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Block 7; Week 3-Michelle > Anemia & Red Cell Disorders II-Usera > Flashcards

Anemia & Red Cell Disorders II-Usera Flashcards

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1
Q

What are some potential causes of normocytic anemias–reticulocyte count of <3%?

A 
Acute blood loss
Early iron deficiency and ACD
Aplastic anemia
Chronic renal failure
malignancy
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2
Q

What are some intrinsic defects that can cause normocytic anemia?

A

Membrane defect
Abnormal Hb
Enzyme deficiency

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3
Q

What are some extrinsic defects that can cause normocytic anemia?

A

Trauma (e.g. aortic stenosis, prosthetic valves)

Immune destruction

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4
Q

What’s the deal with extravascular hemolysis

A

Rbc phagocytosis by splenic and hepatic macs
IgG bound, with or without c3b
Abnormal shape (e.g. spherocytosis, sickling)

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5
Q

What are the lab findings for patients who experience extravascular hemolysis?

A

Increase unconjugated bilirubin

Increased serum LDH

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6
Q

What’s the deal with intravascular hemolysis?

A 
Hemolysis occurs within blood vessels
Enzyme deficiency (g6pd)
Mechanical trauma
Complement/immune destruction
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7
Q

What are the lab findings for patients who are experiencing intravascular hemolysis?

A

Increase unconjugated bilirubin (minimal)
Increased serum LDH
Decreased serum haptoglobin
hemosiderinuria

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8
Q

What is haptoglobin?

A

an acute phase reactant that complexes with hemoglobin

CD163 receptor on macrophages recognizes the Hp-Hb complex.

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9
Q

What does the degradation of Hp-Hb complex by the macrophage produce/release?

A

bilirubin

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10
Q

What’s the deal with hereditary spherocytosis?

A

aut dom
US 1/5K
Intrinsic Defect-usu mutation in ankyrin
could also be caused by mutations in band3, spectrins, protein 4.2

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11
Q

What are the clinical features of hereditary spherocytosis?

A

Jaundice-chronic hemolysis
Gallstones-accumulation of calcified bilirubinate
Splenomegaly
Aplastic crisis in children

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12
Q

What are the lab findings for hereditary spherocytosis?

A

normocytic anemia
increased MCHC–sphere holds more hemoglobin & shrinks at the same time
increased osmotic fragility-bursts in hypotonic solution
elevated LDH & bilirubin

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13
Q

What is the treatment for hereditary spherocytosis?

A

splenectomy

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14
Q

What’s the deal with hereditary elliptocytosis?

A

aut dom
defect in spectrin or protein 4.1 that binds the alpha spectrins
>25% peripheral blood elliptocytes
not a fatal disease

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15
Q

What are the main clinical findings for patients with elliptocytosis?

A

mild anemia

splenomegaly

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16
Q

What causes paroxysmal nocturnal hemoglobinuria?

A

acquired membrane defect in myeloid stem cells
mutation in Pig-A gene
mutation in GPI causes loss of decay accelerating factor

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17
Q

What’s the deal with paroxysmal nocturnal hemoglobinuria?

A

shows intravascular complement mediated lysis
episodic hemoglobinuria
happens at night b/c of the acidosis brought on by shallowing breathing-favors complement
can lead to iron deficiency
increased risk of thrombosis b/c of all the random platelets running around.
increased risk of acute myeloid leukemia

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18
Q

What is the normal role of decay accelerating factor when it isn’t mutated in PNH?

A

it protects the cell from complement-mediated damage by inhibiting C3 convertase
should prevent the formation of the membrane attack complex
mutated GPI prevents DAF from connecting to cells

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19
Q

What would the labs look like for a patient with paroxysmal nocturnal hemoglobinuria?

A

Normocytic anemia with pancytopenia
Decreased haptoglobin
Increased serum/urin hb

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20
Q

What’s the deal with sickle cell anemia?

A

aut rec
missense mutation: glutamic acid substituted with valine
affects the beta globin chain
10% African Americans have the trait (heterozygous)
60% messed up beta globin or more–disease. Called HBS

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21
Q

What is HbS?

