Hemolytic Anemia (Book)

*Question: What is the definition of a hemolytic disorder?
A) Excessive loss of RBCs from the body
B) Inadequate RBC production by bone marrow
C) Increased destruction of RBCs in the blood, bone marrow, or spleen
D) Increased plasma volume with unchanged RBC mass

*Answer: C) Increased destruction of RBCs in the blood, bone marrow, or spleen

*Question: What is the approximate normal RBC lifespan?
A) 60 days
B) 90 days
C) 120 days
D) 180 days

*Answer: C) 120 days

*Question: By how much can healthy bone marrow increase RBC production in response to hemolysis?
A) 2-3 times normal
B) 4-8 times normal
C) 10-12 times normal
D) 15-20 times normal

*Answer: B) 4-8 times normal

*Question: What defines a hemolytic anemia?
A) When RBC destruction is faster than the kidney can compensate
B) When RBC destruction exceeds the increased rate of RBC production
C) When bilirubin levels exceed 5 mg/dL
D) When reticulocyte count drops below normal

*Answer: B) When RBC destruction exceeds the increased rate of RBC production

*Question: Which classification distinguishes between acute and chronic hemolysis?
A) Site of hemolysis
B) Mechanism of hemolysis
C) Onset and duration of symptoms
D) Severity of anemia

*Answer: C) Onset and duration of symptoms

*Question: What is intravascular hemolysis?
A) Hemolysis occurring in bone marrow
B) Hemolysis due to rupture of RBC membrane while in circulation
C) Hemolysis occurring only in the spleen
D) Hemolysis that occurs after RBCs leave capillaries

*Answer: B) Hemolysis due to rupture of RBC membrane while in circulation

*Question: Where does extravascular hemolysis primarily occur?
A) In the bloodstream
B) In the kidneys
C) In macrophages of the spleen and liver
D) In the bone marrow

*Answer: C) In macrophages of the spleen and liver

*Question: What percentage of normal RBC destruction occurs via macrophage-mediated hemolysis?
A) 50%
B) 70%
C) 90%
D) 99%

*Answer: D) 99%

*Question: What is the first step in heme catabolism within macrophages?
A) Conjugation with glucuronic acid
B) Heme oxygenase breaks the protoporphyrin ring
C) Binding to albumin
D) Conversion to urobilinogen

*Answer: B) Heme oxygenase breaks the protoporphyrin ring

*Question: What is biliverdin?
A) The final breakdown product of hemoglobin
B) A linear molecule produced from protoporphyrin ring cleavage
C) A conjugated form of bilirubin
D) An iron-containing protein

*Answer: B) A linear molecule produced from protoporphyrin ring cleavage

*Question: Which enzyme converts biliverdin to bilirubin?
A) Heme oxygenase
B) Biliverdin reductase
C) Glucuronosyltransferase
D) Hemopexin

*Answer: B) Biliverdin reductase

*Question: How is unconjugated bilirubin transported in blood?
A) Free in plasma
B) Bound to hemopexin
C) Bound to albumin
D) Bound to transferrin

*Answer: C) Bound to albumin

*Question: Which transporter protein moves unconjugated bilirubin into hepatocytes?
A) MRP2
B) MRP3
C) OATP1B1
D) OATP1B3

*Answer: C) OATP1B1

*Question: What enzyme conjugates bilirubin in the liver?
A) Biliverdin reductase
B) UGT1A1 (glucuronosyltransferase)
C) Heme oxygenase
D) Alkaline phosphatase

*Answer: B) UGT1A1 (glucuronosyltransferase)

*Question: What makes conjugated bilirubin water-soluble?
A) Binding to albumin
B) Loss of iron
C) Addition of glucuronic acid molecules
D) Reduction by biliverdin reductase

*Answer: C) Addition of glucuronic acid molecules

*Question: Which transporter exports conjugated bilirubin from hepatocytes into bile?
A) OATP1B1
B) MRP2 (multidrug resistant protein 2)
C) MRP3
D) CD163

*Answer: B) MRP2 (multidrug resistant protein 2)

*Question: What converts conjugated bilirubin to urobilinogen in the intestines?
A) Hepatocytes
B) Pancreatic enzymes
C) Gut bacteria
D) Intestinal epithelial cells

*Answer: C) Gut bacteria

*Question: What is the final excretion product of most RBC-derived protoporphyrin?
A) Urobilinogen in urine
B) Bilirubin in bile
C) Urobilinogen in stool (stercobilin)
D) Hemosiderin

*Answer: C) Urobilinogen in stool (stercobilin)

