markers of mature B cells (cytoplasmic or surface membrane Ig)
Rationale: Common ALL is defined by CD10 positivity while lacking mature B-cell markers such as cytoplasmic or surface Ig. This differentiates it from Pre-B ALL and Mature B-ALL.

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17
Q
  1. Which subtype of B-ALL was formerly known as non-T, non-B-ALL or Null-ALL?
    A. Common ALL
    B. Pre-B ALL
    C. Mature B-ALL
    D. Pro B-ALL
A

D. Pro B-ALL
Rationale: Pro B-ALL was previously referred to as non-T, non-B-ALL or Null-ALL and is characterized by HLA-DR and CD19 positivity without additional differentiation markers.

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18
Q
  1. How is mixed or biphenotypic leukemia managed?
    A. Standard AML chemotherapy
    B. Standard ALL chemotherapy only
    C. Pediatric ALL protocol followed by AML consolidation
    D. No specific treatment guidelines
A

C. Pediatric ALL protocol followed by AML consolidation
Rationale: Mixed or biphenotypic leukemias express both myeloid and lymphoid lineage markers and are treated with an initial pediatric ALL protocol followed by AML consolidation therapy.

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19
Q
  1. Which subtype of T-ALL is also referred to as “early T precursor ALL” (ETP-ALL)?
    A. Early Pro/ Pre T-ALL
    B. Cortical T-ALL
    C. Mature T-ALL
    D. Burkitt’s lymphoma
A

A. Early Pro/ Pre T-ALL
Rationale: Early Pro/ Pre T-ALL is also called “early T precursor ALL” (ETP-ALL) and is characterized by the absence of CD1a and CD8, weak CD5 expression, and at least one myeloid/stem cell marker.

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20
Q
  1. Which of the following markers is universally expressed in all cases of T-lineage ALL (T-ALL)?
    A. CD19 and CD10
    B. HLA-DR and TdT
    C. CD7 and cytoplasmic or surface CD3
    D. MPO and CD34
A

C. CD7 and cytoplasmic or surface CD3
Rationale: All cases of T-ALL express T-cell antigen CD7 and either cytoplasmic (cyCD3) or surface (sCD3) CD3, which are essential for confirming T-cell lineage.

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21
Q
  1. Which treatment approach is used for Burkitt’s leukemia?
    A. Low-dose chemotherapy with maintenance therapy
    B. Short intensive chemotherapy with rituximab, no SCT, no maintenance
    C. Pediatric-inspired chemotherapy with frequent SCT
    D. Less intensive chemotherapy with immunotherapy
A

B. Short intensive chemotherapy with rituximab, no SCT, no maintenance
Rationale: Burkitt’s leukemia is treated with short intensive chemotherapy combined with rituximab, without SCT or maintenance therapy, achieving an 80-90% survival rate.

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22
Q
  1. What is the cure rate for frail patients >70-75 years old with ALL?
    A. ≥70-80%
    B. 50-60%
    C. ~30%
    D. ≤10%
A

D. ≤10%
Rationale: Frail elderly patients >70-75 years old have a ≤10% survival rate, highlighting the poor prognosis in this age group.

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23
Q
  1. What is the most common cause of noncancer death among childhood cancer survivors?
    A. Pulmonary fibrosis
    B. Cardiovascular dysfunction
    C. Secondary malignancies
    D. Renal failure
A

B. Cardiovascular dysfunction
Rationale: Cardiovascular dysfunction is the leading cause of noncancer death in childhood cancer survivors due to treatment-related cardiac toxicity.

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24
Q
  1. Which class of chemotherapeutic agents is most commonly associated with cardiac toxicity?
    A. Alkylating agents
    B. Anthracyclines
    C. Platinum-based agents
    D. Antimetabolites
A

B. Anthracyclines
Rationale: Anthracyclines (e.g., doxorubicin, daunorubicin, epirubicin, idarubicin) are the most common drugs causing cardiac toxicity, often leading to congestive heart failure (CHF).

