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Clinical medicine A > Lecture 3: Anemia I (Enoch's first 67, Billie is the rest) > Flashcards

Lecture 3: Anemia I (Enoch's first 67, Billie is the rest) Flashcards

1
Q

Why do females generally have a lower Hb?

A

Lower EPO stimulation due to more dilated kidneys from estrogen.

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2
Q

What anemias are classified as microcytic?

A

Thalassemias
Anemia of chronic disease
Iron defiency
Lead toxicity
Zinc deficiency

Microcytic because mice have TAILZ

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3
Q

What anemias are classified as normocytic?

A

Kidney disease
Non-thyroid endocrine heart failure
Copper deficiency
Mild form of most acquired microcytic or macrocytic etiologies of anemia

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4
Q

What anemias are macrocytic, specifically megaloblastic?

A

B12 deficiency
Folate deficiency
DNA synthesis inhibitors

I think megaloblastic means like the anemias that are caused by the direct inhibition of proliferation and maturation of the cells.

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5
Q

What anemias are macrocytic, specifically non-megaloblastic?

A

Myelodysplasia
Bone marrow failure state (aplastic anemia, marrow infiltrative disorders, etc)
Copper deficiency
Hypothyroidism
Liver disease
Reticulocytosis

“Many Bones can have low RBCs”

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6
Q

What does it mean if a hemolytic anemia is intravascular?

A

RBCs are being lysed within the BLOOD VESSELS.

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7
Q

Why is it significant if a hemolytic anemia is intravascular?

A

It is significant because large amts of Hgb are being released into circulation.

This will appear as a decrease in iron and haptoglobin.
This may appear as hemoglobinuria.
Iron will NOT be recycled. (it will decrease over time)
Schistocytes will form.

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8
Q

What is a schistocyte?

A

A tripolar, popped balloon RBC.

It is like a shredded RBC.

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9
Q

Why does haptoglobin decrease in intravascular anemia?

A

A serum haptoglobin test measures FREE haptoglobin, so if there is a lot of Hgb circulating, then the FREE haptoglobin gets used up.

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10
Q

What does it mean if a hemolytic anemia is extravascular?

A

RBCs are being lysed within ORGANS.

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11
Q

Why is it significant if a hemolytic anemia is extravascular?

A

Minimal Hgb released into circulation.

This means little to no decrease in haptoglobin.
Iron should remain almost unchanged. (because its being reabsorbed)

Spherocytes will form.

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12
Q

What is a spherocyte?

A

It is a smaller, darker, denser, hyperchromic cell.

It is essentially the formation of a damaged RBC repairing itself by collecting excess Hgb.

Excess Hgb = smaller RBC.

They are NOT biconcave and LACK central pallor.

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13
Q

Why does Iron remain unchanged in extravascular anemia?

A

Generally, the spleen is where most RBCs get caught, and it is able to recycle the Hgb from the lysed cells.

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14
Q

What is a key PE finding that may differentiate extravascular hemolytic anemia from intravascular?

A

Red-dark urine in intravascular anemia.
Hepatospenomegaly in extravascular anemia

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15
Q

If I have a hemolytic anemia, what lab changes would I expect on:
Hgb
MCV
Reticulocyte count

A

Hgb would be normal or reduced.

MCV are often increased.

Reticulocytes are often increased.

Note:
Hgb changes little because bone marrow can up the production of RBCs.
Upping RBC production means increasing retic count and generally MCV.

Retic count can remain unchanged if it is a chronic anemia and the bone marrow is exhausted.

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16
Q

If I have a hemolytic anemia, what lab changes would I expect on:
Bilirubin
LDH
Haptoglobin

A

Bilirubin would increase, esp. unconjugated.

LDH would increase.

Haptoglobin would only markedly decrease in intravascular anemia.
Haptoglobin MAY decrease in extravascular anemia.

Note:
Haptoglobin changes are dependent on the severity of the anemia.

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17
Q

What can falsely elevate haptoglobin?

A

Chronic inflammation.

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18
Q

Which hemolytic anemia is structural?

A

Hereditary spherocytosis

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19
Q

What hemolytic anemias are related to Hemoglobinopathies?

A

Thalassemias
Sickle cell disease (SCD)

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20
Q

What hemolytic anemia is metabolic?

