Hem/onc

Question 1

Fetal erythropoeisis

Answer 1

yolk sac (3-8 weeks)
Liver (6 weeks - birth)
Spleen (10-28 weeks)
Bone marrow (18 weeks to adult)

Question 2

Rh hemolytic disease of the newborn

Answer 2

Rh- mother, Rh+ fetus
first pregnancy: mother exposed to fetal blood –> mom form anti D IgG
Subsequent pregnancies: anti D IgG crosses the placenta –> attacks fetal RBC –> hemolysis in fetus
presents as hydrops fetalis, jaundice shortly after birth, kernicterus

Question 3

ABO hemolytic disease of the newborn

Answer 3

Type O mom; Type A or B fetus
preexisting maternal anti A or anti B IgG Ab cross placenta –> hemolysis in fetus
Presents as mild jaundice in the neonate within 24 hours of birth. CAN OCCUR IN FIRST BORN and less severe
Tx: phototherapy or exchange transfusion

Question 4

Neutrophils

Answer 4

Acute inflammatory response cells
Numbers increase in bacterial infection
Phagocytic
multilobed nucleus
specific granules contain leukocyte, alkaline phosphatase, collagenase, lysozyme and lactogerrin
Azurophilic granules contain proteases, acid phosphatase, myeloperoxidase and B glucuronidase

Question 5

Hypersegmented neutrophils

Answer 5

vit B12/ folate deficiency

Question 6

Left shift with increased band cells

Answer 6

increase myeloid proliferation (bacterial infection, CML)

Question 7

Neutrophil chemotaxis

Answer 7

C5a, IL8, LTB4, kalikrein, PAF

Question 8

Erythrocytes

Answer 8

Carry O2 to tissues and CO2 to lungs
Anucleate and lack organelles, biconcave with large surface area to volume taio for rapid gas exchange.
Life span of 120 days
USE GLUCOSE

Question 9

Thrombocytes

Answer 9

primary hemostasis
small cytoplasmic fragments derived from megakaryocytes
Contains Ca2+, ADP, 5HT, Histamine and a granules

Question 10

Monocytes

Answer 10

found in blood, differentiate into macrophages in tissues

Large kidney shaped nucleus

Question 11

Macrophages

Answer 11

phagocytose bacteria, cellular debris and senescent RBCs

Activated by y interferon

Question 12

Eosinophils

Answer 12

heminth infection

Produce histamine, major basic protein, eosinophil peroxidase, cationic protein, neurotoxin

Question 13

Basophils

Answer 13

mediate allergic reaction

heparin

Question 14

Mast cells

Answer 14

mediate local tissue allergic reactions
Can bind IgE to membrane
histamine, heparin, tryptase and eosinophil chemotactic factors
Type 1 hypersensitivity

Question 15

Dendritic cells

Answer 15

APC

MHC II and Fc receptors

Question 16

Lymphocytes

Answer 16

B, T and NK cells

Question 17

Natural killer cells

Answer 17

innate immunity (Intracellular pathogens) 
perforin and granzymes

Question 18

B cells

Answer 18

in follicles of LN, white pulp of spleen, unencapsulated lymphoid tissued
HUMORAL IMMUNITY

Question 19

T cells

Answer 19

mediate CELLULAR IMMUNE RESPONSE

mature in thymus

Question 20

Plasma cells

Answer 20

produce Ab specific to particular antigen

Bone marrow and do not circulate

Question 21

Thrombogenesis

Answer 21

Formation of insoluble fibrin mesh
Aspirin irreversible inhibit COX –> inhibit TXA2 synthesis
Clopidogrel inhibit ADP induced expression of gpIIb/IIIa by irreversibly blocking P2Y12 receptor
Abciximab inhibit gpIIb/IIIa directly
Ristocetin activates vWF to bind gpIb. Failure of aggregation with ristocetin assay occurs in vWd and Bernard Soulier syndrome

Question 22

Vitamin K deficiency

Answer 22

decreased synthesis of Factors 2, 7, 9, 10, protein C and S

Warfarin inhibits vitamin K epoxide reductase. FFP and PCC reverse warfarin actions

Question 23

Anticoagulation

Answer 23

Antithrombin inhibits thrombin and factors 7a, 9a, 10a, 11a, 12a
Heparin enhances the activity of antithrombin

Question 24

Acanthosis

Answer 24

Liver Disease, abetalipoproteinemia

Projections of varying sizes at irregular intervals

Question 25

Echinocytes

Answer 25

Liver disease, ESRD, pyruvate kinase deficiency | Smaller and more uniform projections

Question 26

Dacrocytes

Answer 26

teardrop cells Bone marrow infiltration Mechanically squeezed out of bone marrow

Question 27

Schistocytes

Answer 27

MAHA, mechanical hemolysis | fragmented RBC

Question 28

Degmacytes

Answer 28

G6PD deficiency | removal of heinz bodes by splenic macrophages

Question 29

Elliptocytes

Answer 29

Hereditary elliptocytosis | mutation in genes encoding RBC membrane proteins

Question 30

Spherocytes

Answer 30

Hereditary spherocytosis, autoimmune hemolytic anemia | small spherical cells without central pallor

