Benign RBC Disorders

Question 1

Normal component of BM

Answer 1

50% cellular

50% fat

Question 2

Normal constit of cellular marrow

Answer 2

65% granulocytes
25% RBCs
10% lymphs, monos, and precursors

Question 3

Neutrophilic leukocytosis

Answer 3

acute bacterial inf

sterile inflamm d/t tissue necrosis (MI, burns)

Question 4

Eosinophilic leukocytosis

Answer 4

allergic disorders
parasitic inf
drug reactions
collagen vascular disorders / some vasculitides
atheroembolic disease (xsient)
certain malig

Question 5

Basophilic leukocytosis

Answer 5

rare –> ind of myeloproliferative disease

Question 6

Monocytosis

Answer 6

chronic infections
bacterial endocarditis
rickettsiosis, and malaria
collagen vascular diseases (e.g. SLE)
IBD

Question 7

Neoplastic prolif of RBCs

Answer 7

leukemias
Polycythemia vera
thrombocytosis

Question 8

Sites of hematopoiesis

Answer 8

3rd wk –> yolk sac in mesoderm
3rd month of fetal life –> liver
4th month –> BM
by 18yo –> restricted to long bones and axial skeleton

Question 9

pronormoblast

Answer 9

1. first recognizable cell of RBC lineage

Question 10

basophilic normoblast

Answer 10

2. baso cyto d/t ribosomes and active protein synth

Question 11

polychromatophilic normoblast

Answer 11

3. cyto more pink d/t Hb

more condensed chromatin

Question 12

orthochromic

Answer 12

4. orange/red cyto
   pyknotic
   non-dividing nucleus

Question 13

reticulocyte

Answer 13

5. anucleate RBC

methylene blue stain to see ribos

Question 14

CFU-E

Answer 14

erythropoieting receptor on erythroid precursor cells

Question 15

Acute anemia of blood loss

Answer 15

loss of blood volume
hemodilution –> shift of water from interstitial fluid
reduction on O2 triggers erythropoietin prod
reactive thrombocytosis and leukocytosis
reticulocyte count inc 10-15% after 7d

Question 16

Chronic anemia of blood loss

Answer 16

blood loss exceeds regenerative capacity OR

iron preserves are depleted

Question 17

Morpho features of Hemolytic anemias

Answer 17

inc normoblasts in BM
reticulocytosis in periph blood
cholelithiasis
hemosiderosis

Question 18

Intravascular Hemolytic Anemia

Answer 18

mechanical injury, C' fixation, or exogenous toxic factors
hemoglobinemia
hemoglobinuria
jaundice and hemosiderinuria
low haptoglobin

Question 19

Extravascular hemolysis

Answer 19

injury to red cells, rendered “foreign”, or becoming less deformable

Question 20

Hereditary Spherocytosis

Answer 20

• -AD
• -intrinsic defect in red cell membrane
• -def in spectrin –> reduced membrane stability and spontaneous loss
• -inc osmotic fragility
• -spherocytes are less deformable

Question 21

G6PD deficiency

Answer 21

abnormal HMP shunt or glutathione metab
X-recessive
heinz bodies --> bite cells
hemoglobinuria
chronic non-spherocytic hemolytic anemia

Question 22

Sickle Cell Disease

Answer 22

–pt mutation = valine for glutamic acid @ 6th position of beta-globin chain
deoxygenation = HbS aggregate, polymerize, and sickle
inc Hb release –> inc bilirubin
capillary stasis and thrombosis

Question 23

Sickle Cell Disease pathology

Answer 23

BM : erythroid hyperplasia
can experience extra medullary hematopoiesis
adults have small spleen
cor pulmonale
leg ulcers
pigment gallstones
infarction secondary to vasc occlusion

Question 24

Sickle Cell aplastic crisis

Answer 24

Parvovirus B19

infects RBC progenitor and interrupts production

Question 25

Chronic Sickle Cell --> Bone

Answer 25

widening of medullary space thinning of cortex sparse trabecular pattern

Question 26

Chronic Sickle Cell --> GU

Answer 26

anoxic renal medulla

Question 27

Chronic Sickle Cell --> Spleen

Answer 27

multiple infarcts --> autosplenectomy

Question 28

Chronic Sickle cells --> liver

Answer 28

hepatitis intrahepatic cholestasis gallstones

Question 29

Chronic sickle cell --> heart

Answer 29

tachy | flow murmurs

Question 30

chronic sickle cell --> pulmonary

Answer 30

infarction pneumonia CHF

Question 31

chronic sickle cell --> retina

Answer 31

retinal vessel destruction

Question 32

chronic sickle cell --> CNS

Answer 32

children = 8% risk of stroke

Question 33

Sickle C Disease

Answer 33

equal amounts of HbS and HbC mild to mod hemolytic anemia / target cells painful crisis occurs splenomeg / hematuria / avascular necrosis retinal detachment and prolif retinopathy

Question 34

Sickle Beta Thalassemia

Answer 34

mod severe hemolytic anemia splenomeg primarily HbS on electro 15-30% HbA

Question 35

Thalassemia

Answer 35

mild anemia hypochromic, microcytic red blood cells, basophilic stippling expanded marrow cavity from massive erythroid hyperplasia

Question 36

Beta Thalassemia minor

Answer 36

Hb 10-13g/dL slightly elevated RBC count MCV 60-70 fL inc in HbA2

Question 37

Beta Thalassemia major

Answer 37

``` severe childhood anemia Hb very low (3 gm/dL) hypochromic, anisoctytosis, poikilocytosis, etc slightly inc HbA2 the rest is HbF ```

Question 38

Hydrops fetalis syndrome

Answer 38

deletion of all four alpha globulin genes | see Hb Barts on electro

Question 39

Alpha thalassemia trait

Answer 39

deletion of two alpha glob genes mild microcytic anemia normal to elevated RBC count

Question 40

Autoimmune hemolytic anemias

Answer 40

positive Coombs test warm type : spherocytes cold type: autoagglutination

Question 41

Warm type immunohemolytic anemia

Answer 41

most common IgG extravascular splenomegaly

Question 42

Cold type immunohemolytic anemia

Answer 42

``` IgM type mycoplasma inf mono flu HIV ```

Question 43

Drug induced immune hemolytic anemia

Answer 43

PCN --> Ab against membrane/drug complex Phenacetin --> deposition of complex Methyldopa --> autoimmune process