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Brain Gainz > 4&5. Hemostasis and RBC disorders Pathoma > Flashcards

4&5. Hemostasis and RBC disorders Pathoma Flashcards

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1
Q

what is primary hemostasis

A

injury to a blood vessel that results in a PLATELET PLUG(stabilized by secondary hemostasis)

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2
Q

What is secondary hemostasis

A

Stabilization of the platelet plug via coagulation cascade

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3
Q

Steps of thrombogenisis

A

1)Endothelial damage= vasoconstriction 2)Exposure: vWF is exposed to collagen and binds 3)Adhesion: Platelets bind to vWF via Gp1b (conformational change) platelets release ADP and Thromboxane 4a)ADP helps platelets adhere to endothelium via increased Gp2b/3a receptors4b)platelet aggregation via FIBRINOGEN between Gp2b/3a receptors

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4
Q

Where does vWF come from?

A

Weibel-palade bodies of the endothelial cellsand Alpha-granules of platelets

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5
Q

Clinical signs of Primary hemostasis fuck up

A

Mucosal and skin bleeding(nose -EPISTAXIS-, gi, menstrual))bruising, petechia, purpura

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6
Q

Idiopathic Thrombocytopenia Purpura (ITP)what clinical values will you seeTx?

A

Autoimmune production of IgG antibodies against platelet antigens low platelet count, increased megakaryocytictx-corticosteroids, ivig (the spleen produces the antibodies and also destroys them. so ivig will distract the spleen to give the bodys platelets a chance to recover), splenectomy

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7
Q

Microangiopathic hemolytic anemia

A

Small blood vessels get a platelet thrombus that shears any passing RBCsShcistocytes (helmet cells)

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8
Q

Thrombotic thrombocytopenia purpura (TTP)tx=

A

platelet aggregation that leads to thrombocytopenia (purport from lack of platelets)Deficiency of ADAMTS13 (which breaks down vWF) so you have to much vWF which leads to increased platelet aggregationtx-plasmapharesis, steroids

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9
Q

Hemolytic-uremic syndrome

A

Thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failureseen in children after Etec infection caused by Shiga like toxin

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10
Q

Bernard-Soulier syndrome?whats the defect in?

A

a defect in platelet plug formation due to decrease in Gp1bdefect in platelet-to-vWF adhesionincrease bleeding time

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11
Q

Glanzmann Thrombasthenia

A

genetic deficiency in Gp2b/3aplatelet aggregation is impaired ( G2pb/3a is what connects platelet to platelet via fibrinogen

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12
Q

vWF diseasetx-

A

deficient in vWFtx- desmopressin

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13
Q

Vit. K deficiencey

A

Decrease in synthesis of factors 2,7,9,10,C,S7 having the shortest half-life

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14
Q

Warfarin

A

inhibits vit K synthesis via inhabiting epoxide reductase

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15
Q

Heparin induced Thrombocytopenia (HIT)

A

when you develop IgG antibodies against Heparin-bound platelet factor 4 (PF4)the antibodies activate platelets–> thrombosis and thrombocytopenia elsewhere

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16
Q

Disseminated inter vascular coagulation (DIC)whats the best screening test?

A

Secondary to other dzpathologic activation of coagulation cascadewidespread micro thrombi that leads to ischemia and infarctionConsumption of all these platelets leads to bleeding everywhere else (especially IV and mucosal sites)test- Elevated D dimers (when you split fibrin it produces D dimers)

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17
Q

What blocks activation of plasminogen

A

Aminocaproic acid

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18
Q

What two characteristics would you see in a thrombosis

A

Lines of zahnattachment to vessel wall

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19
Q

Vitamin B12 or folate deficiency leads to elevated ________

A

homocysteine levels

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20
Q

TetrahydroFOLATE to methionine

A

THF–> gives off methyl to Vit. B12—> gives methyl to Homocysteine —-> methionine

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21
Q

Whats the role of Protein C or S

A

Inactivate factors V and VIII in coag cascade

22
Q

whats the deal with factor V leiden

A

Resistant to degradation/inactivation by Protein C

23
Q

Whats are the anemia subtypes (based off of MCV)mean corpuscular volume (size of red blood cell)

A

Microcytic MCV 100

24
Q

Role of ferroportinRole of TransferrinRole of Ferritin

A

Transfers iron into the bloodDelivers iron to liver and bone marrow macrophagesIron Storage

25
Q

Why do you get anemia of chronic dz?

A

With chronic dz, you get prolonged inflammation. and in order to stop whatever is causing the sickness, the body increases hepcidin (which fucks up ferroportin and prevents release of iron from ferritin ) which decreases iron transport —-> iron deficiency —> anemiaferritin goes up/ TIBC goes downSerum iron goes down

26
Q

What does hepcidin do

A

Prevents release of iron from storage sites to deprive any infection of iron and kill it off

27
Q

Sideroblastic anemia

what are some of the causes

Tx?

