11/4 and 11/5 Anemia 1 AND 2 Flashcards

1
Q

List the steps of maturation into a red cell

A
  • Pronormoblast
  • Basophilic Normoblast
  • Polychromatophilic Normoblast
  • Orthochromatic Normoblast
  • Reticulocyte (Polychromatic Erythrocyte)
  • Erythrocyte
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2
Q

Normal Hgb value?

A

12-15

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3
Q

Normal Heatocrit value?

A

40-50%

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4
Q

Normal RBC/uL?

A

5 million

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5
Q

MCV, MCHC, MCH, RDW stand for?

A
  • mean cell vol
  • mean cell Hb conc.
  • Mean cell Hb
  • red cell distribution width
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6
Q

How long after acute blood loss till reticuloctyes show in blood?

A

1 wk (polychromatophilic cells- slightly bluer cytoplasm)

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7
Q

T/F blood smear will show anemia after acute loss of 2,000 mL of blood?

A

F. (takes time for blood dilution to occur)

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8
Q

Labs of hemolytic anemia? (3)

A
  • Up indirect bilirubin
  • Up LDH
  • Down haptoglobin
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9
Q

Chronic hemolytic anemia blood smear features?(2) Marrow features?(1)

A
  • Reticulocytosis (polychromasia)
  • Anisocytosis (variations in size, since young RBC are bigger)
  • More erythroid precursors in marrow
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10
Q

intracorpuscular defect is?

A

problem in the RBC [so extracorpuscular would be any other problem]

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11
Q

intravascular hemolysis is?

A

destroyed inside vessel (only 2 examples are fragmentation anemia & complement-mediated hemolysis) [Thus extravascular would be anything else, including when spleen destroys RBC’s]

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12
Q

Labs of intravascular hemolysis? (4)

A
  • Up: plasma Hb
  • Down: haptoglobin
  • Urine pos. for: Hb, Hemosiderin [these 2 always absent in extravascular!]
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13
Q

hereditary spherocytosis is in what population? incidence? molecular defect?

A
  • N. Europeans
  • 1 in 5000
  • spectrin, ankyrin, band 3 (ankyrin most common)
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14
Q

What causes the hemolysis in hereditary spherocytosis?

A

spleen. [removal is curative!]

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15
Q

G6PD deficiency: how diagnosed? Inheritance?

A
  • quantitate levels of G6PD in blood

- X-linked

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16
Q

Which RBC’s are most affected by G6PD deficiency? How would we treat this?

A
  • older cells [young unaffected cuz have more enzyme]

- avoid oxidants

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17
Q

I have blister cells. What disease do I have?

A

G6PD likely. Heinz bodies will also be present.

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18
Q

I have target cells. What disease do I have?

A

hemoglobinopathies (S, SC, C)

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19
Q

I have shistocytes. What disease do I have?

A

-Fragmentation hemolytic anemia (basically anything that causes shearing of RBC’s, like DIC, TTP, prosthetic heart valve)

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20
Q

I have spherocytes. What disease do I have? (2 possible)

A

-Immune hemolytic anemia (because a key protein is bitten off by spleen)
OR
-Hereditary spherocytosis

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21
Q

I have Howell-Jolly bodies. What disease do I have?

A

Your spleen is gone. Either: 1) Removed (hereditary spherocytosis treatment) or 2) Destructed (by Sickle Cell)

[Howell-Jollies are retained nuclear material]

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22
Q

I have a valine at 6 position of Beta globin chain. what do I have?

A

Sickle trait.

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23
Q

I have a lysine at 6 position of beta globin chain. What do I have?

A

Hemoglobin C trait.

24
Q

When do sickle cells crystalize? What removes these?