A

G6PD deficiency

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21
Q

What hemolytic anemias are immune-related?

A

Autoimmune hemolytic anemia (AIHA)
Paroxysmal nocturnal hemoglobinuria (PNH)
Hemolytic disease of the newborn (HDN)

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22
Q

What is hereditary spherocytosis and what causes it?

A

Abnormal formation of proteins in the RBC membrane.

It is a result of improper cytoskeleton formation and is mainly AUTOSOMAL DOMINANT inheritance.

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23
Q

Is hereditary spherocytosis intravascular or extravascular? What does that mean?

A

Extravascular.

The spherocytes commonly get stuck in red pulp fenestrations in the spleen, which leads to phagocytic destruction

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24
Q

Describe the clinical presentation of spherocytosis.

A

Varying degrees…

It can go undetected for years but:

PE:
Jaundice
Splenomegaly
+/- gallstones in 50% of pts
Presents acutely after infection or stress.

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25
Q

Describe the peripheral blood smear of spherocytosis.

A

High counts of spherocytes.

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26
Q

Describe the lab findings in spherocytosis.

A

Variable decreases in Hgb/Hct
Increased reticulocytes
Increased MCHC (one of the few that can do this in micro/normocytic)
Normal to low MCV
Normal to low Haptoglobin (will drop more if inflammation present)
Peripheral blood smear: spherocytes
Coombs test: negative (ideally)

MOST IMPORTANT CHANGES:
^reticulocytes
^MCHC
- presence of spherocytes

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27
Q

What is the general basic Tx for hereditary spherocytosis? Severe? Definitive?

A

General is folic acid 1mg daily.

Transfusions can be used for severe anemia.
EPO may be used in infants to reduce need for transfusions

Definitive:
Splenectomy (partial or full)
Delay until after 5 yrs of age, or until puberty if moderate
■ If mild - may observe
■ Antipneumococcal vaccination

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28
Q

Why is iron supplementation CI in hereditary spherocytosis?

A

It is an extravascular anemia, so it is not losing iron, but recycling it.

Iron supplementation will overload it.

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29
Q

What are the types of hemoglobin found naturally in the blood?

A

HbA: 97-99% of Hb in RBCs and considered adult Hb. (2 alpha, 2 beta)

HbA2: 1-3% of Hb in RBCs (2 alpha, 2 delta)

HbF: <1% of Hb in RBCs (Fetal Hb). (2 alpha, 2 gamma)

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30
Q

How many copies of each globulin gene do we have?

A

2 copies of alpha-globulin in chromosome 16. (4 total)

1 copy of beta-globulin in chromosome 11. (2 total)
Also includes delta and gamma.

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31
Q

What causes alpha thalassemia?

A

Gene deletions related to the alpha-globulin gene, resulting in reduced alpha-chain synthesis.

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32
Q

What does alpha thalassemia present as on an electrophoresis?

A

Equal proportions of all 3 hemoglobins.

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33
Q

What is the pathology of an alpha thalassemia?

A

Increased small, pale (hypochromic) RBCs.

Excess beta chains precipitate → damage to RBC membranes → hemolysis
in marrow and in splenic vessels

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34
Q

What demographic is most susceptible to alpha thalassemias?

A

SEA and Chinese

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35
Q

At what point does alpha thalassemia start to show lab changes?

A

With 2 gene deletions, aka alpha thalassemia minor/trait. Hct and MCV will begin dropping.

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36
Q

What are the two concerning types of alpha thalassemias?

A

HbH disease (1 normal gene)
Hydrops fetalis (0 normal genes)

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37
Q

What lab findings would I expect in alpha thalassemia?

A

Hb/Hct: normal or decreased
RBC: increased
MCV: Markedly decreased
Retic: Normal or increased
MCH: Decreased
Hb electrophoresis: Presence of HbH band means HbH disease. Normal otherwise.
Inclusion bodies: HbH

Important ones:
^RBC
decreased MCV
decreased MCH
electrophoresis with HbH band or inclusion bodies indicating HbH

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38
Q

How would you describe alpha thalassemia morphologically?

A

Microcytic hypochromic anemia.

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39
Q

How does alpha thalassemia trait/minor present on peripheral smear?