*Question: What gives stool its brown color?
A) Conjugated bilirubin
B) Urobilinogen/stercobilin
C) Hemoglobin
D) Biliverdin

*Answer: B) Urobilinogen/stercobilin

*Question: How is iron from hemoglobin salvage transported in blood?
A) Bound to albumin
B) Bound to hemopexin
C) Bound to transferrin
D) Free in plasma

*Answer: C) Bound to transferrin

*Question: Where is absorbed iron from hemoglobin stored?
A) As hemosiderin in macrophages
B) As ferritin in liver and other tissues
C) As transferrin in blood
D) As hemopexin in plasma

*Answer: B) As ferritin in liver and other tissues

*Question: What is the predominant form of bilirubin in normal plasma?
A) Conjugated bilirubin secreted by the liver
B) Direct bilirubin from hepatocytes
C) Unconjugated bilirubin from the intestines
D) Indirect bilirubin from macrophages

*Answer: D) Indirect bilirubin from macrophages

*Question: What percentage of physiologic RBC destruction occurs via fragmentation?
A) 1%
B) 10-20%
C) 30-40%
D) 50%

*Answer: B) 10-20%

*Question: What protein rapidly binds free hemoglobin in plasma during fragmentation hemolysis?
A) Albumin
B) Hemopexin
C) Haptoglobin
D) Transferrin

*Answer: C) Haptoglobin

*Question: What is the hemoglobin-haptoglobin complex called?
A) Methemoglobin
B) Pseudotetramer
C) Hemosiderin
D) Oxyhemoglobin

*Answer: B) Pseudotetramer

*Question: Which receptor do macrophages use to bind and internalize the Hb-Hpt complex?
A) CD91
B) CD163
C) CD81
D) Transferrin receptor

*Answer: B) CD163

*Question: What happens to haptoglobin levels during excessive fragmentation hemolysis?
A) They increase significantly
B) They remain normal
C) They decrease markedly
D) They fluctuate unpredictably

*Answer: C) They decrease markedly

*Question: What binds free heme when haptoglobin is depleted?
A) Albumin only
B) Hemopexin
C) Transferrin
D) Ferritin

*Answer: B) Hemopexin

*Question: Through which receptor does the heme-hemopexin complex enter hepatocytes?
A) CD163
B) CD91
C) Transferrin receptor
D) LDL receptor

*Answer: B) CD91

*Question: What is methemoglobin?
A) Hemoglobin bound to haptoglobin
B) Oxidized free hemoglobin
C) Hemoglobin with iron removed
D) Conjugated hemoglobin

*Answer: B) Oxidized free hemoglobin

*Question: What complex binds metheme and protects cells from oxidative damage?
A) Haptoglobin complex
B) Transferrin complex
C) Methemoglobin-albumin complex
D) Hemopexin complex

*Answer: C) Methemoglobin-albumin complex

*Question: What is the role of HCP-1 (SLC46A1)?
A) Exports heme from cells
B) Imports heme into hepatocytes and enterocytes
C) Converts heme to bilirubin
D) Binds free hemoglobin

*Answer: B) Imports heme into hepatocytes and enterocytes

*Question: What cells are primarily responsible for reabsorbing iron-containing proteins in the nephron?
A) Glomerular cells
B) Distal tubular cells
C) Proximal tubular cells
D) Loop of Henle cells

*Answer: C) Proximal tubular cells

*Question: Which receptors on proximal tubular cells reabsorb filtered proteins?
A) CD163 receptors
B) Transferrin receptors
C) Megalin and cubilin
D) LDL receptors

*Answer: C) Megalin and cubilin

*Question: What can proximal tubular cells do with excess iron during fragmentation hemolysis?
A) Excrete it in stool
B) Store it as ferritin or convert to hemosiderin
C) Return it immediately to circulation
D) Convert it to bilirubin

*Answer: B) Store it as ferritin or convert to hemosiderin

*Question: What is hemoglobinuria?
A) Hemoglobin in stool
B) Brown urine with hemoglobin/heme filtered into urine
C) Increased hemoglobin in blood
D) Hemoglobin bound to albumin

*Answer: B) Brown urine with hemoglobin/heme filtered into urine

*Question: At what plasma hemoglobin concentration does hemoglobinuria typically occur?
A) 10 mg/dL
B) 25 mg/dL
C) 50 mg/dL
D) 100 mg/dL

*Answer: C) 50 mg/dL

*Question: What causes the coffee-brown or beer-colored appearance of urine in hemoglobinuria?
A) Bilirubin
B) Urobilinogen
C) Methemoglobin and metheme
D) Conjugated bilirubin