Question 31

Macro ovalocytes

Answer 31

Megaloblastic anemia

Question 32

Target cells

Answer 32

HbC disease, asplenia, liver disease, thalassemia

Question 33

Sickle cell

Answer 33

Sickle cell anemia | sickling occurs with low O2 conditions

Question 34

Iron granules

Answer 34

Sideroblastic anemias perinuclear mitochondria with excess iron REQUIRE PRUSSIAN BLUE STAIN

Question 35

Howell Jolly Bodies

Answer 35

functional hyposplenia, asplenia Basophilic nuclear remnants removed by splenic macrophages usually

Question 36

Basophilic stippling

Answer 36

sideroblastic anemia, thalessemia

Question 37

Pappenheimer bodies

Answer 37

sideroblastic anemia | basophilic granules

Question 38

Heinz bodies

Answer 38

G6PD deficiency | denatured and precipitated Hb

Question 39

Microcytic, hypochromic anemias

Answer 39

``` Iron deficiency a thalassemia B thalassemia Lead poisoning Sideroblastic anemia ```

Question 40

Iron Deficiency Anemia

Answer 40

via chronic bleeding, malnutrition, absorption disorders, , GI surgery or increase demand --> low final step in heme synthesis Low Fe, high TBC, low ferritin, high free erythrocytic protoporphyrin, high RDW, low RI Sx: fatigue, pallor, PICA, spoon nails glossitis, cheilosis, Plummer Vinson

Question 41

a thalassemia

Answer 41

a globin gene deletion on Chromosome 16 --> low a globin synthesis More deletion --> more microcytic hypochromic anemia 3 deletions --> B4 (HbH) 4 deletions --> y4- hydrops fetalis

Question 42

B thalassemia

Answer 42

point mutation in splice sites and promoter sequences on chr 11 --> decrase B globin synthesis

Question 43

B thalassemia minor

Answer 43

B chain is underproduced Asymptomatic Dx via high HbA2 on electrophoresis

Question 44

B thalassemia major

Answer 44

B chain absent --> severe microcytic hypochromic anemia with target cells Marrow expansion --> skeletal deformities. Extramedullary hematopoiesis --> hepatosplenomegaly Increase risk of parvovirus B19 aplastic crisis

Question 45

HbS/ B thalassemia heterozygote

Answer 45

mild to moderate sickle cell disease

Question 46

Lead poisoning

Answer 46

Inhibits ferrochelatase and ALA dehydratase --> low heme synthesis and high RBC protophyrin inhibits rRNA degradation --> RBCs retain aggregates of rRNA Lead lines gingivae and metaphyses of long bones on Xray, encephalopathy and erythrocyte basophilic stippling, ab colic and sideroblastic anemia, wrist and foot drop Tx Dimercaprol and EDTA

Question 47

Sideroblastic anemia

Answer 47

X linked defect in ALA synthase gene, myeloblastic syndrome and alcohol, lead poisoning, drugs High iron, normal TIBC, high ferritin ringed sideroblasts in bone marrow. Tx pyridoxine

Question 48

Megaloblastic anemias

Answer 48

impaired DNA synthesis --> maturation of nucleus of precursor cells in bone marrow delayed relative to maturation of cytoplasm. Causes vit B12 deficiency, folate deficiency, meds

Question 49

Folate Deficiency

Answer 49

malnutrition, malabsorption, drugs, high requirement Increased homocysteine, normal methylmalonic acid NO NEURO

Question 50

Vit B 12 deficiency

Answer 50

pernicious anemia, malabsorption, pancreatic insufficiency, gastrectomy, insufficient intake High homocysteine, methylmalonic acid NEURO: reversible dementia Folate correct anemia but worsens neuro

Question 51

Orotic aciduria

Answer 51

inability to convert orotic acid to UMP because defect in UMP synthase. AR failure to thrive, developmental delay and megaloblastic anemia.

Question 52

Nonmegaloblastic anemia

Answer 52

macrocytic anemia in which DNA synthesis is normal Alcoholism and liver disease NO HYPERSEGMENTED NEUTROPHILS

Question 53

Diamond Blackfan

Answer 53

Congenital pure red cell aplasia rapid onset anemia within first year of life due to intrinsic defect in erythroid progenitor cells short, craniofacial abnormality, UE malformation

Question 54

Intravascular hemolysis

Answer 54

low haptoglobin high schistocytes mechanical hemolysis, paraxysmal nocturnal hemoglobinuria, MAHA

Question 55

Extravascular hemolysis

Answer 55

macrophages in spleen clear RBCs spherocytes no hemoglobinuria, hemosideriuria can present with urobilinogen in urine