A

Hypochromic, microcytic cells
Target cells (Ring appearance)

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40
Q

How does HbH disease present on peripheral smear?

A

Hypochromic, microcytic cells
Target cells
Poikilocytosis

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41
Q

How are the minor alpha thalassemias treated?

A

Minima/Silent carrier only require genetic counseling.
Minor/trait may rarely need transfusions.

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42
Q

Why do I need to monitor for iron overload in transfusion patients with thalassemia and how do I treat it?

A

Extravascular anemias such as alpha thalassemias do not lose iron, so it would build up if transfused.
Treated via iron chelation.

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43
Q

How are HbH and hydrops fetalis treated?

A

HbH: folic acid 1mg/day
Avoiding iron supplements and oxidative drugs.
Consider splenectomy and transfusions regularly.

Hydrops fetalis: almost always lethal in utero :(
Recommended to terminate pregnancy.

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44
Q

What causes a beta thalassemia?

A

Gene point mutations resulting in reduced beta-chain synthesis.

Beta+ = reduced
beta0 = absent

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45
Q

How does beta thalassemia present on electrophoresis?

A

Increased HbA2 and HbF.

AKA low HbA since little to no beta chains present anymore.

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46
Q

What demographic is most susceptible to beta thalassemias?

A

Mediterranean

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47
Q

Describe the pathology of beta thalassemia. (what kind of RBC’s and what causes that)

A

Increased numbers of SMALL, PALE (low Hb) RBCs

Excess alpha chains = hemolysis.
Excess alpha chains = inclusion bodies & damaged precursors.
Surviving RBCs will have inclusion bodies and have shortened lifespans.

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48
Q

What are the 4 types of beta thalassemias and how are they broken down in terms of beta genes?

A

Normal

Minor: Beta/Beta+ or Beta/Beta0

Mild(intermedia): Beta+/Beta+

Major/severe: Beta0/Beta+ or Beta0/Beta0

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49
Q

How do lab findings change as the beta thalassemia gets worse?

A

Generally:
MCV decreases
Hct/Hbg decreases
HbF and HbA2 increase
Reticulocytes - increased or normal
MCH - decreased

Transfusions start at mild/intermedia and progress to dependent if major.

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50
Q

What are the two types of beta thalassemia major?

A

Cooley’s anemia or beta-thalassemia major

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51
Q

What are the general lab findings for beta-thalassemia?

A

Hb/Hct decrease
RBC increase
MCV markedly decreased
Retic: increase
MCH: decrease
Hb electrophoresis: Decreased HbA

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52
Q

What are the most pertinent PE findings for mod-severe beta thalassemia?

A

Erythroid hyperplasia will lead to chipmunk facis, thinning of long bones, pathologic fx, and failure to thrive.

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53
Q

How does beta-thalassemia MINOR present on a peripheral smear?

A

Hypochromic, microcytic cells
Target cells

Note:
Similar to an alpha thalassemia.

54
Q

How does beta-thalassemia Intermedia present on a peripheral smear?

A

Hypochromic, microcytic cells
Target cells
Poikilocytosis

55
Q

How does beta-thalassemia Major present on a peripheral smear?

A

Hypochromic, microcytic cells
Target cells
Poikilocytosis
NUCLEATED RBCs

56
Q

How is beta thalassemia minor treated?

A

Minor requires just genetic counseling and rare transfusions.

Note:
Monitor for iron overload just like alpha.

57
Q

How is beta thalassemia intermedia treated?

A

Genetic counseling

Avoid iron supplements
Transfusions
Splenectomy (consider)
Iron overload

58
Q

How is beta thalassemia major treated?

A

Genetic counseling
Transfusion-dependent
Avoid iron supplements and monitor iron overload.
Splenectomy
Luspatercept (Reblozyl)

DEFINITIVE TX: Allogeneic bone marrow transplant.

59
Q

What is Luspatercept? Who uses it?

A

It is an Activin-A-trap drug indicated for transfusion dependent ADULT beta thalassemia pts.

CI: Splenectomy, pregnancy, breastfeeding.

60
Q

What causes SCD?

A

It is an autosomal recessive inherited disease.

Abnormal beta-chain substitution results in a betaS chain.

It can appear as heterozygous (trait) or homozygous (anemia)

61
Q

What is Hemoglobin S?