A

Hemoglobin with the following structure:

alpha2beta2S

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22
Q

When do you recognize that a patient has sickle cell anemia in the absence of testing?

A

only once the cells have sickled!

that is when they are deposited in capillaries. Macrophages desperately try to remove them from circulation.

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23
Q

Initial sickling is reversible via administration of ____.

A

Oxygen!

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24
Q

What type of hemolysis occurs in sickle cell anemia? What are its triggers?

A 
extravascular hemolysis mainly
Triggers:
low ph 
low oxygen tension
Volume depletion
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25
When is the earliest age that sickle cell anemia can present? Why is this?
Earliest: 6 mo b/c neonates still have their fetal hemoglobin HbF that does not consist of beta chains (alpha 2gamma2)
26
What are the clinical findings for sickle cell anemia?
``` Dactylitis—thing in the fingers Acute chest syndrome (most common cause of death) Stroke Gallstones-calcified bilirubinate priapism-yikes! Aseptic necrosis of femoral head Aplastic crisis (ass. With parvovirus) Autosplenectomy (splenomegaly by 2 yo, then loses function) – Howell-jolly bodies ```
27
How does acute chest syndrome present in patients with sickle cell anemia?
dyspnea wheezing chest pain back pain
28
Why are patients with sickle cell anemia more susceptible to infections? Which types?
b/c their spleen is dysfunctional strep pneumonia encapsulated organsims salmonella paratyphi-can cause osteomyelitis
29
What are the renal findings seen with sickle cell anemia?
sickling in peritubular capillaries b/c of low O2 there renal papillary necrosis microhematuria (dead RBCs in tubules)
30
What is the treatment for sickle cell anemia?
Infectious prophylaxis Pain management Transfusion – acute chest syndrome, aplastic crisis—only 2 indications.
31
T/F You should transfuse a patient with sickle cell anemia who presents to the ER with priapism.
FALSE
32
What are some preventative measures for patients with sickle cell anemia--to keep them from experiencing crisis?
Hydroxyurea Immunizations Pneumococcal vaccine Folic acid supplementation
33
What happens with G6pd deficiency?
lack of G6pd means lack of NADPH lack of NADPH means that it is hard to make glutathione this is critical for protecting the cell from oxidative stress at risk for hemolysis & infection
34
Why are patients with g6pd deficiency at greater risk for infection?
b/c our body often uses oxidative stress to fight infections. MPO deals with that oxidative stress but it needs NADPH--produced by g6pd
35
What are some drugs & compounds that compromises enzyme fcn in patients with g6pd deficiency?
drugs: primaquine, an anti-malarial compounds: fava beans (used in middle east)
36
Give some important genetic/demographic info about g6pd deficiency.
``` recessive X--linked pattern affects 22% of U.S. blacks females are asymptomatic older RBCs usually affected young RBCs have normal or near normal levels ```
37
What important inclusion body do you see in g6pd deficiency histo?
heinz bodies: precipitated denatured hemoglobin | **See yellow RBCs & blue bodies.
38
With which condition do you see bite cells on the histo slides? Why?
g6pd deficiency | the macrophage removes the damaged membranes
39
What are the clinical & lab findings in patients with g6pd deficiency?
-sudden onset back pain and delayed hemoglobinuria -Susceptibility to infections (impaired MPO no nadph) -normocytic anemia -Heinz bodies RBC enzyme analysis after hemolytic episode has resolved: *g6pd levels are ~normal in retic and young cells
40
What type of anemia is immunohemolytic anemia? What are its subcategories?
normocytic extrinsic hemolytic anemia with intra or extravascular hemolysis ``` Warm agglutinins Cold agglutinins complement Drug induced Paroxysmal Cold Hemoglobinuria (PCH) ```
41
What's the deal with warm agglutinin immunohemolytic anemia?
``` happens 70% of the time triggered by warm temps IgG coated RBCs eaten by macrophages happens in the central part of the body extravascular hemolytic anemia ```
