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IPAP > Heme 1 Cram > Flashcards

Heme 1 Cram Flashcards

1
Q

EPO specifically stimulates ? cell to start erythropoiesis?

What are the steps of thrombopoiesis?

A

Prothromboblast- erythroblast

Liver puts C-TPO in circulation
C-TPO-> endothelium protein
Receptor forms on platelets, TPO enters cell, internalization
Activation, pseudopodia

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2
Q

Myeloid stem cells differentiate into ?

Lympoid stem cells differentiate into ?

A

Basophil
Eosinophil
Neutrophil
Monocytes

B cells- plasma cells
T cells
Natural Killer Cells

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3
Q

Define Hct

Define MCV

Define MCH

A

% of total blood that’s RBCs

Average size/volume of RBCs

Mean Cell Hgb- average quantitiy of Hgb in RBCs

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4
Q

Define MCHC

Define RDW

Define Anisocytosis

A

Average concentration of Hgb inside RBCs w/ size/vol accounted for

Red Cell Distribution Width, estimates range of volume/size of RBCs

Difference in sizes and volumes between RBCs

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5
Q

Most important function of RES?

What are the two types of RES?

A

Phagocytosis

Intravascular: RBC contents released into circulation, Hgb binds to haptoglobin, taken to liver

Extravascular: in spleen; Fe carried by transferrin to marrow; bili carried by albumin to liver

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6
Q

What part of the erythorcyte life cyle involves the extravascular RES?

What are the a/granular WBCs

WBC w/ Diff includes w/ cells

A

Fe bound to transferrin

Gran: BEN
Agran: LM

BLEMN Baso Lympho Eosino Mono Neutro

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7
Q

Neutrophil

Eosinophils

A

MC form of WBC w/ pale lilac granules
Forms bulk of pus
Bacteria infxn Inflammation Burn Stress

Red/orange granules
Allergic Parasitic AutoImm

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8
Q

Basophils

Lymphocytes

A

Blue/purple granules
Allergy Leukemia Hypothyroid

Agranular w/ sky blue rim around nucleus
Viral infxn Leukemia Mono

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9
Q

Monocytes

Cell mediated immunity release ? five cytokines

A

Agranular horse-shoe shaped nucles w/ gray cytoplasm, foamy
Viral/fungal infxn TB Leukemia Chronic Dz

IL Chemokine Lymphokine
IFN TNF

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10
Q

Define the following prefixes

A/An

Aniso

Pan

Poly

Poikilo

A

W/out

Unequal

All

Variable

Irregular

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11
Q

Define the following suffixes:

  • cytopenia
  • cytosis
  • emia
  • osis
  • penia
A

Absent cells

Refers to cells

Refers to blood

Process or condition

Lack or deficient

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12
Q

What are the 3 severe Sxs of anemia?

What are some PE findings of anemia

A

Syncope Angina MI

Dyspnea
Tachy- relates to severity
Fatigue
Geographic/smooth tongue
Palpitations
Pica
Cheilosis- usually B12 deficient
Spooning nails
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13
Q

? lab result is used to classify anemia?

If suspected anemia, and RTC is high, what are the causes of hemolysis?

A

MCV

Membrane abnormalities
Enzymopathies
Hgbopathies
AutoImm
Microangiopathic
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14
Q

DDx’s for microcytic anemia

When assessing for the cause, all lab results are compared to ?