A

2 alpha chain, 2 betaS chain hemoglobin.

62
Q

What demographic is most susceptible to SCD?

A

African Americans (8% in US are carriers)

63
Q

Describe the pathology of SCD.

A

Unstable HbS polymerization results in sickle shaped cells.

Sickle shaped cells cause vaso-occlusion in all vessels and are sticky to endothelium.

This results in ischemia, pain, and end-organ damage.

64
Q

How does the spleen play a role in SCD? what triggers sickle cell ischemia

A

It sequesters and destroys the sickle RBCs.

sickle cell ischemia triggered by hypoxemia, acidosis, infection, excessive exercise, abrupt temperature changes, and anxiety/stress.

65
Q

Is SCD extra or intravascular?

A

Extravascular.

66
Q

What is the only real change between normal and SCT?

A

Decrease in HbA and presence of HbS on electrophoresis.

67
Q

What changes between SCT and SCD?

A

Decreased Hct
Lack of HbA
Increased HbF
Highly increased HbS

68
Q

How does sickle cell Disease present on lab findings (include electrophoresis)

A

Hgb/Hct - normal if trait, decreased if anemia
MCV - normal
Reticulocytes - increased (10-25% not increased enough to affect MCV)
WBC - elevated
Electrophoresis - presence of HbS band, decreased HbA normal HbA2, HbF normal in trait, elevated in anemia.

69
Q

How does sickle cell anemia present on a peripheral blood smear

A

Target cells, Sickled RBC’s, Howell-Jolly Inclusion body

Howell-jolly inclusion bodies are the little red dots in the cells.

70
Q

What is the clinical presentation of sickel cell trait

A

often asymptomatic
may have sighns/symptoms during high stress (high altitudes, heavy exercise, dehydration ect)

71
Q

What is the clinical presentation of sickle cell anemia

A

signs and symptoms begin around 6 months of age

Main ones to remember:
■ General - often appear chronically ill
■ Skin - jaundice, pallor, poorly healing ulcers of LE (>10 y/o)
■ Extremities - pain and swelling, dactylitis (“sausage fingers/toes”)
■ Eyes - vision changes, retinopathy, retinal detachment
■ Abdomen - splenomegaly (<3 y/o), hepatomegaly, gallstones
■ Chest - cardiomegaly, hyperdynamic precordium

Easy way to remember this is to think of common diabetes symptoms and combine it with common anemic symptoms

72
Q

what is the symptom in this picture called and what is it indicative of

A

dactylitis, indicative of sickle cell anemia

73
Q

What is the clinical presentation of a sickle cell crisis

A

■ Sickled RBCs obstruct microcirculation = ischemic injury to 1+ organs
■ Pain - sudden, lasts from hours to weeks, resolves spontaneously
■ Other symptoms - Fever, tachycardia, tenderness, anxiety

74
Q

what are the most common locations for sickle cell crisis.

how does this vary with age groups?

A

abdomen, bones, joints, soft tissues

most common bone areas for varying ages:
less than 18 months = hands/feet
childhood/teens = long bones of arms and legs
adults = vertebre

75
Q

what are triggers for sickle cell crises

A

physical stressors: hypoxia, dehydration, infection, acidosis
change in temp: fever or enviornmental
Other: fatigue, pregnancy, alcohol, psychosocial stress

76
Q

What should you observe for in sickle cell patients in an eye exam

A

retinopathy!

(the left one is unhealthy, right is healtht)

77
Q

what should you observe for during an abdominal exam in a patient with sicke cell anemia?

A

hepatomegaly and splenomegaly.

78
Q

What are some complications of sickle cell.
(there is literally so much on this card, just do your best)
○ Musculoskeletal -
○ Brain -
○ Renal -
○ Cardiac -
○ Pulmonary -
○ Immune -
○ Eyes -
○ Reproductive -
○ Liver -
○ General -

A

○ Musculoskeletal - necrosis of bone, osteomyelitis, aseptic necrosis
○ Brain - ischemic stroke, cerebral atrophy
○ Renal - chronic kidney disease, hematuria
○ Cardiac - cardiac enlargement and heart failure
○ Pulmonary - pulmonary hypertension
○ Immune - hyposplenism, impaired immune function
○ Eyes - retinopathy, vision loss, blindness
○ Reproductive - delayed puberty, priapism
○ Liver - hepatomegaly, hepatic failure
○ General - sickle cell crisis
■ ischemia manifested by acute pain and
tenderness, fever, tachycardia, and anxiety