42
Which conditions are associated with warm agglutinin immunohemolytic anemia?
associated with SLE, collagen vascular diseases (SLE), CLL, malignant lymphoma (esp. Hodgkin’s), viral infections, etc.
43
What's the deal with cold agglutinin immunohemolytic anemia?
``` happens 30% of the time triggered by colder temps IgM coated RBCs eaten up intra or extravascular hemolysis happens in more distal parts of the body includes Raynaud's Phenomenon ```
44
What are some conditions associated with cold agglutinin immunohemolytic anemia?
causes include: Mycoplasma pneumoniae, infectious mononucleosis, CLL, drugs, etc.
45
What is an example of complement mediated extravascular hemolysis?
C3b coated RBC phagocytosed by liver macrophages
46
What is an example of complement mediated intravascular hemolysis?
RBCs coated by C5-C9 membrane attack complex
47
What are some clinical findings of immunohemolytic anemia?
Jaundice Hepatosplenomegaly Raynaud’s phenomenon
48
WHat are some lab findings associated with immunohemolytic anemia?
``` Positive dat Positive indirect Unconjugated hyperbilirubinemia Hemoglobinuria Decreased haptoglobin Normocytic anemia Rbc agglutination (igm perhaps) ```
49
Explain how penicillin relates to drug induced immunohemolytic anemia.
``` penicillin binds to RBC membrane antibody (IgG) binds to the penicillin fragment extravascular hemolysis direct coombs test positive IHA ```
50
Give some drug-antidrug immune complex examples.
IgG or IgM bind complement IgG → extravascular hemolysis IgM → intravascular hemolysis drugs: quinidine, quinine, INH, sulfonamides
51
What is the appropriate treatment for immunohemolytic anemia?
``` discontinue the offending drug corticosteroids immunosuppression splenectomy IVIG--binds macrophages--tricks them into thinking they have bound the antigen. ```
52
What types of things will you see on a peripheral smear if you are experiencing traumatic hemolysis?
cell fragments | schistocytes, burr cells, helmet cells
53
What is the relationship b/w DIC: disseminated intravascular coagulation & traumatic hemolysis?
DIC can cause traumatic hemolysis | traumatic hemolysis can also leave a bunch of cell fragments lying around & lead to coagulation & DIC
54
What are other possible causes of traumatic hemolysis?
long distance running artificial heart valves thrombotic thrombocytopenia purpura
55
What are some causes of nonimmunologic hemolytic anemia?
``` hypersplenism microangiopathic hemolytic anemia (MAHA) microorganisms, i.e. malaria, babesia, Clostridium perfringens snake venoms, i.e. cobra venom chemical physical, i.e. burns ```
56
What causes alloimmune hemolytic anemia?
transfusion reaction allo--someone else's blood intra or extravascular hemolysis the extra hemoglobin is toxic to the kidneys **also newborn getting maternal antibodies--can lead to anemia & hyperbilirubinemia
57
What are the lab findings for patients with alloimmune hemolytic anemia from a transfusion reaction?
``` DAT anemia depending on severity total and indirect bilirubin elevated haptoglobin ↓ LDH ↑ ```
58
What are the lab findings with alloimmune hemolytic anemia from a newborn getting maternal antibodies?
positive DAT ↑ total and indirect bilirubin LDH ↑ initially hematocrit and hemoglobin may be within normal limits
59
Which antibody is capable of crossing the placenta?
IgG
60
Which systemic disease displays anemia?
``` renal disease (uremia) Normochromic, normocytic mild anisocytosis sometimes hypochromic, microcytic burr cells – shrunken RBCs with irregular projections (echinocytes) usually present when BUN is twice normal ```
61
What are the possible mechanisms by which renal disease causes anemia?
bone marrow suppression hemolysis from impaired renal excretion coagulation defects (in severe disease) leading to blood loss impaired erythropoietin production from renal endocrine failure
62
Explain the deal with anemia with neoplasia.
often seen with bone marrow malignancies. usually normochromic and normocytic unless there is: blood loss hemorrhage a myelophthisic process—relating to the bone marrow mild hemolytic component often present severe with some lymphomas possibly due to the altered endothelium of malignant tissues (a set-up for DIC)
