(M) Hema Lec L3

https://docs.google.com/document/d/126gIwVjfOOGLBf9CTOrkPUBquiPAQyffBjPz-8W0TKc/edit?usp=sharing

C

What type of anemia is characterized by RBCs that are round rather than oval, typically seen in liver disease and alcohol-associated macrocytosis?

• a) Megaloblastic Macrocytic Anemia

• b) Microcytic Hypochromic Anemia

• c) Non-Megaloblastic Macrocytic Anemia

• d) Pure Red Cell Aplasia

• e) Aplastic Anemia

C

Which condition is a genetically predisposed disorder marked by the abnormal nuclear development of erythroid precursors (karyorrhexis) with unaffected leukocytes and platelets?

• a) Diamond-Blackfan Syndrome

• b) Dyskeratosis Congenita

• c) Chronic Dyserythropoietic Syndrome (CDA)

• d) Paroxysmal Nocturnal Hemoglobinuria

• e) Fanconi’s Anemia

A

What term describes the condition resulting from the depletion of hematopoietic precursor stem cells, leading to a decrease in all peripheral blood cell lines?

• a) Pancytopenia

• b) Leukoerythroblastosis

• c) Erythrocytosis

• d) Leukocytosis

• e) Thrombocytosis

B

Which congenital primary aplastic anemia features multiple chromosomal abnormalities and clinical manifestations like brown skin pigmentation (café-au-lait spots) and thumb malformations?

• a) Dyskeratosis Congenita

• b) Fanconi's Anemia

• c) Familial Aplastic Anemia

• d) Shwachman-Bodian-Diamond Syndrome

• e) Diamond-Blackfan Syndrome

E

Which DNA cross-linking agent is used in a diagnostic test to demonstrate increased chromosome breaks in patients with Fanconi's Anemia?

• a) Eosin-5-maleimide

• b) Ascorbate Cyanide

• c) Eculizumab

• d) Methylene Blue

• e) Diepoxybutane

D

What congenital unipotential stem cell defect results in the selective depletion of only the erythroid bone marrow tissue, primarily affecting young children?

• a) Fanconi's Anemia

• b) Shwachman-Bodian-Diamond Syndrome

• c) Dyskeratosis Congenita

• d) Diamond-Blackfan Syndrome

• e) Evan's Syndrome

C

Which viral infection is a known cause of Acquired Pure Red Cell Aplasia (PRCA) by infecting erythroid progenitor cells and causing cellular lysis?

• a) Epstein-Barr Virus (EBV)

• b) Cytomegalovirus (CMV)

• c) Parvovirus B19

• d) Hepatitis B Virus

• e) Human Immunodeficiency Virus (HIV)

A

What type of anemia occurs when the bone marrow is infiltrated and replaced by abnormal nonerythroid cells, such as tumors, cancer, or fibrous tissue?

• a) Myelophthisic Anemia

• b) Spur Cell Anemia

• c) Aplastic Anemia

• d) Macrocytic Anemia

• e) Hemolytic Anemia

D

What characteristic red blood cell morphology is strongly associated with Myelophthisic Anemia?

• a) Acanthocytes

• b) Schistocytes

• c) Spherocytes

• d) Teardrop cells

• e) Target cells

C

In anemia caused by renal disease, what hormone’s production is notably decreased?

• a) Testosterone

• b) Thyroid-stimulating hormone (TSH)

• c) Erythropoietin (EPO)

• d) Cortisol

• e) Aldosterone

B

What term is used when red blood cells are removed and destroyed by macrophages primarily in the spleen and liver?

• a) Intravascular hemolysis

• b) Extravascular hemolysis

• c) Karyorrhexis

• d) Apoptosis

• e) Dyserythropoiesis

C

During hemolysis, what specific breakdown product of protoporphyrin is increased in the blood because its production exceeds the liver's capacity to conjugate it?

• a) Urobilinogen

• b) Conjugated bilirubin

• c) Unconjugated (indirect) bilirubin

• d) Biliverdin

• e) Haptoglobin

A

What intrinsic hemolytic anemia is caused by a vertical interaction defect resulting from a spectrin deficiency?

• a) Hereditary Spherocytosis

• b) Hereditary Elliptocytosis

• c) Hereditary Pyropoikilocytosis

• d) Southeast Asian Ovalocytosis

• e) Hereditary Stomatocytosis

D

What is currently considered the gold standard diagnostic test for Hereditary Spherocytosis?

