B&l: 6. Turnicky : Accelerated Cell Turnover/ Normochromic Macrocytic

Front 1

Premature removal of circulating red cells due to lysis within the circulatory system (intravascular) or premature removal by the reticuloendothelial system of spleen, liver and BM (extravascular)

describes what type of anemia

Back 1

Normocytic hemolytic anemia

Front 2

Pt has a fever with low back pain, this can be a sign of what? what type of pt is this seen in

Back 2

hemolytic anemia

this is normally associated with hemolytic transfusion reactions, you have to stop the transfusion

Front 3

an acute exacerbation of hemolysis due to activation of macrophages usually secondary to viral infection describes?

Back 3

Hemolytic Crisis

seen after infection with reved up macrophages

Front 4

impairment or cessation of BM red cell production describes? What is it associated with?

Back 4

Aplastic crisis

(parvovirus B19)

Front 5

What is hemoglobinemia? is it normal? when is it seen?

Back 5

Hemoglobin in the blood in free form

this is not normal

this is seen When transport proteins are depleted free Hb circulates in the plasma

Front 6

What are the 2 forms of hemolysis?

Back 6

Extravascular: (happens in the spleen, liver, and marrow; do not get hemoglobinemia)

Intravascular: happens in the vessels, leads to hemoglobinemia

Front 7

what is responsible for lysing RBCs in extravascular hemolysis

Back 7

Complement bound to RBC leads to activation of IgM, then leads to macrophages taking a bite out of them... this is not normal, and can be due to viral infection

Front 8

Hemolytic anemia falls under what category of anemia?

Back 8

Normochromic normocytic

Front 9

this type of anemia has >4% retic count, giving you reticulocytosis. Due to the fact that the reticulocytes are larger, you may see an elevated MCV...What does this refer to?

Back 9

Hemolytic anemia

(Normochromic normocytic)

Front 10

Spherocytes, Target cells, and Schistocytes are commonly found cells in what type of anemia?

Back 10

Hemolytic anemia

(Normochromic normocytic)

Front 11

what type of cell are you likely to see in the following:

Hemolytic –uremic syndrome (HUS)
Thrombotic thrombocytopenia purpura (TTP)
Disseminated intravascular coagulation (DIC)
Prosthetic heart valves

Back 11

Schistocytes

Front 12

Premature red cell destruction by an immune mediated process (antibody and/or complement) =

Back 12

Immune Hemolytic Anemia

Front 13

What is the most common form of Immune Hemolytic Anemia? What mediates it?

Back 13

Warm Immune Hemolytic Anemia

Mediated by IgG

Front 14

In Warm Immune Hemolytic Anemia, what is the Ab directed against?

Back 14

the Rh system

This makes it hard for you to be transfused because everyone hast the Rh system

Front 15

IgG
37 degree thermal amplitude
IgG or C3b attachment to macrophage receptors
Extravascular or intravascular hemolysis
Antibody directed against high incidence antigens eg anti-Rh

this describes?

Back 15

Warm Autoimmune Hemolytic Anemia

Front 16

IgM affixes to the red cell membrane in a cool environment, activates C reactivity, <37 degrees.

What does this describe?

Back 16

Cold Autoimmune Hemolytic Anemia

Front 17

What is the temperature that determines warm vs cold hemolytic anemia?

Back 17

Warm >37

Cold<37

Front 18

IgM
<30 thermal activity
Hemolysis due to binding and activation of C by IgM, with complement components becoming attached to rbc in cold
Extravascular hemolysis

this describes what anemia?

Back 18

Cold Autoimmune Hemolytic anemia

Front 19

Why is tranfusion so dangerous with patients who have autoimmune hemolytic anemia?

Back 19

If severe anemia results

Transfusion will involve incompatible units of blood and should proceed only if clinically mandated
Presence of alloantibodies must be excluded, however, the autoantibodies may interfere with blood bank tests

Alloimmune: Antibody formation for foreign antigens (another individual’s red cells)

Front 20

what is Alloimmune, and what 2 populations do you see it in?

Back 20

Alloimmune: Antibody formation for foreign antigens (another individual’s red cells)

seen in:
Hemolytic transfusion reaction
Hemolytic disease of the new born

Front 21

How does hemolytic disease of the newborn work?

**TEST

Back 21

The mother is Rh Negative

The 1st baby is Rh +, so when the fetal blood and mother's blood mix, the mom starts getting sensitized to the foreign blood

If the next baby is Rh+, the next baby is in trouble, B cells will eat up their blood because they see it as foreign

Front 22

What is the treatment for hemolyitc disease of the newborn?

Back 22

Anti-D

give to Rh- mother who just had 1st Rh+ baby

this is to try and prevent the development of antibodies for subsequent births

Front 23

What is ABO incompatibility?