63
Which types of infection can cause anemias?
malaria | bacterial toxins--clostridium perfringens
64
What are the 2 causes of megaloblastic macrocytic anemias?
folate deficiency | vit b12 deficiency
65
What is the cause of the nonmegaloblastic macrocytic anemia?
alcoholism
66
Which histo indication do you see in patients with megaloblastic anemia?
a huge nucleus b/c they are struggling in their mitotic dreams pretty sure you will also see a hyper segmented neutrophil
67
What are some causes of vit b12 deficiency?
``` Pernicious anemia-autoimmune destruction of parietal cells Pure vegan diet Malnutrition Malabsorption ↓ Intrinsic factor ↓ Gastric acid ↓ Intestinal absorption ```
68
Describe how vit b12 is absorbed.
B12 combines with R binders (secreted by chief cells). Acid frees elemental B12 from what you have consumed so that it can combine with R. R goes thru duodenum. Pancreatic enzymes cleave. B12 can combine with intrinsic facotr. This is absorbed in terminal ileum.
69
HOw can PPIs mess with Vit b12 absorption?
don't have enough gastric acid to free the B12 to bind with R binder.
70
How can Crohn's disease mess with Vit B12 absorption?
messes with SI | Vit B12 absorbed in the terminal ileum
71
What are the 3 main categories of causes for folate deficiency?
decreased intake malabsorption drug inhibition
72
What are some decreased intake causes of folate deficiency?
Malnutrition Etoh-inhibits folate absorption Goat milk Infants/elderly
73
What are some malabsorption causes of folate deficiency?
Celiac-not B12, but folate b/c that is absorbed in jejunum | Bacterial overgrowth
74
What are some drug inhibition causes of folate deficiency?
``` 5-FU-chemo MTX-chemo, arrest DNA maturation & synthesis Tpm-sfx Phenytoin Ocp’s etoh ```
75
How long does it usu take to become Vit B12 deficient?
usu about 6-9 years b/c of the b12 that is stored in your liver
76
What does IFB mean in terms of intestines?
Iron--absorbed in the duodenum Folate--absorbed in the jejunum B12--absorbed in the terminal ileum
77
What is the pathogenesis of megaloblastic anemia?
Delayed nuclear maturation Affects all rapidly dividing cells Cellular RNA and protein synthesis unabated **cytoplasm matures faster than nucleus
78
If you are folate or VB12 deficient you could get elevated ______. What are the consequences of this?
homocysteine consequences: damage to vascular endothelial cells vascular disease & atherosclerosis
79
What type of hypersensitivity reaction is involved in pernicious anemia? What else do these patients suffer from?
Type II Hypersensitivity Autoimmune destruction of parietal cells (85-90%) Antibodies that block B12-if binding (60-75%) also suffer from achlorhydria b/c unable to have the parietal cells produce acid
80
Which blood type is more susceptible to pernicious anemia...for whatever reason?
Blood Group A individuals
81
What are some clinical findings for pernicious anemia? Which of these is def not seen in folate deficiency?
smooth/sore tongue & red (lose papillae) peripheral neuropathy** loss of vibration & proprioception--can't sense the tuning fork. dementia
82
What causes the loss of VP in Vit B12 deficiency?
demyelination of dorsal columns
83
Which conditions do you see raised urine methylmalonic acid in?
Vit B12 deficiencies--pernicious or not.
84
What are some lab/clinical findings shared in both Vit B12 & folate deficiencies?
hypersegmented neutrophils high MCV pancytopenia high homocysteine
85
What is the best test for detecting folate deficiency?
Decreased serum and RBC folate (best test)
86
Folate deficiency puts kiddos in utero at risk for what?
neural tube defects
87
What is the treatment for folate deficiency?
Intramuscular injections of b12 | Oral administration of monoglutamic folic acid
88
What's the deal with nonmegaloblastic macrocytosis?
anemia not always present chronic alcoholism is the most common cause liver disease can cause it-check for elevated cholesterol levels on histo you will see target cells

Block 7; Week 3-Michelle (6 decks)

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