*Answer: C) Methemoglobin and metheme

*Question: What does hemosiderinuria indicate?
A) Acute hemolysis within the past hour
B) Chronic or recent significant intravascular hemolysis
C) Liver dysfunction
D) Kidney stones

*Answer: B) Chronic or recent significant intravascular hemolysis

*Question: How is hemosiderin detected in urine?
A) Direct visualization
B) Prussian blue stain
C) Gram stain
D) Wright-Giemsa stain

*Answer: B) Prussian blue stain

*Question: Which laboratory finding distinguishes fragmentation hemolysis from macrophage-mediated hemolysis?
A) Elevated indirect bilirubin
B) Presence of schistocytes on blood film
C) Elevated reticulocyte count
D) Anemia

*Answer: B) Presence of schistocytes on blood film

*Question: What is the typical finding for serum haptoglobin in fragmentation hemolysis?
A) Increased
B) Normal
C) Decreased
D) Variable, no consistent pattern

*Answer: C) Decreased

*Question: In macrophage-mediated hemolysis, what happens to urine hemoglobin?
A) It is positive
B) It is markedly elevated
C) It is negative
D) It shows hemosiderinuria

*Answer: C) It is negative

*Question: What is the expected indirect bilirubin level in macrophage-mediated hemolysis?
A) Decreased
B) Normal
C) Delayed elevation
D) Immediate elevation

*Answer: C) Delayed elevation

*Question: What causes jaundice in hemolytic anemia?
A) Decreased hemoglobin
B) Increased serum unconjugated bilirubin
C) Liver failure
D) Kidney dysfunction

*Answer: B) Increased serum unconjugated bilirubin

*Question: Where is jaundice most readily visible in early stages?
A) Palms of hands
B) Soles of feet
C) Sclera of the eyes
D) Nail beds

*Answer: C) Sclera of the eyes

*Question: What term describes jaundice caused by unconjugated bilirubin before liver processing?
A) Obstructive jaundice
B) Hepatic jaundice
C) Prehepatic jaundice
D) Post-hepatic jaundice

*Answer: C) Prehepatic jaundice

*Question: What is kernicterus?
A) Yellow coloring of skin
B) Bilirubin-induced brain damage in newborns
C) Liver damage from hemolysis
D) Kidney damage from hemoglobin

*Answer: B) Bilirubin-induced brain damage in newborns

*Question: Why do newborns with hemolytic anemia have increased risk of bone deformities?
A) Calcium deficiency
B) Vitamin D deficiency
C) Enlarged marrow space from compensatory erythropoiesis
D) Direct effect of bilirubin on bones

*Answer: C) Enlarged marrow space from compensatory erythropoiesis

*Question: What causes splenomegaly in chronic hemolysis?
A) Iron accumulation
B) Bilirubin deposition
C) Increased amount of bilirubin processing
D) Increased RBC removal workload

*Answer: D) Increased RBC removal workload

*Question: What causes gallstones in chronic hemolysis?
A) Increased cholesterol
B) Increased calcium
C) Increased bilirubin in bile (cholelithiasis)
D) Decreased bile salts

*Answer: C) Increased bilirubin in bile (cholelithiasis)

*Question: What may accompany acute hemolytic anemia and be confused with infection?
A) Rash
B) Hemolytic malaise, aches, vomiting, fever
C) Seizures
D) Respiratory distress

*Answer: B) Hemolytic malaise, aches, vomiting, fever

*Question: What can profound prostration and shock in acute hemolysis lead to?
A) Liver failure
B) Acute renal failure
C) Brain hemorrhage
D) Cardiac arrest

*Answer: B) Acute renal failure

*Question: What is the most commonly used laboratory test to detect accelerated erythropoiesis?
A) Hemoglobin level
B) White blood cell count
C) Reticulocyte count
D) Platelet count

*Answer: C) Reticulocyte count

*Question: What does an elevated reticulocyte count with anemia suggest?
A) Bone marrow failure
B) Iron deficiency
C) Hemolysis or hemorrhage
D) Aplastic anemia

*Answer: C) Hemolysis or hemorrhage

*Question: What blood film finding strongly suggests hemolysis?
A) Hypochromic cells
B) Microcytic cells
C) Polychromatic RBCs (reticulocytes)
D) Target cells

*Answer: C) Polychromatic RBCs (reticulocytes)

*Question: What does an elevated RDW indicate in the context of hemolysis?
A) Uniform cell size
B) Increased variation in RBC size
C) Decreased hemoglobin content
D) Normal bone marrow response