A
  • homozygous trait with low oxygen tension

- macrophages

25
What organs are most affected by painful infarcts in sickle cell?
- Spleen, bones | - Also strokes, retinopathy, kidney, lungs
26
Why do you not want your spleen removed?
Increases chance for infection
27
T/F sickle cells are seen in a heterozygote for sickle? (S/A) T/F sickle cells are seen in a heterozygote for S/C?
F. (asymptomatic, no blood abnormalities!!) | T. (sickle crises occur but are less severe)
28
T/F people with sickle cell disease have a complete lack of Hb A on electrophoresis?
T! (a key lab finding, along with presence of Hb S)
29
T/F Hb C crystals are present in C trait (CA)? | How about in SC disease?
F. (also asymptomatic!) | T.
30
What are the symptoms of Hb CC disease?
MILD anemia WITHOUT painful crisis
31
3 types of fully assembled hemoglobin are each composed of what globin chains? HbA, HbA2, HbF
HbA=alpha, beta HbA2=alpha, delta HbF=alpha, gamma
32
I have a disease class with BOTH ineffective erythropoiesis and hemolysis. What class of disease do I have? What does this give me protection against?
- Thalassemia | - Plasmodium falciparum malaria
33
Alpha thalassemias: what type of mutation? What happens when 2 genes are affected? 3? 4?
- deletion | - 2 is mild anemia (3 is moderate anemia w/ Hemoglobin H, 4=hydrops fetalis)
34
Beta thalassemia: What type of mutation?
-point, splicing
35
My RBC's have membrane damage due to a precipitate, I have no or mild anemia with microcytosis, and elevated Hb A2. What disease do I have?
Beta thalassemia minor (alpha chains precipitate).
36
I have a furry skull X-ray, skeletal deformities, systemic iron overload, and excess alpha chains. What disease do I have?
Beta thalassemia major (iron overload due to chronic transfusions)
37
I have a positive direct antiglobulin test (Coombs) and spherocytes. What disease?
Immune hemolytic anemia (Antibodies against red cell membrane)
38
2 types of microangiopathic hemolytic anemia are?
- TTP (thrombotic thrombocytopenic purpura) | - DIC
39
I have anemia, low reticulocytes count, oval macrocytes, hypersegmented neutrophils, hypercellular bone marrow, and ineffective erythropoiesis. What disease?
Megaloblastic anemia.
40
I have megaloblastic anemia, but I started taking a supplement for it and it went away. Now I'm starting to go insane. What do I have?
B12 deficiency (the anemia can be corrected by folate supplementation, neural defects can't)
41
Most common cause of B12 deficiency?
- Pernicious Anemia (autoimmune to intrinsic factor) | - Others: vegans, tapeworms, etc
42
I've got anemia with high homocysteine & methylmalonic acid labs. What anemia?
B12 deficient
43
I've got anemia with high homocysteine & normal methylmalonic acid labs. What anemia?
Folate deficient.
44
Folate stores: where? how long do they last?
- liver | - 2-4 months
45
B12 is absorbed where? how long do stores last?
- terminal ileum | - several years
46
Where does iron get absorbed in GI? Where is it stored in the body?
- duodenum | - RBC's
47
Fe deficiency due to chronic blood loss is most commonly due to what?
GI bleed, or menses in reproductive age women
48
I have microcytic, hypochromic anemia with high TIBC. What will my serum ferritin levels be?
low (I have Fe deficient anemia)
49
I have hypochromic anemia with low TIBC and high ferritin. What will my serum iron be?
-low (I have anemia of chronic inflammation)
50
What is the most common cause of anemia? 2nd?
- Fe deficiency | - Anemia of chronic inflammation
51
I have anemia of chronic inflammation. What cytokines are increased? What is the end effect of these cytokines?
- IL-6, BMP-2 | - Liver makes hepcidin, which causes iron storage in marrow macrophages
52
Aplastic anemia is usually caused by? Peripheral blood finding is?
- idiopathic (fatty replacement of marrow, MAYBE activated T cells release cytokines which suppress stem cells) - pancytopenia
53
My marrow has been replaced, and now I have anemia. What's my anemia called?
myelophthisic anemia
54
I've got low erythropoietin. What's my anemia?
Anemia of renal failure or uremia
55
I've got myelodysplastic syndrome. What is that, anyway?
neoplastic marrow stem cell clones
56
Polycythemia vera is caused by? what's treatment?
- neoplastic marrow stem cell proliferation due to JAK2 V617F gain of function mutation - phlebotomy
57
A 50-year old tells you he itches like crazy when he gets out of the tub, and occasionally has thrombosis, and sometimes bleeding. What does he have?
polycythemia vera