79
Q

what are the five types of sickle cell anemia treatment

A

avoidance of triggers
supportive medications
disease modifying medications
acute crisis treatment
definitive treatment

80
Q

What are the supportive medications that a person with SCD may use. FAVOPTA

this is also alot oml sorry

A

Folic acid (1mg daily)
vaccinations (to prevent triggers)
ACE inhibitors (for patients w proteinuria due to kidney damage)
Omega 3 fatty acids (to reduce vaso-occlusive crisis)
transfusions (PRN for aplastic or hemolytic crisis)
pain management medications (opiates often used)
antibiotics (prohphylactic PCN daily until age 5 and then optional after that. this is to prevent infections and therefore sickle cell crisis)

81
Q

What are the three disease modifying medications for SCD

A

Hydroxyurea
Crizanlizumab
L-glutamine

82
Q

what disease modifying treatment is considered the mainstay of treatment for SCD. How does it work what are SE

A

Hyroxyurea - 500-750mg/d for patients with 3+ pain crisis per year

this med increases HbF levels. this reduces vaso-occlusive episodes and hospitalizations and prolongs survival of patients.

SE are major - bone marrow suppression (decreased WBC especially neutrophils) and may cause increase cancer risk

83
Q

what is the criteria for SCD patients to be treated with hydroxyurea

A

to have 3+ pain crisis per year

84
Q

what is hydroxyurea CI in

A

pregnancy! it is a teratogenic!

85
Q

what is the mechanism of action for crizanlizumab

A

(remember if a drug ends with MAB its usually a monoclonal antibody)

monoclonal antibody against P-selection proteins on endothelium that sickle cells tend to stick to. this means less likely vaso-occlusion.

SE are very mild

very expensive only given IV and must be given every 2-4 weeks

86
Q

What is the mechanism of action of L-glutamine and when is it used

A

used when patient cannot tolerate hydroxyurea

decreases vasoocclusive episodes possible by reducing oxidative stress that promotes sickling.

its expensiveeee

87
Q

what is the acute crisis treatment for sickle cell crisis

A

identify and treat underlying cause of crisis
“HOP” - hydrate, oxygenate, pain control

exchange transfusion done during crisis that are severe (pulling out patients blood and replacing it with donor blood that is not sickled)

88
Q

splenic sequestration crisis
what is it
what are effects
what is treatment

A

when alot of blood is stuck in the speen instead of in the rest of their body.

this causes a rapidly enlarging spleen and can lead to shock and death (10-15% mortality rate!)

this is usually treated via splenectomy

89
Q

what is the only definitive treatment for SCD

A

allogenic hematopoietic stem cell transplant.
only curative if organ damage is not already done

90
Q

What causes G6PD deficiency

A

X-linked recessive genetic defect
deficit in glucose-6-phosphate dehydrogenase enzyme (G6PD)
most common enzyme deficiency in humans

91
Q

what demographic is most common with G6PD deficiency

A

African-american males

rarely seen in females because x linked recessive

92
Q

what is the pathology of G6PD deficiency

A

RBCs are especially vuulnerable to oxidative stress.
Oxidative stress → Hb denatures, forms precipitate (Heinz bodies)
Heinz bodies damage RBC membrane → destruction by spleen (extravascular)
Cells can also spontaneously rupture (intravascularly)

93
Q

what is the presentation of G6PD deficiency

A

usually asymptomatic with episodic hemolytic anemia.
no splenomegaly
prolonged jaundice in newborns

acute episodes show malaise, weakness, abdominal or lumbar pain, jaundice and dark urine.

94
Q

what are the three main triggers for G6PD deficiency

A

infection, food (fava beans, other beans, blueberries, red wine) and certain medications.
medications include:
antibiotics: sulfas, quinolones, nitrofurantoin
phenazopyridine, aspirin.

95
Q

why is splenomegaly not commonly present in G6PD patients

A

spleen is only intermittently destroying RBC’s because this is an episodic disease.