*Answer: B) Increased variation in RBC size

*Question: What cellular change is characteristic of macrophage-mediated hemolysis?
A) Schistocytes
B) Spherocytes
C) Target cells
D) Elliptocytes

*Answer: B) Spherocytes

*Question: Why does plasma not become visibly red/brown until hemoglobin exceeds 50 mg/dL?
A) Hemoglobin is immediately cleared below this level
B) The plasma salvage systems work efficiently below this level
C) Albumin masks the color
D) Bilirubin interferes with color

*Answer: B) The plasma salvage systems work efficiently below this level

*Question: What form of bilirubin is measured as "direct" bilirubin?
A) Unconjugated bilirubin
B) Conjugated bilirubin
C) Indirect bilirubin
D) Free bilirubin

*Answer: B) Conjugated bilirubin

*Question: What is the normal reference range for total serum bilirubin?
A) 0-0.5 mg/dL
B) 0.5-1.0 mg/dL
C) 2-5 mg/dL
D) 5-10 mg/dL

*Answer: B) 0.5-1.0 mg/dL

*Question: What is the expected urobilinogen finding in macrophage-mediated hemolysis?
A) Decreased
B) Absent
C) Normal
D) Increased

*Answer: D) Increased

*Question: What is the expected fecal urobilinogen finding in both types of hemolysis?
A) Decreased
B) Normal
C) Increased
D) Absent

*Answer: C) Increased

*Question: What enzyme may be elevated in hemolysis but is not specific?
A) ALT
B) AST
C) Lactate dehydrogenase (LDH)
D) Alkaline phosphatase

*Answer: C) Lactate dehydrogenase (LDH)

*Question: Why is LDH not typically used alone to diagnose hemolysis?
A) It's too expensive to measure
B) Other conditions like MI or liver disease also increase it
C) It's never elevated in hemolysis
D) It only rises in fragmentation hemolysis

*Answer: B) Other conditions like MI or liver disease also increase it

*Question: What test uses radioactive isotopes to measure RBC survival?
A) Complete blood count
B) Chromium-51 labeling
C) Reticulocyte count
D) Coombs test

*Answer: B) Chromium-51 labeling

*Question: What is the normal RBC half-life using chromium labeling?
A) 10-15 days
B) 25-32 days
C) 45-60 days
D) 90-120 days

*Answer: B) 25-32 days

*Question: What RBC half-life suggests mild hemolysis?
A) 5-10 days
B) 15-20 days
C) 25-30 days
D) 35-40 days

*Answer: B) 15-20 days

*Question: What RBC half-life indicates severe hemolysis?
A) Less than 5 days
B) 5-10 days
C) 10-15 days
D) 15-20 days

*Answer: A) Less than 5 days

*Question: Why are RBC survival studies using isotopes not commonly used clinically?
A) Too expensive
B) Require radioactive isotopes and time-consuming
C) Not accurate
D) Better alternatives available

*Answer: B) Require radioactive isotopes and time-consuming

*Question: What may result in falsely lowered glycated hemoglobin in diabetics with hemolysis?
A) Increased glucose levels
B) Decreased RBC life span
C) Increased insulin resistance
D) Liver dysfunction

*Answer: B) Decreased RBC life span

*Question: What laboratory test can quantify haptoglobin?
A) Immunochromatography only
B) Immunoturbidimetry or substrate depletion
C) Electrophoresis only
D) Spectrophotometry only

*Answer: B) Immunoturbidimetry or substrate depletion

*Question: What may cause falsely low haptoglobin besides hemolysis?
A) Iron deficiency
B) Infection
C) Liver disease impairing synthesis
D) Vitamin B12 deficiency

*Answer: C) Liver disease impairing synthesis

*Question: When may haptoglobin be falsely normal despite hemolysis?
A) In iron deficiency
B) During acute phase response/infection
C) In vitamin deficiency
D) In kidney disease

*Answer: B) During acute phase response/infection

*Question: What is the most reliable indicator to differentiate hemolytic anemia from other anemias with reticulocytosis?
A) Hemoglobin level
B) White blood cell count
C) Markedly decreased serum haptoglobin
D) Elevated bilirubin alone

*Answer: C) Markedly decreased serum haptoglobin

*Question: What does an increase in mean cell volume (MCV) with extreme reticulocytosis suggest?
A) Bone marrow failure
B) "Shift" reticulocytes (larger, premature cells)
C) Iron deficiency
D) Vitamin B12 deficiency

*Answer: B) "Shift" reticulocytes (larger, premature cells)