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7. What is the most common early-phase radiation-induced pulmonary complication? A. Pulmonary fibrosis B. Acute pneumonitis C. Pleural effusion D. Pneumonia
B. Acute pneumonitis Rationale: Acute pneumonitis occurs within 4 weeks post-radiation and can extend beyond the radiation field.
26
8. What is the most common late-phase complication of radiation-induced pulmonary toxicity? A. Acute pneumonitis B. Pulmonary fibrosis C. Pleural effusion D. Interstitial lung disease
B. Pulmonary fibrosis Rationale: Pulmonary fibrosis is the most common late complication following chest radiation.
27
9. Which of the following is NOT a major risk factor for radiation-induced pulmonary dysfunction? A. Advanced age B. Smoking C. Radiation dose D. High BMI
D. High BMI Rationale: Advanced age, smoking, high radiation dose, and preexisting lung conditions increase the risk, but high BMI is not a direct risk factor.
28
10. What is the most sensitive test for detecting radiation-induced pulmonary dysfunction? A. Chest X-ray B. Pulmonary function test (PFT) C. DLco (Diffusion capacity of the lungs for carbon monoxide) D. High-resolution CT scan
C. DLco (Diffusion capacity of the lungs for carbon monoxide) Rationale: DLco is the most sensitive test for early detection of radiation-induced pulmonary dysfunction.
29
2. Hodgkin’s lymphoma survivors are at increased risk for which secondary malignancy? A. B-cell non-Hodgkin lymphoma B. Thyroid cancer C. Bladder cancer D. Sarcomas
A. B-cell non-Hodgkin lymphoma Rationale: Hodgkin’s lymphoma survivors are at higher risk for B-cell non-Hodgkin lymphoma.
30
4. Which chemotherapy agents are most associated with secondary malignancies? A. Antimetabolites B. Alkylating agents C. Platinum-based agents D. Vinca alkaloids
B. Alkylating agents Rationale: Alkylating agents increase the risk of thyroid, lung, breast, bladder cancer, and sarcomas.
31
5. Which of the following drugs is associated with a 1-2% risk of endometrial cancer? A. Tamoxifen B. Rituximab C. Methotrexate D. Paclitaxel
A. Tamoxifen Rationale: Tamoxifen carries a 1-2% risk of endometrial cancer.
32
7. Which cancer treatments are most associated with infertility? A. Monoclonal antibodies B. Radiation to the head, neck, pelvis, and alkylating agents C. Topoisomerase inhibitors D. Anti-angiogenic agents
B. Radiation to the head, neck, pelvis, and alkylating agents Rationale: These treatments pose the highest risk for infertility in both males and females.
33
2. Which class of chemotherapy agents is most associated with stocking-glove neuropathy? A. Platinum-based agents B. Vinca alkaloids C. Alkylating agents D. Monoclonal antibodies
B. Vinca alkaloids Rationale: Vinca alkaloids cause dose-related stocking-glove neuropathy, autonomic neuropathy, and cranial nerve palsies.
34
3. Which chemotherapy agent is most commonly associated with sensorimotor neuropathy and hearing loss? A. Methotrexate B. Cisplatin C. Vincristine D. Doxorubicin
B. Cisplatin Rationale: Cisplatin is known to cause sensorimotor neuropathy and ototoxicity (hearing loss).
35
4. What is Lhermitte’s sign, a common radiation-induced neurological dysfunction? A. Progressive cerebellar ataxia B. Loss of deep tendon reflexes C. Electric shock sensation down the spine upon neck flexion D. Sudden onset hemiparesis
C. Electric shock sensation down the spine upon neck flexion Rationale: Lhermitte’s sign is a self-limiting condition occurring 6-12 weeks post-radiation and resolves over time.
36
5. Which of the following is the most severe form of radiation-induced CNS injury? A. Lhermitte’s sign B. Diffuse radiation injury C. Necrotizing encephalopathy D. Posterior reversible encephalopathy syndrome (PRES)
C. Necrotizing encephalopathy Rationale: Necrotizing encephalopathy is the most severe form of radiation injury and is associated with methotrexate.
37