A 
Thalassemia
Anemia Chronic Dz
Iron deficient*
Lead poisoning
Sideroblastic

Fe Deficient

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15
Q

Microcytic anemia Dx chart

A 
ITFTTSL
Thal: UDU
ACD: DDUDDNL
IDA: DUDUDUH
LP: UNU
Side: UNU
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16
Q

What is the use of Ferritin

What lab result directly relates to the amount of Fe stored in the body

A

Storage protein w/in cells, reflects amount of stored Fe

Serum Ferritin

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17
Q

What is the use of Transferrin

What is the use of TIBC

What is the use of Hepcidin

A

Transport protein of Fe

Blood’s capacity to bind Fe w/ Transferrin

Hormone that regulates Fe storage, released by liver during infection/inflammation

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18
Q

What is the use of Erythroferrone

What is the use of Ferroportin

What is Hemosiderin

A

Suppresses hepcidin synthesis

Transports Fe from diet across intestinal lumen

Insoluble form of Fe only found inside of cells, formed when body has Fe excess

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19
Q

Where is Fe absorbed in the GI tract

How much does Fe fluctuate in/out per day

What is a normal Fe content for the whole body

A

Duodenal erythrocytes

1-2mg

3-4gm

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20
Q

How is Fe storage broken up across the body

What lab result can NOT be used to Dx Fe Deficiency

Under normal conditions, how much of transferrin is bound by Fe

A 
RBC Hgb- 1800mg
Liver parenchyma- 1000mg
Spleen/ReticuloMacro- 600mg
Marrow/Muscles- 300mg
Plasma transferrin- 3mg

Serum Fe

1/3

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21
Q

What are the 4 stages of IDA development

How is mild/mod IDA Tx

How are severe cases Tx

A

1: dec ferritin (Fe stores)
2: RBCs turn hypochromic
3: Hgb dec
4: MCV dec

1st line w/ PO replacement:
Ferrous Sulfate 325mg QD, up to TID

IV Fe therapy w/ IMC consult

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22
Q

Ferrous Sulfate can interact w/ ? med

What is the most appropriate response to Tx of IDA

How long is Tx continued after values return to normal

A

Levothyroxin, take 4hrs apart

Hct returns halfway w/in 3wks, to baseline w/in 2mon
Hct/Hgb corrected w/in 8wks

6mon to replace stores

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23
Q

How is ACDz Tx

Define a thalassemia

A

Underlying condition
If severe- RBC transfusion or IV recombinant EPO

D/o of defected globin chain synthesis leading to reduced Hgb synthesis

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24
Q

What is the Hgb make up of Hgb F

What is the composition of Hgb Bart’s

What is the composition of Hgb H

What is the composition of Hydrops Fetalis

A

A2G2

GGGG

BBBB

–/– ‘0 alpha’

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25
Q

What Hgb is the last one to start developing in utero but lasts into adulthood?

How are thalassemia’s definitively Dx?

A

D

Electrophoresis

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26
Q

What type of Hx will PTs w/ thalassemia have?

What type abnormal RBC morphologies may be seen on smears?

A

FamHx
Hx of microcytic anemia

Microcytes
Acanthocytes- Greek, thorn
Codocytes- target cells

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27
Q

What would Chromosome 16 look like in a PT w/ A-Thalassemia (minima/trait)

What would the chromosome look like in a PT w/ A-Thalassemia minor

A

AA/AA Normal
AA/A- Trait

AA/– Homozygous
A-/A- Heterozygous

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28
Q

What would Chromosome 16 look like in a PT w/ A-thalassemia Intermedia

What form of A-Thalassemia mainly affects Asians or Blacks?

A

Hgb H: A-/–

Heterozygous A-/A- Asian
Homozygous AA/– Black

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29
Q

What is the life expectancy for PTs w/ A-thalassemia minor trait

How are PTs w/ Hgb H Dz managed

A

Normal

Daily folate, avoid iron
Transfusion during hemolytic exacerbations

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30
Q

What drug classes do PTs w/ Hgb H Dz need to avoid

Since B-thalassemias have impaired B production, what substitutions can occur?

A

Anti malaria bacteria
Sulfa
Analgesis
TB

Excess A chains can pair w/ D chains (HbA2) or G chains (HbF)

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31
Q

What happens to the A-globins in cases of B-thalassemia

What PTs have more severe cases of B-thalassemias?

