Path: RBC, Bleeding Disorders

Question 1

Low ferritin/High TIBC/Low Hepcidin; Low serum Fe/Low % saturation; High RDW/High FEP; Low Hct/Low Hb

Answer 1

Iron-Deficiency Anemia (IDA)

Question 2

Associated Illness with IDA

Answer 2

Plummer-Vinson Syndrom (Anemia, Esophageal Webs, Dysphagia)

Question 3

Increased IL-6 APP –> Increased Hepcidin –> decreased Ferroportin and EPO

Answer 3

Anemia of Chronic Disease

Question 4

High ferritin/low TIBC; low serum Fe/Low % saturation; High FEP; low EPO

Answer 4

Anemia of Chronic Dz

Question 5

Deletion in Chr. 16

Answer 5

Alpha Thalassemia

Question 6

Mutation in Chr. 11

Answer 6

Beta Thalassemia

Question 7

Hb Barts

Answer 7

Gamma tetramers, 4 gene deletion in Alpha Thalassemia

Question 8

HbH

Answer 8

Beta tetramers, 3 gene deletion in Alpha Thalassemia

Question 9

HbF > HbA2

Answer 9

B-Thal Major

Question 10

HbA2 > HbF

Answer 10

B-Thal Minor

Question 11

Low RBC, hypersegmented Neutrophils, MCV >100

Answer 11

Megaloblastic Anemia

Question 12

Incr. Homocysteine, Normal Methymalonic Acid, MCV >100

Answer 12

Folate Deficiency

Question 13

Incr. Homocysteine, Incr. Methylmalonic Acid, MCV >100

Answer 13

B12 Deficiency

Question 14

AI Atrophic Gastritis –> AI T-cells

Answer 14

Pernicious Anemia –> B12 deficiency

Question 15

Ankyrin, Spectrin, Band3, Howell-Jolly Bodies

Answer 15

Hereditary Spherocytosis (Extravascular)

Question 16

HbS

Answer 16

Valine substitution –> Sickle-Cell Anemia

Question 17

HbSC

Answer 17

Valine & Lysine substitutions –> mild sickling

Question 18

PIGA –> GPI proteins (MIRL, DAF, C8 binding protein)

Answer 18

Paroxysmal Nocturnal Hemoglobinuria (PNH) = intravascular hemolysis

Question 19

Heinz Bodies, Bite Cells; X-linked recessive; intermittent hemolysis

Answer 19

G6PD Deficiency

Question 20

Warm Agglutinins

Answer 20

IgG

Question 21

Cold Agglutinins

Answer 21

IgM

Question 22

D-dimers, Schistocytes

Answer 22

Microangiopathic Hemolytic Anemia

Question 23

Fanconi Anemia

Answer 23

Aplastic Anemia

Question 24

Parvovirus B19

Answer 24

Pure Red Cell Aplasia (decreases RBCs)

Question 25

Space occupying lesion --> suppressed BM --> Tear-drop RBC and Leukoerythroblastosis (immature granulocytes and RBC)

Answer 25

Myelophthistic Anemia

Question 26

Uremia + Extracorpuscular defects + Decreased EPO synthesis --> underproduce RBCs

Answer 26

Chronic Renal Failure

Question 27

Gaisbock Syndrome

Answer 27

Stress Polycythemia = reduced plasma volume with HTN, obese, anxious patient

Question 28

Stabilized HIF1-alpha --> increased EPO

Answer 28

Secondary Polycythemia

Question 29

PTT, Heparin pathway (less important)

Answer 29

Intrinsic

Question 30

PT, Tissue damage, Coumadin pathway (most important)

Answer 30

Extrinsic

Question 31

Purpuric rash, colic, polyarthralgia, acute glomerulonephritis

Answer 31

Henoch-Schonlein Purpura

Question 32

TGF-B; tortuous veins, epistaxis and other very serious bleeding complications

Answer 32

Weber-Osler-Rendu Syndrome

Question 33

Peripheral Megathrombocytes, Increased BM megakaryocytes, large peripheral platelets, no splenomegaly or LAD; petechial hemorrhages

Answer 33

Chronic Immune Thrombocytopenic Purpura (hemorrhage)

Question 34

Follicular B-cell hyperplasia --> Auto-Abs to GPIIb/IIIa

Answer 34

HIV-associated Thrombocytopenia

Question 35

ADAMTS13 --> excessive platelet activation

Answer 35

Thrombotic Thrombocytopenic Purpura (thromboses)

Question 36

E.Coli activated thrombotic microangiopathy

Answer 36

Hemolytic Uremic Syndrome (HUS)

Question 37

GPIb-Ix (Aut. Rec.) --> Platelet Adhesion defect

Answer 37

Bernard-Soulier Syndrome

Question 38

GPIIb/IIIA (Aut. Rec.) --> Platelet Aggregation defect

Answer 38

Glanzmann Thrombasthenia

Question 39

Prolonged PTT

Answer 39

vWF, Hemophilia A/B

Question 40

ADAMTS13 + normal platelets, prolonged PTT, hemarthoris

Answer 40

vWF (Type 3) = Aut. Recessive

Question 41

Factor VIII deficiency; hemarthorses; prolonged PTT

Answer 41

Hemophilia A (X-linked Recessive)

Question 42

Factor IX deficiency; hemarthroses; prolonged PTT

Answer 42

Hemophilia B (X-linked recessive)

Question 43

Non-immunologic Thrombocytopenia, MAJOR bleeding, triggered by Tissue Factor; hemorrhage and thromboses

Answer 43

DIC

Question 44

IgG reaction to IgA in donor blood

Answer 44

IgA deficiency

Question 45

Acute Transfusion hemolysis

Answer 45

IgG

Question 46

Delayed Transfusion hemolysis

Answer 46

IgM

Question 47

Acute Respiratory Depression, Hypotension, hypoxemia, increased activated neutrophils AFTER transfusion

Answer 47

TRALI