1. What is the most common late complication affecting the gastrointestinal system in cancer survivors? A. Pancreatitis B. Malabsorption C. Gastroesophageal reflux disease (GERD) D. Bowel obstruction
B. Malabsorption Rationale: Malabsorption is one of the most common late complications in cancer survivors, along with colitis and chronic diarrhea.
38
3. Which chemotherapy drug is most associated with reversible kidney damage? A. Cyclophosphamide B. Ifosfamide C. Cisplatin D. Doxorubicin
C. Cisplatin Rationale: Cisplatin is known to cause reversible kidney damage, primarily acute tubular necrosis.
39
4. Which chemotherapy agents are most commonly associated with hemorrhagic cystitis? A. Methotrexate and 5-FU B. Cyclophosphamide and Ifosfamide C. Vincristine and Paclitaxel D. Cisplatin and Carboplatin
B. Cyclophosphamide and Ifosfamide Rationale: Cyclophosphamide and Ifosfamide can cause hemorrhagic cystitis, a severe bladder toxicity.
40
1. What is the preferred first-line IV iron chelator for transfusion-dependent iron overload? A. Deferasirox B. Deferiprone C. Deferoxamine D. Ferric citrate
C. Deferoxamine Rationale: Deferoxamine (Desferal) is the first-line IV iron chelator, given via slow IV or subcutaneous infusion.
41
5. What is the most significant advantage of Deferasirox over Deferoxamine? A. Higher efficacy B. Oral administration (better compliance) C. No need for monitoring D. Faster onset of action
B. Oral administration (better compliance) Rationale: Deferasirox is an oral iron chelator, making it easier to use and improving patient compliance, especially in older children and adults.
42
1. What is the most common type of anemia? A. Hemolytic anemia B. Hypoproliferative anemia C. Megaloblastic anemia D. Aplastic anemia
B. Hypoproliferative anemia Rationale: Hypoproliferative anemias (e.g., iron deficiency, anemia of chronic disease) are the most common type of anemia.
43
2. What is the most common cause of acute anemia? A. Blood loss B. Iron deficiency C. Bone marrow failure D. Chronic disease
A. Blood loss Rationale: Acute anemia is most commonly due to blood loss, such as GI bleeding or trauma.
44
3. What is the most common cause of chronic anemia? A. Iron deficiency B. Vitamin B12 deficiency C. Folate deficiency D. Hemolysis
A. Iron deficiency Rationale: Iron deficiency anemia is the most common cause of chronic anemia, particularly in women and children.
45
4. What is the most common presenting symptom of anemia? A. Dizziness B. Pallor C. Fatigue D. Tachycardia
C. Fatigue Rationale: Fatigue is the most common symptom of anemia due to reduced oxygen delivery to tissues.
46
5. What is the most common sign of severe anemia? A. Splenomegaly B. Jaundice C. Mucosal pallor D. Petechiae
C. Mucosal pallor Rationale: Pallor (seen in the mucosa, conjunctiva, and palmar creases) is the most common sign of severe anemia.
47
6. What is the most common genetic disorder worldwide? A. Cystic fibrosis B. Thalassemia C. Hemophilia A D. Sickle cell disease
B. Thalassemia Rationale: Thalassemia is the most common genetic disorder worldwide, especially in Mediterranean, African, and Southeast Asian populations.
48
7. What is the most common structural hemoglobinopathy? A. HbC disease B. HbE disease C. HbS (Sickle Cell Disease) D. α-thalassemia
C. HbS (Sickle Cell Disease) Rationale: HbS (Sickle Cell Disease) is the most common structural hemoglobinopathy, resulting from a point mutation in the β-globin gene.
49
8. What is the most common complication of Sickle Cell Disease (SCD)? A. Splenic sequestration crisis B. Painful vaso-occlusive crisis C. Aplastic crisis D. Acute chest syndrome
B. Painful vaso-occlusive crisis Rationale: Painful vaso-occlusive crisis is the most common and hallmark complication of Sickle Cell Disease, triggered by dehydration, infection, or hypoxia.
50
1. What is the most common inherited bleeding disorder? A. Hemophilia A B. Von Willebrand Disease (VWD) C. Bernard-Soulier Syndrome D. Factor VII deficiency