A

Unstable and damage RBC membranes

Homozygous impaired B-globin synthesis

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32
Q

What two types of B-Thalassemia have basophilic stippling

What form of B-Thalassemia has nucleated RBCs?

A

B minor/trait
B intermedia

Major- Cooley’s Anemia

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33
Q

What form of thalassemia appears abnormal on electrophoresis

Only ? thalassemia has heterozygous genes?

A

B, due to G/D globin chains
G= Hgb F
D= Hgb A2

B-Thalassemia minor

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34
Q

B thalassemia intermedia globin combos

B thalassemia major globin combos

A

B+/B+

Bo/Bo or,
B+/B+

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35
Q

? Thalassemia is transfusion dependent and is a majority of HgF

How is A-Thalassemia trait or mild B-thalassemia Tx

A

B-Thalassemia Majors

None, identify to avoid future eval/Tx for Fe deficiency
Hgb H- Folic acid supplements and avoid Fe/Oxidative drugs

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36
Q

How is B-Thalassemia intermedia/major Tx

What can develop if these PTs are over Tx?

A

Transfusions Folate Splenectomy
Marrow Transplant- TxOC for Major

Too many transfusions= iatrogenic Fe overload syndrome

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37
Q

What lab result is Dx of Sideroblastic anemia

What causes this d/o

A

Bone marrow Fe >10% ringed sideroblasts

Serum Fe is avail but isn’t utilized by mitochondria= RBCs w/ iron granule in mitochondria

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38
Q

What lab method is used to visualize ringed sideroblasts

Why are stippled basophils also seen in this lab test?

A

Prussian blue (Perl’s reaction) shows ring of blue granules

Indicative of disrupted erythropioesis

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39
Q

How is sideroblastic anemia Tx

How does lead poisoning cause anemia

A 
Support
Hgb <7= PRBC transfusion
PO Pyridoxine (B6)

Impairs heme synthesis
Inc RBC destruction

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40
Q

What lab result is used to differentiate lead poisoning as a recent or chronic exposure

How is lead poisoning anemia Tx

A

Free Erythrocyte Protoporphyrin
Inc L, Normal FEP= recent
Inc L, Inc FEP= chronic

1st: Succimer
2nd: Penicillamine
Severe/lead encephalopathy: Dimercaprol + EDTA (crosses BBB- causes Peds neuro/learning disabilities)

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41
Q

What are the 4 conditions that present w/ basophilic stippling?

What do the lead panel results look like for results w/ stippling in them?

A

B minor/intermediate
Lead poisoning
Sideroblast

Inc TIBC Fe
Low Ferritin

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42
Q

What are the two types of Macrocytic anemias due to?

A

Mega:
B12/Folate deficient, Drugs

Non-mega: AHEAD MS
Alcohol
Hypothyroid
Emphysema
Accelerated erythopoiesis
Drug induced
Marrow d/o
Surface area, inc on RBC
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43
Q

To initiate hematopoiesis, EPO stimulates ? cell

Megaloblastic anemia has their etiology starting w/ ? cell

A

Proerythroblasts

Erythroblastic stage

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44
Q

If hypersegmented neutrophils are seen on a peripheral smear, this indicates?

What is the criteria for hypersegmented neutrophil?

A

Megaloblasts in marrow

Neutrophil nuclei w/ +6 lobes or,
+3% neutrophils w/ five nuclear lobes

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45
Q

How is it brought into the GI system for use

A

HCl releases pepsin, seperates B12 from foods
Parietal cells release IF, absorbs B12 in terminal ileum
TC2 from WBCs carries B12

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46
Q

Liver contains ?mcg of stored B12 meaning it takes ? long for a deficiency to occur

What is B12 roles in DNA synthesis

A lack of B12 causes a build up of ?