• a) Direct Antiglobulin Test (DAT)

• b) Osmotic Fragility Test

• c) Ascorbate Cyanide Test

• d) EMA (Eosin-5-maleimide) Binding Test

• e) HAM's Acidified Serum Test

B

What test is used to differentiate Hereditary Spherocytosis from Autoimmune Hemolytic Anemia (AIHA)?

• a) Flow Cytometry

• b) Direct Antiglobulin Test (DAT)

• c) Indirect Antiglobulin Test (IAT)

• d) Sucrose Hemolysis Test

• e) Ascorbate Cyanide Test

A

What rare hereditary membrane defect is characterized by RBCs that fragment at a significantly lower temperature (45-46°C) than normal cells?

• a) Hereditary Pyropoikilocytosis

• b) Hereditary Elliptocytosis

• c) Hereditary Spherocytosis

• d) Southeast Asian Ovalocytosis

• e) Hereditary Xerocytosis

C

What acquired clonal stem cell disorder causes cells to lyse when blood pH lowers slightly, typically at night?

• a) Pure Red Cell Aplasia

• b) Evan's Syndrome

• c) Paroxysmal Nocturnal Hemoglobinuria (PNH)

• d) Autoimmune Hemolytic Anemia

• e) Paroxysmal Cold Hemoglobinuria (PCH)

B

What specific membrane structures are missing in the RBCs of a patient with Paroxysmal Nocturnal Hemoglobinuria, detectable via the FLAER test?

• a) Band 3 proteins

• b) GPI anchors

• c) Spectrin dimers

• d) Telomeres

• e) Hemoglobin F

C

What specific morphologic feature (cell type) is the hallmark of Spur Cell Anemia due to excess free cholesterol in severe liver disease?

• a) Target cells

• b) Spherocytes

• c) Acanthocytes

• d) Schistocytes

• e) Elliptocytes

A

What is the most common RBC enzymopathy that causes episodic hemolysis induced by oxidative stress and is linked to favism?

• a) G6PD Deficiency

• b) Methemoglobin Reductase Deficiency

• c) Pyruvate Kinase Deficiency

• d) ADAMTS13 Deficiency

• e) Spectrin Deficiency

B

In G6PD deficiency, what inclusions form inside the RBCs due to the oxidation of hemoglobin and are visible only with a supravital stain?

• a) Howell-Jolly bodies

• b) Heinz bodies

• c) Pappenheimer bodies

• d) Basophilic stippling

• e) Cabot rings

A

Which test rapidly oxidizes hemoglobin to create a discernible brown methemoglobin color to screen for G6PD deficiency?

• a) Ascorbate Cyanide Test

• b) Fluorescent Spot Test

• c) Osmotic Fragility Test

• d) HAM's Test

• e) Sugar Water Screening Test

D

Because RBCs lack mitochondria, a deficiency in which enzyme severely impairs ATP production via the glycolytic pathway?

• a) ADAMTS13

• b) Methemoglobin Reductase

• c) Glucose-6-Phosphate Dehydrogenase

• d) Pyruvate Kinase (PK)

• e) Telomerase

C

What class of antibodies primarily drives Warm Autoimmune Hemolytic Anemia, active at 37°C?

• a) IgA

• b) IgE

• c) IgG

• d) IgM

• e) IgD

B

What condition represents an Autoimmune Hemolytic Anemia (AIHA) that occurs concurrently with Immune Thrombocytopenic Purpura (ITP)?

• a) HELLP Syndrome

• b) Evan's syndrome

• c) Budd-Chiari syndrome

• d) Diamond-Blackfan Syndrome

• e) Shwachman-Bodian-Diamond Syndrome

D

Cold Agglutinin Disease is primarily driven by large, pentameric antibodies of which immunoglobulin class?

• a) IgA

• b) IgE

• c) IgG

• d) IgM

• e) IgD

A

Which specific antibody mediates Paroxysmal Cold Hemoglobinuria (PCH) by targeting P blood group antigens?

• a) Donath-Landsteiner antibody

• b) Anti-CD55 antibody

• c) Anti-Band 3 antibody

• d) Anti-ADAMTS13 antibody

• e) Anti-GPI antibody

D

What term describes nonimmune hemolysis caused by mechanical shearing as RBCs pass through microthrombi in small vessels?

• a) Macroangiopathic Hemolytic Anemia

• b) Alloimmune Hemolytic Anemia

• c) Autoimmune Hemolytic Anemia

• d) Microangiopathic Hemolytic Anemia (MAHA)

• e) Traumatic Cardiac Hemolytic Anemia

A

What fragmented red blood cell morphology is the hallmark finding in Microangiopathic Hemolytic Anemia (MAHA)?