A

Defect in protoporphyrin synthesis –> defect in Hb. due to X-linked defect in ALA synthase

defect in protoporphyrin leads to iron getting trapped in mitochondria of erythroblasts

congenitcal (ALA synthase), lead poisoning, alcoholism, or B6 Deficiency (pyridoxine)

tx-B6 pyridoxine

28
Q

what attaches Protoporphyrin to Iron (to make heme)

where does this happen

A

Ferrochelatase

in mitochondria

29
Q

Ringed sideroblasts

A

when protoporhyin synthesis is messed up so iron gets loaded into mitochondria.

This iron has nothing to bind to so its trapped there. These iron-laden mitochondrias form rings around the nucleus

30
Q

Thalassemia

A

Decrease in synthesis of Globulin chains

(leads to microctyic anemia)

31
Q

alpha Thalassemia

4 allele deletion

3 allele deletion

2 and 1 allele deletion

A

Normally you have 4 alpha alleles

Defect in A-globin deletion

4 allele-no alpha globin, excess gamma globin, (makes gamma4 -Hb Barts) Hydrops fetalis

3 allele- very little alpha globin, Excess ß-globin (HbH)

2/1 not clinicilaly severe

32
Q

whats the significance of cis vs trans deletions in alpha-thalassemia

A

cis is when 2 alpha alleles are knocked out on the same chromosome. this can lead to to severe thalasemia in offspring (Prevalent in Asains)

Trans isnt that bad (African population)

33
Q

ß-Thalassemia (2 beta genes)

Minor (ß/ß+) (normal/diminished)

Major (ß°/ß°) both B are wiped out

A

Gene mutation

minor-asymptomatic with Increase in HbA2

major

Absent Beta chain, target cells,

massive erythroid hyperplasia- expansion of hematopoiesis into marrow of Skull and facial bones (crew cut and chipmunk like face)

extramedulary hematopoiesis- liver and spleen starts to make it (hepatosplenomegaly)

Risk of Parvovirus B19- aplastic crisis

2° hemochromatosis

34
Q

Macrocytic Anemia

(Megaloblastic anemia)

MCV? what deficiencies cause it? why do the deficiencies lead to it

A

MCV>100

Folate or Vit. B12 deficiency

(1 less division so theyre macro) due to mess up of DNA precursors

Hypersegmented Neutrophils

35
Q

whats the role of folate and Vit B12 in synthesis of DNA (flow chart)

A

Tetrahydrofolate has a methyl and in order for it to make DNA it has to give that methyl to Vit. B 12

Vit B12 then gives that methyl to Homocystiene.

Homocystiene + methyl = Methionine

36
Q

Folate

Where is folate absorbed?

what casuses its deficiency

Clinical findings of deficiency

Lab findings

A
  • In the Jejunum
  • Poor Diet (alcoholics,elderly), Increased demand (preggos, cancer, hemolytic anemia), Drugs methotrexate
  • Megaloblastic anemia (macrocytic RBCs, Hypersegmented Neutrophils), Glossitis
  • Decreased serum folate(duh),
  • Increased* Homocysteine( piles up because folate doesnt pass Methyl to vit.b12 –>so vit b12 cant change homocysteine to methionine

Methylmalonic acid is Normal

37
Q

Vit. B12 (Cobalamin)

How is it absorbed/where

What causes its deficiency (alot)

Clinical and lab findings

A

First needs to be cleaved by pancreatic enzymes

Then, it binds to Intrinsic Factor (IF) to be absorbed in the Ileum. Large hepatic stores so it takes a long time to develop deficiency.

-caused by insufficient uptake (Vegan), malabsorption (Crohn dz), Pernicious anemia (Autoimmune destruction of parietal cells- cant make IF)

Diphyllobothrium Latum (fish tapeworm)

  • Macrocytic anemia with Hypersegmented neutrophils, Glossitis
  • Subacute Combined Degeneration of Spinal Cord -B12 converts methylmalonic acid –> Succinyl Coa. so without B12 you get a shit tone of mma which is toxic to myelin
38
Q

Pernicious Anemia

A

Autoimmune destruction of Parietal cells.

This leads to a deficiency of Intrinsic Factor (IF)

Which means you cant absorb Vit B12

39
Q

What are the 2 major processes that Vit B12 are invovled in?

A
  • In the synthesis of DNA, by accepting methyl from Tetrahydrofolate, then giving it to Homocysteine
  • Converts Methylmalonic acid —-> Succinyl CoA myelin synthesis

too much methylmalonic acid is toxic to myelin

40
Q

Normocytic Anemia

MCV?

what does the reticulocyte count look like?