A

2-5000mcg
>3yrs after absorption ceases

Used for converting m-CoA into s-CoA in mitochondria

Methylmalonic acid

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47
Q

What is the most sensitive test for B12 deficiency at the cellular level

The lack of B12 for DNA synthesis causes ? to be formed

A

MMA test

Hypersegmented neutrophils

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48
Q

What types of diets can cause B12 deficient anemia

? type of drug abuse can cause this?

What is the name of the cobalamin metabolism d/o that can lead to this?

A

Strict vegan no dairy, meat, fish
Only breast fed baby of vegan mother

NO- present w/ spinal degeneration

Imerslund Grasbeck Syndrome

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49
Q

What are the biggest 3 Sxs that present w/ B12 anemia in order of presentation

Why does B12 deficiency cause neuro Sxs?

A

Peripheral neuropathy: paresthesia of hands
Ataxia
Dementia

Involved w/ myelin production

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50
Q

What is the ‘5 P’s of B12 Deficiency” mnemonic?

A 
Pancytopenia
Peripheral neuropathy
Papillary atrophy (atrophic glossitis)
Posterior column neuropathy
Pyramidal tract signs
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51
Q

What is the only lab result that differs between B12 deficiency anemia and pernicious anemia?

How is B12 deficiency anemia Tx

A

IFBA/Serum Gastrin tests

Genetic cause: lifetime Tx
Reversible: until corrected and Sxs are gone
Vit B12 but NOT nasal route

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52
Q

Pts w/ B12 Deficiency anemia but no Sxs are Tx w/ ?

What PT population may need more aggressive repltion and how is that done?

A

PO 1000mcg/day
IM 1000mcg/mon

Sx anemia
Neuro/psych Sxs
Pregnant
1000mcg every other day x 2wks
Cyanocobalamin qMon or,
Hydroxocobalamin once q2-3mon
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53
Q

How is Pernicious Anemia Tx

How are PTs w/ Lichtheims Dz that have B12 and folate deficiency Tx

A

Parentera Tx for life
Initial dose 1000mcg IM or DeepSQ x 4wks then
1000mcg qMon

Replace B12 first, prevents neuro Sxs
Folate would only correct Heme Sxs, not neuro

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54
Q

What is the sequence of response to Tx for B12 deficiency

Where is folate acid normally found, absorbed and how can it be destroyed?

A

Erythropoiesis normalizes 2-3 days
Hypersegment neutrophils gone by 14 days
Neuro Sxs gone in 6mon if reversible

Fruit Veggies Animal protein
Prolonged cooking
Prox small intestine

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55
Q

Body normally stores about ? folate which is a ? supply

What meds were highlighted as causing folate deficient anemia

A

5000mcg
2-3mon

Anti convulsants
ABX- sulfas
HIV meds

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56
Q

How is folate deficient anemia Tx

A

PO 1000mcg PO per day x 1-4mon or until complete hematological recovery

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57
Q

What would be the difference in lab results form someone w/ folate deficient anemia and someone w/ combo B12 and folate anemia

What are the two classic characteristics of megaloblastic anemia not seen in Non-Magalo Macro Anemia

A

Combo: Dec B12, Inc MMA

Oval macrocytes
Inc RDW

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58
Q

When is a marrow biopsy warranted during a non-megalo work up?

Sine there are no megalobasts in non-mega anemia, what would be seen on a marrow sample?

A

No Aplastic CLDz ETOH
Unexplained megalo or,
Myelodysplasia suspected

Megaloblastoid RBC precursors (proerythroblasts) w/ nuclear chromatin

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59
Q

? anemia has inc RBC surface area

? meds can cause this type of anemia w/ inc surface area

A

Non megalo macro- inc deposition for cholesterol/lipids

Benzenes

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60
Q

Define MDS

What are 3 causes

A

Marrow d/o of ineffective erythropoiesis, mutated menatopoietic stem cell

Chemo/Rad Tx
Benzene meds

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61
Q

PTs w/ MDS are at risk of developing ? type of leukemia

How is this Dx fore shadowed?