B. Von Willebrand Disease (VWD) Rationale: Von Willebrand Disease (VWD) is the most common inherited bleeding disorder, due to deficiency or dysfunction of von Willebrand factor (vWF).
51
2. What is the first-line treatment for mild Von Willebrand Disease (VWD)? A. Factor VIII concentrate B. Fresh Frozen Plasma (FFP) C. Desmopressin (DDAVP) D. Heparin
C. Desmopressin (DDAVP) Rationale: Desmopressin (DDAVP) increases vWF release from endothelial cells, making it the first-line treatment for mild VWD.
52
3. What is the most common site of bleeding in Hereditary Hemorrhagic Telangiectasia? A. Gastrointestinal tract B. Brain C. Lungs D. Nose (Epistaxis)
D. Nose (Epistaxis) Rationale: Epistaxis (nosebleeds) is the most common site of bleeding in Hereditary Hemorrhagic Telangiectasia (HHT).
53
4. What is the most common cause of thrombocytopenia? A. Drug-induced (Heparin, Vancomycin, Quinine, Sulfonamides) B. Immune Thrombocytopenic Purpura (ITP) C. Aplastic anemia D. Disseminated Intravascular Coagulation (DIC)
A. Drug-induced (Heparin, Vancomycin, Quinine, Sulfonamides) Rationale: Drug-induced thrombocytopenia is the most common cause of low platelet counts, especially due to heparin, vancomycin, quinine, and sulfonamides.
54
5. What is the most common thrombotic microangiopathy (TMA) in adults? A. Hemolytic Uremic Syndrome (HUS) B. Disseminated Intravascular Coagulation (DIC) C. Thrombotic Thrombocytopenic Purpura (TTP) D. Heparin-Induced Thrombocytopenia (HIT)
C. Thrombotic Thrombocytopenic Purpura (TTP) Rationale: TTP is the most common TMA in adults, caused by ↓ ADAMTS13, leading to widespread platelet aggregation and microvascular thrombosis. Plasma exchange is the treatment of choice.
55
6. What is the most common thrombotic microangiopathy (TMA) in children? A. Hemolytic Uremic Syndrome (HUS) B. Thrombotic Thrombocytopenic Purpura (TTP) C. Heparin-Induced Thrombocytopenia (HIT) D. Paroxysmal Nocturnal Hemoglobinuria (PNH)
A. Hemolytic Uremic Syndrome (HUS) Rationale: HUS is the most common TMA in children, often caused by E. coli O157:H7 infection. Supportive care is the main treatment.
56
7. What is the most common cause of DIC? A. Sepsis (Gram-negative bacteria) B. Trauma C. Pregnancy-related complications D. Malignancy
A. Sepsis (Gram-negative bacteria) Rationale: Sepsis, especially from Gram-negative bacteria, is the most common cause of DIC, leading to widespread coagulation activation.
57
8. Which hematologic malignancy is most commonly associated with DIC? A. Acute Myeloid Leukemia (AML) B. Acute Promyelocytic Leukemia (APL) C. Chronic Lymphocytic Leukemia (CLL) D. Hodgkin Lymphoma
B. Acute Promyelocytic Leukemia (APL) Rationale: Acute Promyelocytic Leukemia (APL) is the most common hematologic malignancy causing DIC due to the release of pro-coagulant granules.
58
9. Which platelet receptor is responsible for platelet adhesion? A. GpIb B. GpIIb/IIIa C. GpVI D. PAR-1
A. GpIb Rationale: GpIb binds vWF, mediating platelet adhesion to damaged endothelium. Bernard-Soulier Syndrome (GpIb defect, giant platelets)
59
10. Which platelet receptor is responsible for platelet aggregation? A. GpIb B. GpIIb/IIIa C. GpVI D. P-selectin
B. GpIIb/IIIa Rationale: GpIIb/IIIa binds fibrinogen, facilitating platelet aggregation and clot formation. Glanzmann’s Thrombasthenia: Absence of Gp IIb/IIIa receptor, affecting platelet aggregation.
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2. Which therapy is associated with increased iron demand? A. Corticosteroids B. Erythropoietin therapy C. Heparin therapy D. Beta-blockers
B. Erythropoietin therapy Rationale: Erythropoietin therapy stimulates red blood cell production, increasing the demand for iron.
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4. Which medical treatment can lead to iron deficiency due to increased blood loss? A. Dialysis B. Phlebotomy for polycythemia vera C. Blood transfusion D. Intravenous iron therapy
B. Phlebotomy for polycythemia vera Rationale: Phlebotomy, used in polycythemia vera, removes blood regularly, leading to iron loss.
