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IHO Week 2 > Anemia 2- Krafts > Flashcards

Anemia 2- Krafts Flashcards

1
Q

Hemoglobinopathies: Things you must know

Part of hemolytic anemias

A
  • Qualitative hemoglobin abnormality
  • Sickle cell is most important
  • Sickle cells –>hemolysis, vaso-occlusion
  • Usually just a point mutation in the beta chain
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2
Q

Hemoglobinopathies:

-Best lab test?

A
  • structurally abnormal Hgb
  • Best lab test: Hgb electrophoresis
    • Hb chains will migrate to different areas depending on if they have normal, heterozygous, of homozygous traits.
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3
Q

Process of sickle cell formation

A
  1. point mutation in beta chain gene (val for glutamat) –>HgbS
  2. aggregates and polymerizes on deoxygenation –>cell becomes sickle shaped
  3. hemolyze and also clog up small vessels
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4
Q

Clinical findings in SC anemia

A
  • African Americans (8% are heterozygous)
  • variable severity
  • Chronic hemolysis
  • vaso-occlusive disease
  • INCREASE infections (autosplenectomy) –especially encapsulated organisms
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5
Q

Morphology of SCA:

A
  • Sickle cells —DUH
  • “Post-splenectomy blood picture”
    • nucleated red blood cells
    • target cells
    • Howell-Jolly bodies: pieces of nucleus left
    • Pappenheimer bodies: pieces of iron
    • increased platelet count
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6
Q

Tx of SCA:

A
  • Prevent triggers: infection, fever, dehydration, hypoxemia
  • Vaccinate against encapsulated bugs (S. pneumoniae & H.influenzae
  • Blood transfusion
  • Bone marrow transplant
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7
Q

Thalassemia: things you must know

weird size

A
  • Quantitative defect in Hgb
  • Can’t make enough alpha or beta chains
  • severity varies
  • Hypochromic, microcytic anemia with INCREASED RBCs and target cells
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8
Q

Type of Hgb as a fetus?
At birth?

Adult?

A

Hgb F = 2alpha and 2gamma

At birth you make a little delta (Hgb A2 =2 alpha and 2 delta) –always make a little bit of this.

After birth you start making beta chains for normal Hgb A =2 alpha & 2 beta

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9
Q

Beta Thalassemia is better than alpha. Why?

A
  • can sometime make delta or gamma to make up for loss of Beta chains
  • NO replacement for alpha chains :(
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10
Q

Normal globin genes?

A

4 alpha chain genes

2 beta chain genes

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11
Q

Alpha vs beta thal?

A 
Alpha = DELETION of alpha chain gene 
Beta = DEFECTIVE beta chain gene (transcription, translation, or mRNA processing)
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12
Q

Beta thal severity?

A 
Beta = normal gene 
Beta+ = produces some Beta chains
Beta^0 = produces NO beta chains

beta-thal minor = asymptomatic
beta thal intermedia = less severe
beta-thal major =severe

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13
Q

Alpha thal problem

A

Alpha chain genes are ABSENT

  • must be missing 3 of 4 genes to get severe disease
  • missing all 4 = hydrops fetalis (incompatible with life)
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14
Q

What can cause anemia in alpha thalassemia?

A
  1. not enough alpha chains
  2. Excess unpaired beta, gamma, or delta chains

Newborns -gamma tetramers
Adults - beta tetramers –> eat up my macs

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15
Q

What can cause anemia in beta thal?

A
  1. not enough beta chains
  2. excess unpaired alpha chains
    - -> cells get eaten up by macs.
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16
Q

Morphology of Thal?

A

Hypochromic, microcytic anemia (**MOST COMMON)

  • some anisocytosis and poikilocytosis
  • target cells
  • basophilic stippling
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17
Q

How do you tell IDA from Thal?

A
  • -don’t have as much anisocytosis

- -all cells will be about the same size in Thal

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18
Q

X-ray findings in thalassemia?

A
  • Medually expansion –make bone marrow in places you usually don’t –>skull
  • in severe cases only
19
Q

Alpha Thal: clinical findings

A
  • Asians and African Americans
  • Carrier state and thal trait: asymptomatic
  • HbH disease: moderate –> severe disease
  • Hydrop fetalis: fatal in utero
20
Q

Beta Thal: clinical findings

A

Mediterranean, Blacks, Asians

  • Thal minor: usually asymptomatic
  • Thal major: variable severity, usually presents in infancy.
21
Q

Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD):

Things to know :)

A
  • decreased G6PD –> increased peroxides –>cell lysis
  • Oxidant exposure
  • Bite cells (removal of Heinz bodies)
  • Self-limiting!
22
Q

What’s so great about G6PD?