A

AML

Pre-leukemia syndrome, precedes Dx by 10yrs

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62
Q

How do PTs w/ MDS present in clinic?

What lab report is Dx

A

Fatigue out of proportion to anemia

Dec Hct
Inc B12 and RDW
All other values Norm

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63
Q

What is the difference in lab results between Non-megalo anemia and MDS

What is the best recognized morphologic abnormality of erythrocytes in MDS

What lab tests are key to the Dx of MDS

What are 3 Dzs that can present mimicking MDS but must be r/o

A

MDS- inc RDW

Oval macrocytosis

Peripheral smear
Unilateral marrow biopsy

HIV
B12/Folatedeficient

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64
Q

How is MDS Tx

A 
Support/Sxs
Transfusion Hgb <7
Platelet transfusion if 
<50K before surgery
<20K and bleeding
<10K and ASx
Chemo/Marrow transplant
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65
Q

Can you use MCV for Dx B12 deficiency?

What are the 4 causes of normocytic anemia

A

No, lacks sensitivity/specificity for cobalamin deficiency

Chronic Dz
Aplastic/Hemolytic anemia
Acute blood loss

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66
Q

What are the 3 causes of hemolytic anemia

What is the 2nd MC anemia

A

Hereditary Spherocytosis
G6PD
Sickle Cell

Normocytic Anemia, ACD

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67
Q

What is the pathophysiological reason behind normocytic anemia, ACD?

A

Inflammation causes:
Disturbed homeostasis- dec Fe and RBC

Macrophages release IL-6, stims hepatocytes, release hepcidin= dec Fe absoprtion/release from macrophages
Inflammation dec avail Fe for erythropioesis

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68
Q

When marrow erythropoiesis fails, what organ picks up 25% of the extramedullary work load?

How is Normocytic ACD Tx

A

Liver

Fe supplements (unless no deficiency)
Transfusions
ESA w/ anemia Sxs AND Hgb <10g:
CA on chemo
CKD GFR <30
HIV Tx w/ myelosuppression
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69
Q

What is the goal of Tx for Normocytic ACD

What lab result is not used for routine measurement

A

Hgb 11 and Hct >33%

Serum EPO

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70
Q

Define Aplastic Anemia

What are the two types of Aplastic?

A

Hypoproliferative anemia due to marrow failure, leading to pancytopenia

Primary: IgG against marrow
Secondary: acquired

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71
Q

What is the #1 cause of aplastic anemia

What metals can cause it?

What toxins can cause it?

What viruses can cause it?

A

Idiopathic

Gold Arsenic

Glue Benzene Solvents

Seronegative non A-G hepatitis
HIV
Other HSV
EBV

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72
Q

What is the typical bimodal presentation of Aplastic Anemia

What are the Sxs

What are the Signs

A

15-25 and >60y/o

Thrombocytopenia
Anemia
Neutropenia

Pallor Purpura Petechiae

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73
Q

What PE findings are absent in aplastic anemias?

What lab result is the hallmark of aplastic?

How is a Dx confirmed?

A

Hepatosplenomegaly
Lymphadenopathy
Bone tenderness

Inc fat, dec stem cells= Pancyotpenia

Hypocellular bone marrow

74
Q

What does the term ‘non-clonal’ mean?

What are two non-clonal d/os causing aplastic anemia?

A

Not bone marrow

Hypersplenism
Lupus

75
Q

How is aplastic Tx

What are the intrinsic etiology of hemolytic anemia

A

Young PT w/ functional status= marrow transplant
Older PT- ImmSupp to let remaining marrow recover

Defected RBC, hereditary
Membrane defect- spherocyt.
Oxidaiton- G6PD
Hgbopathies- Sickle, Thalassemia

76
Q

What are the extrinsic factors causing hemolytic anemia

A

External factors
Immune: AutoImm, drugs

Microangiopathic- TTP HUS Cardiac valve hemolysis

Infection- Borrelia Malaria Clostridium

77
Q

What are the two types of schistocytes seen in hemolytic anemia

What will lab analysis show?