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5. Which gastrointestinal disorder is associated with iron malabsorption? A. Crohn’s disease B. Peptic ulcer disease C. Gastroesophageal reflux disease (GERD) D. Irritable bowel syndrome (IBS)
C. Gastrectomy Rationale: Gastrectomy and bariatric surgery can impair iron absorption, increasing the risk of iron deficiency anemia.
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A 32-year-old woman presents with fatigue, pallor, and spoon-shaped nails. Laboratory findings show serum iron <30 µg/dL, TIBC >360 µg/dL, ferritin <15 µg/L, and percent saturation <10%. Peripheral smear reveals microcytic, hypochromic RBCs. What is the most likely diagnosis? A. Iron deficiency anemia B. Anemia of chronic disease C. Thalassemia D. Sideroblastic anemia
A. Iron deficiency anemia 🧠 High-Yield Rationale: Microcytic, hypochromic RBCs suggest a microcytic anemia. Low serum iron (<30 µg/dL) and ferritin (<15 µg/L) with high TIBC (>360 µg/dL) confirm iron deficiency anemia (IDA). Percent saturation <10% further supports IDA. Common causes: Chronic blood loss (e.g., heavy menstruation, GI bleeding), inadequate dietary intake, malabsorption.
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A 22-year-old male of Mediterranean descent presents with lifelong mild anemia and target cells on a peripheral smear. Lab findings show normal to high serum iron, normal TIBC, ferritin 200 µg/L, and percent saturation 40%. Hemoglobin electrophoresis shows increased HbA2 and decreased HbA. What is the most likely diagnosis? A. Iron deficiency anemia B. Anemia of chronic disease C. Beta-thalassemia trait D. Sideroblastic anemia
C. Beta-thalassemia trait 🧠 High-Yield Rationale: Thalassemia presents with microcytic anemia but normal to high serum iron and ferritin levels. Target cells on smear are characteristic. Hemoglobin electrophoresis shows increased HbA2 (>3.5%), confirming beta-thalassemia trait. Key distinction: Unlike IDA, thalassemia does NOT have low ferritin or high TIBC.
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A 32-year-old woman presents with fatigue, pallor, and brittle nails. Laboratory results show MCV of 72 fL, serum iron <30 µg/dL, TIBC >360 µg/dL, ferritin <15 µg/L, and percent saturation <10%. What is the most likely diagnosis? A. Iron deficiency anemia B. Anemia of chronic disease C. Anemia due to renal disease D. Hypometabolic state anemia
A. Iron deficiency anemia 🧠 High-Yield Rationale: Microcytic anemia (MCV 60-90 fL) suggests a problem with iron availability. Low serum iron (<30 µg/dL) with high TIBC (>360 µg/dL) and very low ferritin (<15 µg/L) confirms iron deficiency anemia (IDA). Iron stores are absent (0+), further supporting IDA. Common causes: Chronic blood loss (menorrhagia, GI bleeding), poor diet, malabsorption (e.g., celiac disease).
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1. Epstein-Barr virus (EBV) is most strongly associated with which of the following lymphoid malignancies? A. Diffuse large B-cell lymphoma B. Non- Hodgkin’s lymphoma C. Burkitt’s lymphoma D. Hairy cell leukemia
C. Burkitt’s lymphoma Rationale: EBV is highly associated with Burkitt’s lymphoma, particularly in endemic African cases.
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5. Which of the following viruses is most strongly associated with Adult T-cell leukemia/lymphoma? A. Epstein-Barr virus (EBV) B. HTLV-1 C. HIV D. Hepatitis C virus
B. HTLV-1 Rationale: HTLV-1 (Human T-cell Lymphotropic Virus-1) is strongly linked to Adult T-cell leukemia/lymphoma, primarily in Japan and the Caribbean.
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6. HIV increases the risk of which of the following lymphoid malignancies? A. Chronic lymphocytic leukemia B. Diffuse large B-cell lymphoma C. Waldenström’s macroglobulinemia D. Follicular lymphoma
B. Diffuse large B-cell lymphoma Rationale: HIV-associated immunosuppression increases the risk of diffuse large B-cell lymphoma (DLBCL).