96
Q

is G6PD deficiency intravascular or extravascular

A

BOTH!!:)

97
Q

what are the lab findings in G6PD deficiency

A

Hgb/Hct - normal, low during episode
MCV - normal
Reticulocyte - normal, increased during episodes
MCH - normal
G6PD assay - decreased, normal during or just after episode. (this measures how much of the enzyme is active)

MAIN:
during episodes:
Hgb/Hct - low
retic - increased
G6PD is normal during or just after an episode. it is usually decreased.

98
Q

What does the peripheral blood smear look like in patients with G6PD deficiency

A

“Bite” cells - look like they have a bite taken out of them
“Blister” cells - little small white spots in cells
Polychromataphils/reticulocytes

also heinz bodies but only seen when you add stain to the smear

99
Q

what are preventative measures for G6PD deficiency

A

avoidance of oxidant drugs
avoidance of trigger foods
genetic counseling

100
Q

what are the therapeutic measures for G6PD deficiency

A

Removal of offending agent
supportive - folic acid supplementation
rarely transfusions are needed

101
Q

what is the cause of autoimmune hemolytic anemia

A

Antibody forms against ssurface RBC antigens (mostly IgG)
50% - idiopathic (unknown cause)
Other 50% - associated with autoimmune diseases mainly Lupus or associated with WBC disorders like leukemia or lymphoma.

102
Q

what must autoimmune hemolytic anemia be distinguished from?

A

drug-induced immune hemolytic anemia
typically caused by ABX, -dopas, or NSAIDS

103
Q

presentation of AIHA

A

abrupt, rapid onset, potentially life threatening anemia.
fatigue, jaundice, splenomegaly
up to 10% mortality rate

104
Q

what is the pathology of AIHA

A

RBCs “tagged” for destruction by immune system
○ Splenic macrophages remove portions of RBC membrane → spherocyte
formation results
○ Spherocytes become trapped in spleen and are destroyed
○ Complement can also tag RBC for destruction by Kupffer cells in liver
○ IgM can help facilitate MAC-induced direct intravascular hemolysis

basicaly spleen and liver recongnize RBC’s as bad and start to send cells to eat them

105
Q

what are the two types of AIHA

A

warm - occurs at normal body temp
cold - only becomes active at colder temps (cold agglutinins)

106
Q

what would lab findings look like in AIHA

A

Hbg/Hct - decreased, can drop as low as 4g/dL and 10% within days
RBC - decreased
MCV - normal
Reticulocytes - increased ( not enough to increase MCV)
MCH - normal (hgb decreased because low RBC’s, therefore there is still adequate hgb on each rbc therefore MH is normal)
Platelets - 10% have immune thrombocytopenia (low platelets)

Main:
Hgb/Hct - severe decrease
RBC - decrease
retic - increase
Platelets - low

107
Q

what diagnostic testing is run when AIHA is suspected

my definition may be confusing, look on slide 75-77 for a better understanding if needed:) sorrrryyyyyy

A

antiglobulin (Coombs) test
Direct - tests whether RBC’s have Ig, complement or both on their surface (done by mixing IgM that attacks IgG or complement with patients RBCs to see if RBCs are attacked)
Indirect - testing plasma/serum for Ig against RBCs
( done by mixing serum/plasma with donor RBCs to see if they tag the cells and attack)

108
Q

what does a peripheral blood smear look like in AIHA

A

marked microspherocytosis
polychomataphils/reticulocytes

109
Q

what are treatements for AIHA

A

Immunosuppression - Prednisone 1-2mg/kg/day orally in divided doses.
Splenectomy if severe
Treatment of underlying comorbidities
cold avoidance if cold AIHA
transfusions often required

110
Q

why is it okay to give AIHA patients blood without type and crossing in emergencies

A

because the immune system is going to attack the blood cells anyways so it wont make a difference.

111
Q

what is the cause of Hemolytic disease of the newborn and what is it also known as

A

AKA: erythroblastosis fetalis
Cause: maternal IgG to antigens on surface of fetal RBCs
(moms body reacts to fetal blood cells and attacks them)

112
Q

what causes Hemolytic disease of the newborn (what causes antibodies to form)

A

fetal-maternal hemorrage
maternal transfusion
maternal pre-existing antibodies

113
Q

what are the ways that Fetal-maternal hemorrage can occur

A

fetal-maternal hemorrage is when moms blood and babys blood mixes
this happens with: placental abruption, invasive in-utero procedures, abortion, and childbirth.

