Le6 REVIEW HEMATOLOGY Flashcards
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. 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.
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
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.
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
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).
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
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.
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
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.
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
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.
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
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.
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
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.
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
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)
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
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. ✅
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)
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.
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
D. Hematologic relapse
🧠 High-Yield Rationale:
- Hematologic relapse = >5% blast cells in BM or blood. ✅
- This patient has 6% blasts, confirming relapse.
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
B. Molecular failure/MRD positivity
🧠 High-Yield Rationale:
- <5% blast cells = NOT a hematologic relapse.
- MRD >0.01% = Molecular failure. ✅
- 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
D. Mature B-ALL
Rationale: Mature B-ALL expresses surface IgM (sIgM) and is linked to Burkitt’s lymphoma.
- 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
B. Common ALL
Rationale: Common ALL accounts for 49% of cases, making it the most prevalent subtype of B-ALL.
- 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+
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.
- 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
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.
- 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
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.
- 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. 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.
- 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
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.
- 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
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.
- What is the cure rate for frail patients >70-75 years old with ALL?
A. ≥70-80%
B. 50-60%
C. ~30%
D. ≤10%
D. ≤10%
Rationale: Frail elderly patients >70-75 years old have a ≤10% survival rate, highlighting the poor prognosis in this age group.
- 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
B. Cardiovascular dysfunction
Rationale: Cardiovascular dysfunction is the leading cause of noncancer death in childhood cancer survivors due to treatment-related cardiac toxicity.
- Which class of chemotherapeutic agents is most commonly associated with cardiac toxicity?
A. Alkylating agents
B. Anthracyclines
C. Platinum-based agents
D. Antimetabolites
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).