• a) Schistocytes

• b) Acanthocytes

• c) Spherocytes

• d) Teardrop cells

• e) Bite cells

C

Thrombotic Thrombocytopenic Purpura (TTP) is caused by a deficiency in which specific enzyme, leading to VWF multimer accumulation?

• a) G6PD

• b) Pyruvate Kinase

• c) ADAMTS13

• d) Methemoglobin Reductase

• e) CD59

B

Typical Hemolytic Uremic Syndrome (HUS), which causes microthrombi in glomeruli, is most commonly associated with which bacterial toxin?

• a) Cholera toxin

• b) Shiga toxin

• c) Tetanospasmin

• d) Botulinum toxin

• e) Diphtheria toxin

D

What condition, occurring in pregnancy (preeclampsia/eclampsia), stands for Hemolysis, Elevated Liver enzymes, and Low Platelets?

• a) Evan's Syndrome

• b) TTP

• c) DIC

• d) HELLP Syndrome

• e) HUS

A

What disorder is characterized by widespread clotting activation that consumes platelets and coagulation factors, leading to an increased D-dimer?

• a) Disseminated Intravascular Coagulation (DIC)

• b) Hemolytic Uremic Syndrome (HUS)

• c) Thrombotic Thrombocytopenic Purpura (TTP)

• d) HELLP Syndrome

• e) Immune Thrombocytopenic Purpura (ITP)

B

Exercise-Induced Hemoglobinuria, commonly seen in runners due to repetitive mechanical impact on the feet, is also known by what term?

• a) Traumatic Cardiac Anemia

• b) March hemoglobinuria

• c) Paroxysmal Nocturnal Hemoglobinuria

• d) Paroxysmal Cold Hemoglobinuria

• e) Spur Cell Anemia

D

What unique red blood cell morphology is distinctly observed in the peripheral smears of patients with extensive thermal trauma (burns)?

• a) Macrocytes and target cells

• b) Acanthocytes and echinocytes

• c) Teardrop cells and elliptocytes

• d) Microspherocytes and budding fragmentation

• e) Bite cells and blister cells

B

Which hereditary membrane defect is described as a horizontal interaction defect (spectrin dimer-dimer) and shows large numbers of elongated cells on the smear?

• a) Hereditary Spherocytosis

• b) Hereditary Elliptocytosis

• c) Hereditary Stomatocytosis

• d) Hereditary Xerocytosis

• e) Southeast Asian Ovalocytosis

A

A mutation in Band 3 that provides malaria resistance and causes cells to feature one or two transverse ridges is known as what?

• a) Southeast Asian Ovalocytosis

• b) Hereditary Pyropoikilocytosis

• c) Hereditary Spherocytosis

• d) Spur Cell Anemia

• e) Xerocytosis

C

What type of hemolysis occurs due to a hemolytic transfusion reaction where antibodies from one individual react with the RBCs of another?

• a) Autoimmune hemolysis

• b) Drug-induced hemolysis

• c) Alloimmune hemolysis

• d) Microangiopathic hemolysis

• e) Macroangiopathic hemolysis

D

What treatment is indicated to reverse the formation of Ferric iron (Fe3+) in patients with Methemoglobin Reductase Deficiency?

• a) Folic acid

• b) Plasma exchange

• c) Eculizumab

• d) Methylene blue

• e) Corticosteroids

E

What bone marrow profile (biopsy finding) is characteristic of classic Aplastic Anemia?

• a) Hypercellular with erythroid hyperplasia

• b) Megaloblastic with hypersegmented neutrophils

• c) Cellular with normal M:E ratio

• d) Fibrotic with teardrop cells

• e) Hypocellular with >70% yellow marrow (fat infiltration)

Mean Corpuscular Volume (MCV)

What is the specific measurement used to morphologically classify anemias as macrocytic, normocytic, or microcytic?

CFU-E (Colony-Forming Unit-Erythroid)

In Pure Red Cell Aplasia (PRCA), what specific unipotential stem cell has a defect causing selective erythroid depletion?

Familial Aplastic Anemia

What congenital condition causes a subset of Fanconi's anemia but presents without the typical congenital physical defects?

Dyskeratosis Congenita

What acquired condition causes Aplastic Anemia through defective telomere maintenance?

Benzene

What chemical agent containing a specific ring structure is a known acquired cause of Secondary Aplastic Anemia?

Hb F (Fetal Hemoglobin)

What is the major hemoglobin finding that is typically increased (5%-25%) in patients with Diamond-Blackfan Syndrome?