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8. Hepatitis C virus (HCV) is most associated with which lymphoid malignancy? A. Lymphoplasmacytic lymphoma B. Follicular lymphoma C. Chronic lymphocytic leukemia D. Marginal zone lymphoma
A. Lymphoplasmacytic lymphoma Rationale: HCV infection is linked to lymphoplasmacytic lymphoma, which can progress to Waldenström’s macroglobulinemia.
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9. Helicobacter pylori infection is most strongly linked to which lymphoma? A. Hodgkin’s lymphoma B. Gastric MALT lymphoma C. Splenic marginal zone lymphoma D. Anaplastic large cell lymphoma
B. Gastric MALT lymphoma Rationale: Helicobacter pylori infection is a well-established cause of gastric MALT lymphoma, which may regress after antibiotic treatment.
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10. Human herpesvirus 8 (HHV-8) is most commonly associated with which of the following malignancies? A. Follicular lymphoma B. Primary effusion lymphoma C. Mantle cell lymphoma D. Acute lymphoblastic leukemia
B. Primary effusion lymphoma Rationale: HHV-8 is the primary cause of primary effusion lymphoma, often seen in HIV/AIDS patients.
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1. Which of the following best describes Stage I lymphoma? A. Involvement of lymph node regions on both sides of the diaphragm B. Involvement of a single lymph node region or a single extranodal site C. Diffuse involvement of multiple extranodal organs D. Involvement of multiple lymph node regions on one side of the diaphragm
B. Involvement of a single lymph node region or a single extranodal site Rationale: Stage I lymphoma is characterized by localized involvement of one lymph node region (I) or a single extranodal site (IE).
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2. What defines Stage II lymphoma? A. Two or more lymph node regions on the same side of the diaphragm B. Lymph node involvement on both sides of the diaphragm C. Diffuse extranodal disease D. Single lymph node involvement
A. Two or more lymph node regions on the same side of the diaphragm Rationale: Stage II lymphoma involves two or more lymph node regions or lymphatic structures on the same side of the diaphragm, with possible extralymphatic organ involvement (IIE).
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3. What is the defining characteristic of Stage III lymphoma? A. Involvement of lymph nodes on both sides of the diaphragm B. A single extranodal site without lymph node involvement C. Widespread dissemination to multiple extranodal organs D. Involvement of a single lymph node group
A. Involvement of lymph nodes on both sides of the diaphragm Rationale: Stage III lymphoma is defined by lymph node involvement on both sides of the diaphragm, with possible spleen (IIIS) or extranodal organ involvement (IIIE, IIIES).
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4. What is the hallmark of Stage IV lymphoma? A. Multiple lymph node regions on one side of the diaphragm B. Disseminated extranodal involvement with or without lymph node disease C. Single extranodal involvement D. Only spleen involvement
B. Disseminated extranodal involvement with or without lymph node disease Rationale: Stage IV lymphoma is diffuse or disseminated with multiple extranodal organs involved, with or without lymph node disease.
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5. What is the significance of an "A" or "B" designation in Ann Arbor staging? A. A = Aggressive lymphoma, B = Indolent lymphoma B. A = No systemic symptoms, B = Presence of systemic symptoms C. A = No bone marrow involvement, B = Bone marrow involvement D. A = Early-stage disease, B = Late-stage disease
B. A = No systemic symptoms, B = Presence of systemic symptoms Rationale: Ann Arbor stages are further categorized as "A" (no systemic symptoms) or "B" (presence of fevers, night sweats, and/or weight loss >10% over 6 months).
