Red Blood Cell Disorders Flashcards

1
Q

What is hematopoiesis - and what sites produce RBC

A

Creation of RBCs and as a fetus occurs in the the spleen, LNs and liver, moves into bone marrow and is produced there from birth and in adulthood.

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2
Q

Approach to anemia

A

Macrocyctic (High MCV) - B12/folate deficiency, drug induced, liver disease, hypothyroidism
Microcytic (Low MCV) - Thalassemia, chronic disease, iron deficiency, lead pointing, sideroblastic
Normocytic (normal MCV) - bleeding/ hemolysis (high reticulocytes), aplastic, renal disease, leukaemia (low reticulocytes)

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3
Q

How do the kidneys regulate RBCs?

A

EPO - kidney senses low O2 and releases EPO from HIF2Alpha, if kidney damage will have anemia

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4
Q

Mechanism of coagulation

A

Vasoconstriction, Primary hemostasis - platelet aggregation (VWF), secondary hemostasis (formation for platelet clot - fibrin clot formation - cross linking), fibrinolysis

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5
Q

Order of the clotting cascade?

A
12.
   11. 
      9.      7 
        8.   3
        10 
          5
          2
          1

7 is the extrinsic pathway, 12 is the intrinsic, Fibrin is activated factor 13 which is activated by the common pathway, and factor 10 activates thrombin

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6
Q

Mechanism of clot break down

A

TPA converts plain which cuts up the fibrin mesh, and healthy cells actually secrete things to prevent breakdown
D- dimer is the breakdown products

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7
Q

Vitamin K dependant clotting factors?

A

2, 7, 9, 10 - needed to convert to functional form, produced by gut bacteria and found in diet. Warfarin stops recycling vitamin K

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8
Q

When to transfuse blood

A

Hbg <70 and symptomatic, consider if hemodynamically stable

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9
Q

Iron supplementation?

A

Ferrous gluconate is the preferred supplementation - ideally three times a day. But it not tolerated very well.

IV - people will get a reaction, just order Benadryl in advance.

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10
Q

What does ferritin indicate? What is the TIBC?

A

Serum ferritin - indicated level of iron stores - low = iron deficiency, high can be an inflammatory marker

TIBC - indirect measure of transferrin (transporter that allows for cellular storage)

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11
Q

Reason for anemia in CKD?

A

Decreased EPO.

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12
Q

Signs and Sx of anemia

A

Pallor, fatigue, tachycardia, nail changes, glossitis

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13
Q

How do you test for hemolytic disease of the newborn?

A

direct coombs

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14
Q

What is HUS? And what is it caused by?

A

Hemolysis due to shiga toxin (e.coli) - most common cause

Will present with AKI, bloody diarrhea, weakness, fatigue

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15
Q

What causes TTP?

A

Deficiency of ADAMTS13, clotting in small vessels leading to low platelet count. Results from excessive vWF on platelets

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16
Q

Abnormally high Hb?

A

Consider polycythemia Vera, could be secondary to COPD/ pulmonary fibrosis, kidney disease, dehydration, smoking (CO exposure), hypoxia (adaptive response to high altitudes), EPO/ synthetic supplementation.

17
Q

Hemochromatosis?

A

Too much iron in body.

18
Q

Risk of transfusions?

A

Autoimmune rxn, possible infection from blood borne illness, volume overload, hyperkalemia, iron overload

19
Q

How does endothelial injury trigger clotting?

A

Exposure of of VWF and collagen, combined with decreased release of NO and PG will trigger platelet and immune recruitment. VWF will trigger platelet binding. Fibrogen will bind the platelets together and get cleaved to fibrin to form a mesh. This process is mediated by the clotting cascade.

20
Q

Microcytic anemia?

A

TAILS

Thalassemia, Anemia of chronic disease, Iron deficiency, Lead intoxication and Sideroblastic anemia

21
Q

Normocytic anemia

A

Hemorrhage
Hypoxia
Hemolysis

22
Q

Macrocytic anemia?

A

B12 and folate, liver disease, thyroid dysfunction, aplastic anemia