A

Bite cell
Helmet cell

Dec haptoglobin
Inc: 
Indirect bili 
Hemoglobinuria
LDH
RTC
78
Q

What is the MC intrinsic hemolytic anemia

What does this issue become evident

A

Hereditary spherocytosis- abnormal spectrin/actin (RBC membrane structure)

Less deformable and can’t pass through spleen= rupture and trapped

79
Q

What type of hereditary passage does hereditary spherocytosis have?

What lab results are indicative of a Dx

A

Auto Dom- abnormal gene from one parent= Dz

Inc MCHC
Neg Coombs test= not AutoImm
Inc osmotic fragility test

80
Q

How is hereditary spherocytosis Tx

What is the MC enzymatic d/o of RBCs

A

Support/Transfuse Hgb <7
Folic supplements
Splenectomy- rarely needed

G6PD- RBCs hemolysis when under oxidative stress

81
Q

What type of hereditary passage does Sickle have?

What PT population does it affect most?

A

X linked recessive

SE Asia
Mediterranean- severe, chronic hemolytic anemia
AfAM

82
Q

Define Homo/Hetero/Hemizygous

When radicals are formed during oxidative stress they attack ?

A

Homo: two identical alleles
Hetero: two different alleles
Hemi: only one allele

DNA Lipids Proteins

83
Q

Define Favism

What type of cell is Dx of G6PD

A

All PTs w/ favism= G6PD
Not all G6PD= favism

Heinz bodies- seen w/ crystal violet stains
Causes bite cells during spleen passage

84
Q

What 2 blood d/os have bite cells?

What will lab analysis of G6PD show

A

Normo, hemolytic anemia
Normo, G6PD

Dec: haptoglobin G6PD enzyme
Inc: Indirect bili LDH RTC

85
Q

When are splenectomys recommended for G6PD PTs

What genetic d/o causes sickle cell?

A

Severe episodic/chronic hemolysis

Auto Recessive- point mutation on 6th AA of B-globin gene
HbA, HbS= trait
HbS, HbS= Dz

86
Q

How/why is Sickle Cell Dx so early

The sickling of RBCs is sensitive to ?

A

Hgb F drops off= Hgb S

Intracorpuscular concentration of deoxygenated hgb

87
Q

When is sickling of RBCs increased?

What are PTs w/ Sickle Trait at risk for?

A

Dehydration
Changes to oxygen dissociation curve: hypoxia acidosis altitude

Sudden cardiac death
Rhabdo

88
Q

What is a visible to the eye Sx of a chronic sickle cell anemia PT

What are the MC sites for sickle PTs to have vaso occlusive crisis?

A

Ulceration on lower anterior tibia

Spine
Kidneys
Long bones
Lungs

89
Q

Define a sickle Acute Chest Syndrome

If Sickle PT has osteomyelitis, ? is the microbe

A

Angina
Hypoxemia
Pulm infiltrates on CXR

Salmonellae

90
Q

What will be seen on lab analysis of Sickle?

What is the hallmark of hyosplenism in sickle cell

A

Inc WBC, indirect bili
Dec Hct

Howell Jolly bodies
Target cells

91
Q

How are sickle cell PTs managed for Tx

When do these PTs need transfusions?