A

Need G6PD so you have NADPH to keep glutathione in proper state.

23
Q

Why do G6PD deficient red cells die?

A
  • Can’t reduce nasties!
  • ->Nasties attack Hbg bonds
  • ->Heme breaks away from globin
  • ->globin denatures, sticks to RBC membrane (Heinz body)
  • ->Spleen bites out Heinz bodies
24
Q

Where is highest incidence of G6PD?

A

-Areas with MALARIA

25
Q

G6PD deficiency: Clinical Findings

A
  • many asymptomatic
  • Others have episodic hemolysis
  • Usually you have enough G6PD to be a great person.
  • Triggers: FAVA BEANS & DRUGS (antibiotics, aspirin)
  • Spontaneous resolution
26
Q

G6PD deficiency: Morphology

A
  • Without exposure? no anemia
  • Exposure to oxidating agent –> acute hemolysis
    • -bite cells, fragments
    • -Heinz bodies (need special stain)
27
Q

Microangiopathic Hemolytic anemia (weird shape)

Things you musttttttt know, Morgan:

A
  • physical trauma to RBCs
  • Schistocytes (fragmented red cell)
  • Find out why! –lots of different reasons that you can help with.
28
Q

What are the causes of MAHA?

A
  • Artificial heart valve
  • Anything causing DIC, TTP, HUS –>get little blood clots all over the body.

(disseminated intravascular coagulation, thrombotic throbocytopenia purpura, hemolytic-uremic sydnrome) –but you don’t need to know these right now :)

29
Q

How fragmented cells form:

A

RBC gets caught on fibrin and is ripped open.

Seals itself back up as a fragmented cell / microspherocyte

30
Q

Schistocyte

A

in MAHA ONLY.

  • Triangulocytes
  • No central pallor
31
Q

Anemias with Normal Shape and Sized RBCs

A
  • Anemia of blood loss
  • AOCD
  • Anemia in kidney, liver disease
  • Aplastic anemia
32
Q

Anemia of blood loss:

Things to know!

A
  • Cause: traumatic, acute blood loss
  • At first, Hbg is NORMAL!!!
  • 2-3 days, see reticulocytes (BM is kicking up output)
  • Chronic blood loss is different (IDA)
33
Q

Anemia of Chronic Disease (AOCD)

Things you must know:

A
  • Infections, inflammation, malignancy
  • Iron metabolism disturbed
  • Normochromic, normocytic anemia
  • Anemia usually mild
34
Q

Pathogenesis of AOCD:

A

Disturbed iron metabolism–> hepcidin is over produced (ugh, can’t get iron out of storage)

  • shortened RBC survival
  • Impaired marrow response to anemia
35
Q

Labs: AOCD vs IDA

A

ACD: increased ferritin and marrow storage iron. Decreased TIBC

IDA: *decreased ferritin. decreased iron stores. Increased TIBC.

36
Q

Anemia of Renal Disease: things to know–>

A
  • end stage renal failure
  • cause: lack of erythropoietin
  • May see echinocytes (bumps on red cell)
37
Q

What causes anemia of renal disease?

A

Lack of erythropoietin :((((

38
Q

Anemia of Renal Disease:
Clinical Features?

Tx?

A

-Anemia severity roughly == degree of renal failure

tx: mild=none
severe=replace erythropoietin.

39
Q

Anemia of Liver Disease: things 2 know:

A
  • Anemia is frequent in liver disease (75%)
  • lots of causes
  • cases are usually complicated
  • Might see acanthocytes, targets
40
Q

Red cell shape in kidney vs liver disease?

A

Kidney = echinocytes: small bumps on membrane

Liver-acanthocytes: more pointy bumps

41
Q

Aplastic Anemia: Things to know!

A
  • Pancytopenia (low # of RBC, WBC, platelets)
  • Empty Marrow
  • Most are idiopathic
42
Q

Causes of aplastic anemia:

A

Idiopathic: most common

drugs, viruses, pregnancy, Fanconi anemia (congenital)

43
Q

Aplastic anemia: Clinical findings?

A
  • Pallor, dizziness, fatgue (anemia
  • Recurrent infection (leukopenia)
  • Bleeding, brusing (thrombocytopenia)
44
Q

Tx of aplastic anemia?

A
  • avoid further exposure
  • give blood products
  • Drugs: G-CSF, prednisone, ATG
  • BM transplant as last resort
  • 3 yr survival: 70%

IHO Week 2 (14 decks)

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