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6. Which of the following is considered a systemic B symptom in lymphoma? A. Persistent cough B. Pruritus C. Weight loss >10% in 6 months D. Lymphadenopathy
C. Weight loss >10% in 6 months Rationale: Systemic B symptoms include unexplained fever, night sweats, and weight loss >10% of body weight over 6 months.
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1. Which of the following is the most common drug-induced cause of thrombocytopenia? A. Amoxicillin B. Heparin C. Metformin D. Warfarin
B. Heparin Rationale: Heparin-Induced Thrombocytopenia (HIT) is a well-known cause of drug-induced thrombocytopenia, often leading to thrombosis rather than bleeding.
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2. Which NSAID is associated with drug-induced thrombocytopenia? A. Acetaminophen B. Naproxen C. Ibuprofen D. AOTA
D. AOTA Rationale: Ibuprofen, Acetaminophen, and Naproxen have been reported to cause drug-induced thrombocytopenia. Nonsteroidal Anti-Inflammatory Drugs (NSAIDs) Acetaminophen Ibuprofen Naproxen
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4. Which antiarrhythmic drug is associated with thrombocytopenia? A. Amiodarone B. Naproxen C. Lidocaine D. AOTA
A. Amiodarone Rationale: Amiodarone, a potent antiarrhythmic, has been linked to drug-induced thrombocytopenia. Cardiovascular Drugs Amiodarone (Antiarrhythmic) Amlodipine (Calcium channel blocker) Furosemide (Loop diuretic) Rosiglitazone (Thiazolidinedione - used for diabetes)
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1. What defines Complete Hematologic Remission (CHR) in leukemia? A. No leukemia cells detectable by flow cytometry B. No leukemia cells detectable by molecular testing C. Leukemic cells not detectable by light microscopy (<5% blasts in bone marrow) D. No leukemia cells present in peripheral blood
C. Leukemic cells not detectable by light microscopy (<5% blasts in bone marrow) Rationale: CHR is defined as <5% blast cells in the bone marrow (BM) by light microscopy, indicating no visible leukemia.
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2. What is the criterion for Complete Molecular Remission (MRD Negativity)? A. <5% blasts in bone marrow B. ≤0.01% leukemia cells (≤1 cell in 10,000 BM cells) C. No leukemia cells in the peripheral blood D. Absence of B symptoms
B. ≤0.01% leukemia cells (≤1 cell in 10,000 BM cells) Rationale: MRD negativity means leukemia cells are undetectable at a molecular level (≤0.01%), indicating a deep remission.
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3. A patient is in Complete Hematologic Remission but has detectable leukemia cells at >0.01% on molecular testing. What is the diagnosis? A. Complete molecular remission B. Molecular failure/MRD positivity C. Hematologic relapse D. Partial remission
B. Molecular failure/MRD positivity Rationale: Molecular failure (MRD positivity) means the patient is in CHR but has detectable MRD >0.01%, indicating a higher risk of relapse.
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4. What defines Molecular Relapse in leukemia? A. Reappearance of blasts in peripheral blood B. >5% blasts in bone marrow C. Detectable leukemia cells in BM despite prior molecular remission D. Presence of B symptoms
C. Detectable leukemia cells in BM despite prior molecular remission Rationale: Molecular relapse means the patient had prior MRD negativity but now has detectable leukemia cells in the BM (<5%), suggesting early recurrence.
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5. What is the defining criterion for Hematologic Relapse in leukemia? A. Detectable MRD >0.01% B. >5% blasts in BM or peripheral blood C. Symptoms of fever, weight loss, and night sweats D. Persistent neutropenia
B. >5% blasts in BM or peripheral blood Rationale: Hematologic relapse is defined as >5% blasts in the bone marrow or blood, indicating active leukemia recurrence.