Back 23

Mother type O; baby A or B
First pregnancy and subsequent pregnancies may be affected
Mild anemia, mild bilirubinemia usually peaking 48 hrs after birth

Front 24

A baby is born and looks pretty yellow, maybe a bit weak. You are not too worried though, because you know you will treat with light therapy and that baby will be fine. What happened to the kid?

Back 24

ABO incompatibility

Front 25

Penicillin and cephalosporin can cause what anemic problem? What are the 2 types?

Back 25

Drug-related Hemolytic Anemia

Drug/protein(in plasma and not red cell) form neoantigen
IgG or IgM adsorbed on to the rbc in a nonimmune reaction, but activates C

Incomplete C3d, extravascular phagocytosis
Complete C activation; intravascular lysis

Front 26

What is the Coombs test?

Back 26

detects red cells sensitized with Antibody and/or Complement invivo

Front 27

Indirect Antihuman Globulin Test does what?

Back 27

detects antibodies in the SERUM (as opposed to antibody on patient cells as in DAT/Coombs)

Front 28

What are the two types of Iron and which binds O2?

Back 28

ferrous (Fe+2) --> O2 binding

ferric non-oxygen binding form (Fe+3)

Front 29

Heinzbody formation comes from a deficiency of what?

Back 29

G6PD (reduces free radicals!)

Front 30

What happens in Pyruvte Kinase deficiency?

Back 30

Deficient conversion of ADP to ATP resulting in:

Rbc potassium leak and membrane deformity
Sequestration in the red pulp of spleen

Front 31

What happens in hereditary spherocytosis?

Back 31

Deficiency in cytoskeleton spectrin protein

Cells lack flexibility, trapped in spleen.

Only one with elevated MCHC

Front 32

RBC membrane disorders are more common in blacks and is due to...

Back 32

Defective spectrin chains with increased permeability to Na+

Front 33

pt says that when they wake up in the morning their urine is dark red. They say that during they day they have no problems thought. What could be going on?

Back 33

Membrane disordersParoxysmal Nocturnal Hemoglobinuria

Intermittent bouts of IVH and nocturnal hemoglobinuria
Exacerbataed during sleep, remits in day
Simply a chronic ongoing hemolysis

Front 34

in Paroxysmal Nocturnal Hemoglobinuria what is causing the problem (mechanism)

Back 34

Two normal regulators for preventing complement amplification are missing on the rbc membrane

so the RBCs are getting lysed

Front 35

what is the only acquired hemolytic anemia?

Back 35

Paroxysmal Nocturnal Hemoglobinuria

Front 36

40% of Paroxysmal Nocturnal Hemoglobinuria pts will develop what during the course of their disease

Back 36

thrombosis

Front 37

The following can cause what type of anemia?

Disseminated intravascular coagulation
Disseminated cancer
Eclampsia/pre-exlampsia
Hemolytic uremic syndrome
Thrombotic thrombocytopenia purpura

Back 37

Microangiopathic Hemolytic Anemia

Front 38

What are the 3 most common/significant Microangiopathic Hemolytic Anemias?

Back 38

DIC
TTP
HUS

Front 39

What is the classic finding in Microangiopathic Hemolytic Anemias?

Back 39

Schistocyte

Front 40

What is the tetrad found in Hemolytic Urimic Syndrome (HUS)?
*

Back 40

Hemolytic anemia with schistocytes
Acute renal failure
Thrombocytopenia
CNS symptoms (seizures)

Front 41

kid comes in, mom says he has diarrhea that she thinks he got shortly after a picnic. You find schistocytes in his blood. What does he likley have?

Back 41

HUS

Front 42

Why is TTP such a big deal

Back 42

90% will die without treatment due to multiorgan failure

Front 43

TTP is a disorder of what?

Back 43

vWF due to decreased metalloprotease (cannot cleave vWF appropriately)

Endothelium secretes vWF in a form that contains unusually large vWF multimers that are normally cleaved by a metalloprotease to smaller vWF multimers.

This metalloprotease is also referred to as vWF cleaving protease or ADAMTS-13

Front 44

vWF cleaving protease or ADAMTS-13 =

Back 44

TTP

Front 45

What will the metalloprotease levels be like in TTP and HUS respectively

Back 45

Patients with TTP have decreased metalloprotease (congenital or acquired secondary to autoantibody to the metalloprotease)

Patients with HUS do not have a decrease in metalloprotease

Front 46

Therapy for TTP?

Back 46

Daily plasma exchange or plasma infusion with corticosteroids.

Splenectomy
Immune suppression
Rituxin

Front 47

Normochromic Normocytic anemia resulting from premature red cell destruction =
**

Back 47

Hemolytic Anemia

Front 48

What is a constant feature of Hemolytic anemia?
**

Back 48

Reticulocytosis

Front 49

in Hemolytic anemia, what will you see in a peripheral smear?
**

Back 49

Spherocytes
schistocytes

Front 50

what type of bilirubin might you see in hemolytic anemia?

Back 50

Unconjugated

look for juandice!