A 
FOAHL
Folic acid- 1mg PO per day
Omega 3- reduce occlusive
ACEI if + microalbuminuria
Hydroxyurea and L-glutamine for pain crisis

Stroke
Occlusive/pain crisis
Acute chest syndrome
Priapism

92
Q

When are Sickle PTs admitted

How can this Dz be definitively Tx

A

Acute chest syndrome
Aplastic crisis
Unresponsive Pain crisis

Stem cell transplant before end organ damage

93
Q

How/why does acute blood loss lead to anemia

A

Baroreceptors release vasopressin
Shifts fluids extra to intra cellular= hemodilution
Hypovolemia changes to anemia
Marrow response takes 7 days to respond w/ inc RTC

94
Q

Define Massive Transfusion

Doing these procedures can cause ? metabolic shifts/issues

A

> 50% of PTs volume in 24hrs
10 PBRC units in 24hrs

Metabolic acidosis
Hypo Ca/thermia
HyperK
Coagulopathy
Dilutional thrombocytopenia
95
Q

What is the MC transfusion reaction

How can the chances of this reaction be reduced

How is it Tx

A

Leukoagglutinin- reaction to Abs on WBCs from PRBCs; febrile non-hemolytic reaction
F/c w/in 12hrs of receipt

Leukopore filters- filters donor WBCs out prior to storage

Diphenhydramine
Acetaminophen
CCS

96
Q

What causes acute hemolytic transfusion reactions

What is the MC cause of this reaction

When do the most severe reactions occur

A

Mismatched ABO/Rh blood= massive intravascular hemolysis

Clinical error

Surgery under general anesthesia

97
Q

What are the classic S/Sxs of AHTR

PTs under anesthesia can’t/wont present with classic signs of AHTR but will present w/ ?

A

Fever/rigor HOTN HA/back pain

Oliguria Bleeding

98
Q

How long for DHTR to present w/ ? unique Ab is seen in this type of reaction

What is the name of the reaction

A

5-10 days
Alloantibody- produced against foreign tissues

Anamnestic responses- enhanced immune response

99
Q

Blood product contamination is usually by ? type of microbe infecting ? blood cell

How would PTs present if the not MC microbes infected their product

What is the most and least likely transfusion associated infection?

A

Gram neg- platelets not refrigerated

Gram Pos=
Fever, Bacteremia
Rarely sepsis

Most: Hep B
Least: HTLV

100
Q

How does kidney damage occur from blood product contamination?

Fresh Whole Blood

A

Gram neg microbes causes transfusion endotoxins

RBCs Plasma WBCs and Platelets
Kept for <24hrs
Use: cardiac surgery/massive hemorrhage, +10 units in 24hrs
Exact blood match- MUST

101
Q

PRBC

Define CMV negative, leukocyte reduced filtered RBC

A

MC transfused product
1 unit inc Hgb x 1g and Hct x 4%
1 unit= 300ml vol inc

Dec leukocyte blood to prevent transmission of CMV, causes post-transplant I/G/H Dz

102
Q

When are CMV neg, WBC reduced RBCs used

How long can frozen RBCs be stored?

A

Hx febrile non-hemolytic transfusion reactions
Cardiovascular surgery
Transplant candidates
Chronically transfused PTs

10yrs- expensive, reserved for rare blood types

103
Q

Define Irradiated RBCs

What part of blood contains coagulation factors

A

Completely removes WBCs
Used for ImmComp PTs

Plasma

104
Q

When is FFP used

1 unit of apheresis platelets= + units of whole blood platelets

A

PTs w/ active bleeding or at high risk for bleeds

6 units
1 unit= inc platelets by 5-10K w/in 1hr

105
Q

Define Platelet refractoriness

A

Platelets don’t inc w/in 1hrs after transfusion
Non-Imm MC cause: sepsis
Imm: Abs against HLA Ags are primary cause

106
Q

What is the use of Freeze Dried Plasma

Who is universal donor and recipient

A

Only avail for SF, sterile water reconstitute

D: O-
R: AB+

107
Q

Erythroblastosis Fetalis

How is it prevented

A

Immune Hydrops or Rh Dz of Pregnancy
Mother Imm system destroys Rh+ cells in baby

ImmGlob 300mcg @ 28wks
W/in 72hrs of terminated pregnancy
Post-any vaginal bleed
Amniocentesis or chorionic villi sample
Avoid manual removal of placenta
108
Q

What are the 3 steps of forming the initial platelet plug

This process requires ? two phases to work togeth

A

Adherence
Activation
Aggregation

Vascular
Platelet

109
Q

Define Secondary Hemostasis

What are the 3 phases

A

Cascade of activating clotting factors to form fibrin clot

Vascular
Platelet
Coagulation cascade phase

110
Q

What happens during vascular phase of hemostasis

What happens during platelet phase of hemostasis

What happens during coagulation phase?