Liver Disease

What condition is caused by abnormal lipid metabolism altering the lipid composition of the RBC membrane, often resulting in target cells or acanthocytes?

Hypothyroidism

What hormone deficiency causes decreased cellular metabolic rate and tissue oxygen requirement, thereby decreasing EPO production?

Intravascular hemolysis

What specific term describes RBCs that rupture entirely within the blood vessels, releasing hemoglobin directly into the plasma?

Na-K pump

In Hereditary Spherocytosis, the membrane becomes leaky to Na+ and K+, which forces what specific pump to use more ATP?

Aplastic crisis

What known clinical complication of Hereditary Spherocytosis can be induced suddenly by viral infections?

Osmotic Fragility Test

What test is utilized because spherocytes have low membrane reserve and will burst early in hypotonic solutions?

Protein 4.1 deficiency

What is the structural protein deficiency primarily responsible for spherocytic Hereditary Elliptocytosis (a subtype making up 10% of HE cases)?

Hereditary Xerocytosis

What hereditary membrane defect is characterized by an altered membrane permeability to cations resulting in dehydrated target cells with decreased osmotic fragility?

FLAER test

What is the gold standard screening test via flow cytometry used to detect the absence of GPI anchors in PNH?

Budd-Chiari syndrome

In Paroxysmal Nocturnal Hemoglobinuria, what classic thrombotic complication occurs specifically in the hepatic veins?

NADPH

What compound normally tied to glutathione reduction cannot be sufficiently produced due to a G6PD deficiency?

Bite cells (or blister cells)

What specific RBC morphological markers are created when splenic macrophages remove Heinz bodies in G6PD deficiency?

NADP reduction rate

What quantitative assay is measured spectrometrically at 340 nm to diagnose G6PD deficiency?

HAM's Acidified Serum Test

What diagnostic test uses 0.2N HCl to screen for Paroxysmal Nocturnal Hemoglobinuria?

Sugar Water Screening Test

What screening test is also known as the Sucrose Hemolysis Test, used for PNH?

Cold Agglutinin Disease

What type of Autoimmune Hemolytic Anemia causes acrocyanosis due to antibody activity at temperatures between 0-4°C?

Complement (C3b/C3d)

What specific protein is detected on the surface of RBCs in vivo when a Direct Antiglobulin Test (DAT) is positive for Cold Agglutinin Disease?

Extrinsic Hemolytic Anemias

What broad category of Hemolytic Anemias indicates that if normal RBCs were transfused into the patient, they would also be destroyed?

ADAMTS13

What enzyme is deficient in typical Thrombotic Thrombocytopenic Purpura (TTP)?

Eculizumab

What specific monoclonal antibody is the treatment of choice for Atypical Hemolytic Uremic Syndrome (aHUS) caused by complement dysregulation?

PT/aPTT (Prothrombin Time/Activated Partial Thromboplastin Time)

What specific test evaluates the coagulation pathway and is found to be prolonged in Disseminated Intravascular Coagulation (DIC) alongside decreased fibrinogen?

Macroangiopathic Hemolytic Anemia

What term describes nonimmune hemolysis caused by the mechanical destruction of RBCs in large blood vessels due to turbulent flow?

Schistocytes

What specific red blood cell morphology is generally absent in Exercise-Induced (March) Hemoglobinuria, differentiating it from other mechanical traumas?

Hemolytic Uremic Syndrome (HUS)

Burr cells and RBC fragments are characteristically seen in what renal-associated microangiopathic disorder?

LDH (Lactate Dehydrogenase)

What laboratory parameter (enzyme) is typically elevated in intravascular hemolysis due to RBC breakdown, alongside indirect bilirubin?

Haptoglobin

What specific protein is characteristically decreased in the plasma during intravascular hemolysis because it binds to free hemoglobin?

Prosthetic heart valves (or native paravalvular leaks)

What specific chronic complication results in hemosiderinuria and decreased ferritin in patients with Traumatic Cardiac Hemolytic Anemia?

X-linked recessive

What is the inheritance pattern of G6PD Deficiency, explaining why males are affected and females are carriers?

normochromic, normocytic; pancytopenia

What type of red blood cells are seen in the peripheral blood smear in Shwachman-Bodian-Diamond Syndrome as part of the overall bone marrow failure?

Myelophthisic anemia

Teardrop cells

G6PD Deficiency

Bite/Blister cells & Heinz bodies

Schistocytes/Helmet cells

MAHA, Traumatic Cardiac Hemolysis, Mechanical trauma

Severe liver disease

Acanthocytes (Spur cells)

Target cells

Xerocytosis, Alcohol-associated macrocytosis