*Question: When should MCV be assessed for accuracy in hemolysis evaluation?
A) Before any reticulocyte response
B) During peak hemolysis
C) Before shift reticulocytes emerge
D) After recovery from anemia

*Answer: C) Before shift reticulocytes emerge

*Question: What is the expected RBC distribution width (RDW) in hemolysis?
A) Decreased
B) Normal
C) Increased
D) Variable with no pattern

*Answer: C) Increased

*Question: What morphologic finding on blood film is most specific for fragmentation hemolysis?
A) Spherocytes
B) Schistocytes
C) Target cells
D) Elliptocytes

*Answer: B) Schistocytes

*Question: What does bone marrow examination typically show in hemolytic anemia?
A) Hypocellular marrow
B) Erythroid hyperplasia
C) Decreased M:E ratio
D) Both B and C

*Answer: D) Both B and C

*Question: What is the myeloid-to-erythroid (M:E) ratio normally?
A) 1:1
B) 2:1
C) 4:1
D) 8:1

*Answer: C) 4:1

*Question: When is bone marrow examination most useful in hemolytic anemia evaluation?
A) In all cases of suspected hemolysis
B) To determine degree of compensation
C) When diagnosis remains unclear after other tests
D) As first-line test

*Answer: C) When diagnosis remains unclear after other tests

*Question: What distinguishes acute hemolytic anemia from hemorrhage?
A) Reticulocyte count
B) Hemoglobin level
C) Presence of indirect bilirubinemia in hemolysis
D) RBC morphology

*Answer: C) Presence of indirect bilirubinemia in hemolysis

*Question: What finding helps differentiate acute fragmentation hemolysis from hemorrhage?
A) Decreased hemoglobin
B) Delayed reticulocyte response in hemorrhage
C) Elevated white count
D) Normal bilirubin

*Answer: B) Delayed reticulocyte response in hemorrhage

*Question: In hemolysis, when does hemoglobinemia/hemoglobinuria occur?
A) Only in macrophage-mediated hemolysis
B) In both types equally
C) Only in intravascular hemolysis
D) Never

*Answer: C) Only in intravascular hemolysis

*Question: What helps differentiate hemolytic anemia from recovery after hemorrhage?
A) Hemoglobin level
B) Normal hemoglobinemia/haptoglobin in recovery from hemorrhage
C) Reticulocyte count
D) MCV

*Answer: B) Normal hemoglobinemia/haptoglobin in recovery from hemorrhage

*Question: What distinguishes chronic inherited hemolytic anemia from acquired?
A) Severity of anemia
B) Persistent vs transient nature
C) Bilirubin levels
D) Reticulocyte response

*Answer: B) Persistent vs transient nature

*Question: What classification distinguishes fragmentation from macrophage-mediated hemolysis?
A) Acute vs chronic
B) Inherited vs acquired
C) Intravascular vs extravascular
D) Intrinsic vs extrinsic

*Answer: C) Intravascular vs extravascular

*Question: Which hemolysis type typically shows spherocytes on blood film?
A) Fragmentation hemolysis
B) Macrophage-mediated hemolysis
C) Both types equally
D) Neither type

*Answer: B) Macrophage-mediated hemolysis

*Question: What is the hallmark finding of fragmentation hemolysis on blood film?
A) Spherocytes
B) Schistocytes
C) Target cells
D) Elliptocytes

*Answer: B) Schistocytes

*Question: Which type of hemolysis shows positive urine hemoglobin?
A) Macrophage-mediated only
B) Fragmentation only
C) Both types
D) Neither type

*Answer: B) Fragmentation only

*Question: What causes the Prussian blue stain of urine sediment to be positive?
A) Bilirubin
B) Hemosiderin
C) Urobilinogen
D) Schistocytes

*Answer: B) Hemosiderin

*Question: In which condition are hereditary conditions most common: intrinsic or extrinsic hemolysis?
A) Extrinsic
B) Both equally
C) Intrinsic
D) Neither

*Answer: C) Intrinsic

*Question: Which classification includes immune hemolysis and infectious agents?
A) Intrinsic defects
B) Fragmentation hemolysis
C) Extrinsic hemolysis
D) Hereditary hemolysis

*Answer: C) Extrinsic hemolysis

*Question: What is paroxysmal nocturnal hemoglobinuria classified as regarding intrinsic vs extrinsic?
A) Intrinsic defect
B) Extrinsic defect
C) Acquired disorder involving intrinsic defect
D) Neither category

*Answer: C) Acquired disorder involving intrinsic defec