A
  • MCV 80-100
  • Increased reticulocyte count/percentage (falsely elevated) due to destruction of RBCs

You must correct it tho.

41
Q

In normocytic anemia, what are the perameters for when you correct the reticulocyte count?

Whats the equation

A

>3% indicates good response by bone marrow; suggests peripheral destruction

underprodcution

Reticulocyte count x (Hematocrit/45)

in the example it would be a bone marrow problem

42
Q

Extravascular hemolysis

Which Anemia does it cause

What are clinical findings

A
  • Normocytic anemia
  • Extravascular destuction occurs from macrophages in spleen
  • Anemia with Splenomegaly, Jaundice due to unconjugated bilirubin (increased risk for gallstones), marrow hyperplasia with corrected reticulocount >3%

remember that when hemoglobin is broken down, the protoporhyrin of heme gets turned into Unconjugated billirubin

43
Q

Intravascular Hemolysis

What type of anemia does it cause

lab findings

A
  • Normocytic Anemia
  • Destruction of Hemoglobin in the blood vessel. Haptoglobin (binds to free Hb and brings it back to spleen)

Hemoglobinemia, Hemoglobinuria, Hemosiderinuria

44
Q

Haptoglobin

A

Binds free hemoglobin (Hb) and brings it back to spleen.

levels of haptoglobin are decreased in intravascular hemolysis bc they get used up

45
Q

Hereditary Spherocytosis

clinical findings

how is it diagnosed

tx?

A

Extravascular hemolysis due to defect in RBC membrane (ankyrin, band 3, spectrin are protein fuckups)

Small round RBC with no central pallor (premature removal by spleen)

Increased MCHC (mean corpuscular hemoglobin concentration) only dz with this

  • Splenomegaly, Aplastic crisis (ParvoVirus B19 infection)
  • osmotic fragility test
  • Splenectomy
46
Q

Sickle Cell Anemia

whats the mutation?

what causes the sickling

What protects against the sickling (tx)

clinical findings

A

Auto Recesive mutation in the ß gene (glutamic acid with valine)

  • Stressors such as Low O2, high altitude, acidosis, dehydration
  • HbF(fetal), give hydroxyurea to increase HbF

‘-Aplastic crisis (parvovirus B19),

Painful crisis: dactylitis (sausage fingers), acute chest syndrome, avascular necrosis

Autosplenectomy (howell jolly bodies)- due to infarct of the spleen. increases risk of infection S. pneumonia

Salmonella osteomylitis

47
Q

Hemoglobin C

whats the mutation

A

Auto Recessive mutation in ß Chain

Glutamic acid is replaced by Lysine

48
Q

Paroxysmal nocturnal hemoglobinuria (PNH)

why does it happen?

lab findings

what does it lead to

Tx

A

aquired dsyfunciton of GPI via hematopoietic stem cell

(anchors decay-accelerating factor -DAF CD55- that protects RBC from complement activation)

at night we breath slower leading to respiratory acidosis –> activation of compliment (intravascular hemolysis) which will fuck up these RBCs since theyre predisposed already

  • hemoglobinemia, hemoglobinuria
  • leads hemolytic anemia (duh), pancytopenia, thrombosis

10% of pts develops AML

-tx eculizumab (compliment inactivator)

49
Q

G6PD deficiency

whats its function

what causes oxidative stres

what cells does this produce?

Clinical signs

A

X-linked Recessive d.o –>reduced half life of G6PD

(increases RBCs succeptible to oxidative stress)

G6PD helps create NADPH. NADPH helps produce glutathione which protects against oxidative stress

-Infections, Fava Beans

Heinz bodies & Bite cells

Back Pain from all the released Hb(nephrotoxic)

50
Q

Immune hemolytic Anemia

What are the different types

what are they associated with

A

Warm IgG: EXTRAVASCULAR chronic anemia due to IgG binding to RBC and removing membrane (results in spherocytes). Associated with SLE and CLL (Methyldopa) / (Warm weather is GREAT)

Cold IgM: Acute anemia triggered by cold. Associated with CLL, Mycoplasma pneumonia, Infectious Myonucleosis. (Cold weather is MMMiserable)

51
Q

What is the Coombs test?

Direct. vs indirect

A

direct- do i have cells already bound by IgG? you give them anti- IgG. so if the answer is yes, the RBCs wil agglutinate and percepitate

Indirect- Does the pt have antibodies in their serum? normal RBCs added to patients serum. if serum has the antibodies, the RBCs agglutinate when Coombs is added

52
Q

Whats the CD for hematopoeitic stem cells?

Give the lineage of everything that it can produce

A

CD34

Brain Gainz (30 decks)

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