114
Q

what causes Hemolytic disease of the newborn (what causes antibodies to form)

A

fetal-maternal hemorrage
maternal transfusion
maternal pre-existing antibodies

115
Q

pathology of HDN

A

maternal IgGs go through the placenta and into the babys blood stream to attack babys RBCs

116
Q

What are the most common and most severe types of HDN

A

most common is anti-ABO antibodies
most severe is anti-Rh antibodies

117
Q

what is the presentation of a newborn with HDN

A

severe jaundice
anemia at birth
positive DIRECT coombs test
hepatomegaly
splenomegaly
edema is possible
heart failure is possible

118
Q

How would the Coombs test differ between mom and baby in HDN? why do they differ

A

Mom - Coombs INDIRECT test positive, because her serum contains the Ig’s that are doing the attacking (direct is negative becuase she does NOT have any tagged RBC’s)
Baby - Coombs DIRECT test positive, because the RBCs of the baby are tagged with complement and Ig that marks them for attack. (indirect is negative because babys serum will not have Ig’s that are doing the attackng)

119
Q

What is before birth, after birth treatment for HDN

A

before birth:
Intrauterine fetal transfusion
Early induction of labor once fetus is old enough to survive
maternal plasma exchange (reduces circulation Ig levels)

after birth:
transfusion
supportive care

120
Q

what is the only preventative care for HDN

A

RhoGAM: prevents immune response to Rh+ blood in Rh- mother

121
Q

what is the cause of Paroxysmal Nocturnal Hemoglobinuria

A

Acquired genetic defect that leads to lysis of RBC’s
deficit in complement regulating cell membrane proteins CD55 and CD59

122
Q

what demographics are most affected by paroxysmal nocturnal hemoglobinuria

A

initial presentation most common in young adults but can happen in patients of any age. no evidence of inheritability, equally present in both genedrs

123
Q

pathology of Paroxysmal nocturnal hemoglobulinuria

A

RBC’s are vulnerable to lysis by complement
MAC formation → RBC destruction
Free Hb depletes nitric oxide
^^Causes esophageal spasms, erectile
dysfunction, renal damage,
thrombosis

124
Q

presentation of paroxysmal nocturnal hemoglobinuria

A

○ Episodic hemoglobinuria
■ Often first thing in AM → drop in blood pH overnight facilitates
hemolysis of RBC
■ Often improves throughout the day
○ May present with s/s of thrombosis
■ Especially in veins of mesentery, liver, skin, and CNS
■ Significant PNH - 10-15 year life expectancy
○ May present with s/s of anemia

125
Q

lab findings of paroxysmal nocturnal hemoglobinuria

A

○ Hb/Hct - Variably decreased
○ Reticulocytes - Normal or increased
○ MCV - Normal or increased
○ MCH - Normal
○ Iron - Normal or decreased
○ WBC - Normal or decreased
○ Platelets - Normal or decreased

126
Q

diagnostic tests for PNH

A

urine hemosiderin - can be + even after urine Hemoglobin is cleared
flow cytometry - gold standard

127
Q

treatment for PNH

A

mild: observe, no tx needed
severe or aplastic anemia: allogenic hematopoietic stem cell transplant
severe hemolysis or thrombosis - eculizumab (monoclonal ABX against C5)

supportive Tx - transfusions, iron replacement, corticosteroids to reduce hemolysis

128
Q

is iron deficiency anemia acute or chronic

A

chronic

129
Q

what is the main concern in patients who are experiencing anemia due to blood loss, what will be the problem with the CBC in this case?

A

hypovolemia
CBC will no show anemia due to fluid imbalance

130
Q

once a patient is no longer hypovolemic after blood loss, what will the CBC show

A

CBC will show anemia reflective of severity of blood loss

131
Q

what is the recovery process after fluids have been given in a scenario where a patient has lost alot of blood

A

bone marrow atempts to replace lost RBC’s with new ones (can increase RBC production up to 8x normal)
transient reticulocytosis occurs

132
Q

what are the supportive treatments for anemia due to blood loss

A

transfusion as indicated
fluid replacement as indicated
may need supplemental iron

Clinical medicine A (32 decks)

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