Anemia II

Question 1

anemia of chronic disease/inflammation

Answer 1

normochronic, normocytic, hypoproliferative

can also by hypo chromic and microcytic

Question 2

pathogenesis of anemia of chronic disease

Answer 2

autoimmune dysreg –> downstream effects

• increased hepcidin –> less iron absorption
• iron gets trapped in macrophages
• direct inhibition of erythropoiesis

–> functional iron deficiency

Question 3

labs in anemia of chronic disease

Answer 3

ferritin normal-high
transferrin low to normal
T sat low-normal
iron low
marrow iron stores high
sed rate high

Question 4

tx of ACD

Answer 4

transfusion if severe anemia

erythropoietin if nec

Question 5

aplastic anemia

Answer 5

diminished or absent hematopoietic precursors due to injury to HSC –> pancytopenia and empty bone marrow

Question 6

causes of aplastic anemia

Answer 6

primary: idiopathic, fanconi anemia (genetic)
secondary: radiation, chemo, other drugs, viruses (EBV)

Question 7

anemia of chronic kidney disease

Answer 7

decreased erythropoietin production due to decreased renal cortical cells,
+ freq phlebotomy, shortened RBC life span –> normocytic anemia

Question 8

other causes of hypoproliferative normocytic anemias

Answer 8

drugs (chemo)
infiltration of bone marrow (malignancy or infection)
endocrine: hypothyroidism, panhypopituitarism, low testosterone

Question 9

intravascular hemolysis of RBCs

Answer 9

haptoglobin binds Hgb

HgB-haptoglobin removed by RES – prevents iron-utilizing bacteria from benefitting from hemolysis

Question 10

extravascular hemolysis of RBCs

Answer 10

occurs in spleen and liver

Question 11

lab findings in hemolysis (both intra- and extra-vascular)

Answer 11

anemia w/ increased reticulocytes
increased LDH
indirect bilirubin
decreased haptoglobin

Question 12

lab findings specific to intravascular hemolysis

Answer 12

schistocytes
hemoglobinemia
hemoglobinuria

Question 13

clinical findings differentiating extravascular hemolysis from intravascular

Answer 13

intravascular –> hemoglobinemia and -uria

extravascular –> NO hemoglobinemia or-uria

Question 14

Immune hemolytic anemia

Answer 14

antibodies against RBCs

positive DAT or direct coombs

Question 15

Direct coombs test

Answer 15

are RBCs in here already bound by antibodies?

Question 16

tx for autoimmune hemolytic anemias

Answer 16

address cause
steroids
immunosuppression
anti-CD20 therapy (rituximab)
splenectomy

Question 17

IHA due to warm antibodies

Answer 17

IgG - spleen destroys antibody coated RBCS

Question 18

IHA due to cold antibodies

Answer 18

IgM - bind in cold areas (extremities, face), pick up complement on way to body center –> RBCs lyse in circulation or in the liver
caused by virus and mycoplasma

Question 19

drug induced hemolytic anemia - 3 mech

Answer 19

1. hapten (ex penicillin)
2. innocent bystander (ex quinine)
3. antigenic - drug incorporated into RBC membrane (ex methyldopa)

Question 20

microangiopathic hemolytic anemia

Answer 20

non-immune hemolytic process w/ prominent red cell fragmentation (schistocytes)

Question 21

causes of microangiopathic hemolytic anemia

Answer 21

1. TTP-HUS
2. DIC
3. malfunctioning heart valves

Question 22

TTP - HUS

Answer 22

thrombocytopenia and RBC fragmentation
assoc w/ ADAMSTS mutations, HIV, shiga-toxin E coli
tx plasmapheresis

Question 23

membranopathies causing normocytic anemia

Answer 23

1. hereditary sperocytosis

2. Paroxysmal nocturnal hemoglobinuria

Question 24

hereditary spherocytosis

Answer 24

autosomal dom inheritance, common in northern europeans
spectrin mutation –> decreasesd membrane mechanical stability –> RBCs can’t pass through splenic microcirculation, RBCs die prematurely
tx splenectomy

Question 25

paroxysmal nocturnal hemoglobinuria

Answer 25

acquired defect in cell membrane | missing GPI due to PIG-A gene defect --> RBCs vulnerable to complement --> intravascular hemolysis

Question 26

PNH s/sx

Answer 26

patient wakes up in morning w/ dark urine | significant risk of thrombosis

Question 27

PNH dx

Answer 27

flow cytometry - missing CD59 and CD55

Question 28

PNH tx

Answer 28

transfusions fe and folate supplementation eculizumab (anti-complement) stem cell transplant

Question 29

G6PD deficiency

Answer 29

problems w/ maintaining Hgb in face of oxidative stress dx: measure G6PD activity path: Heinz bodies and best cells x-linked

Question 30

what two infections cause hemolytic anemia?

Answer 30

- malaria | - babeosis

Question 31

sickle cell mutation

Answer 31

single amino acid substitution in beta chain of chrome 16: valine for glutamic acid --> Hemoglobin S

Question 32

how long do sickle cells live?

Answer 32

10-20 days (most removed by spleen)

Question 33

clinical manifestations of sickle cell disease

Answer 33

``` hemolysis delayed growth infection vaso-occlusive cerebrovascular events retinopathy aplastic episodes (parvovirus) ```

Question 34

tx for sickle cell

Answer 34

supportive tx for pain crises hydroxyurea - increases Hgb F, decreases Hgb S Hematopoietic stem cell transplant