A

Spasms of smooth muscle, lasts 30min

Endothelial cells release vWFs, results in platelet adhesion

Enzyme thrombin activates fibrinogen to form fibrin= clot

111
Q

Define vWF

This cell carries ? clotting factor

A

Blood glycoprotein used as bridge for platelet aggregation

Factor 8

112
Q

Steps of platelet adhesion

A

Adhere to endothelial cells

Activated platelets release ADP and thromboxane

ADP binds to platelet receptor, activates Gp2b/3a to accept fibrinogen

Platelet aggregation= plug

113
Q

? binds fibrinogen and is considered the final common location for platelet to platelet aggregation

What activates the intrinsic pathway

This in turn activates ?

A

Gp1b/3a

Trauma exposing blood to subendothelail collagen

Extravascular/tissue trauma phase

114
Q

What is the only clotting factor used in all of the pathways?

There is no Factor _

A

Factor 4: Ca ions, accelerate clotting pathway

6- prothrombinase, combo of 5 and 10 factors

115
Q

Coagulation Cascade

A 
Xa + Va= prothrombinase
2 to Thrombin
Thrombin to 1
Fibrin + stabilizer
Cross linked fibrin- Factor 8
Clot
116
Q

What do blood banks add to Ca2 during clot formation

Thrombin is needed to activate ? factors

A

Citric acid

8 11 5 13

117
Q

Fibrin stabilizer is factor ?

What is it’s function

A

13

Combos w/ thrombin and Ca= Factor 13a- caused cross link fibrin

118
Q

What clotting factors are vital to the common pathway?

Factors _ + _= Activated 10,a

A

1 2 4 5 10 13

8a + 9a
Thrombokinase

119
Q

Where is the intrinsic pathway found?

What is the most important part of the extrinsic pathway?

A

In vasculature system

Tissue factor
TF + 7a= 10a

120
Q

What lab test is used to identify issues in the intrinsic, extrinsic pathway

What are the Vit K dependent factors

A

In: PTT
Ex: PT

2 7 9 10
Protein C, S

121
Q

Vit K deficiency= ?

Coumadin is a ?

A

Prolonged PT, extrinsic

Vit K antagonist
Inhibs 2 7 9 10 Protein C, S

122
Q

Why is coumadin bridged w/ heparin?

Factor 1

A

Coumadin causes transient hypercoagulable state during Protein C decreases faster than coagulation factors

Fibrinogen- activated to form fibrin

123
Q

Define DIC

What carries Factor 8

A

Depleted fibrinogen and fibrin (1A) levels

vWF

124
Q

A deficiency of vWF can lead to ?

What factors are involved w/ Hem A, B and C

A

Defected platelets and coagulation= Von Willebrand Dz

Hem A: Factor 8
Hem B: Factor 9
Hem C: Factor 11

125
Q

Platelet problems usually present as ?

What is LESS severe, platelet of coagulation d/o

A

Mucous membrane bleeds

Platelet

126
Q

Platelet count below ?K puts PTs at risk for spontaneous massive hemorrhages

Coagulation d/os are usually associated w/

A

<10K

Large vessel bleeds-
Hemarthrosis
Hemotoma
Ecchymosis
Excessive bleeds
127
Q

What is the hallmark red flag to bleeding

Define Menometrorrhagia

A

Hemarthrosis causing pain

Irregular prolonged and heavy bleeds

128
Q

What are 4 types of life threatening hematomas

How are issues w/ intrinsic pathway tested for?

A

Air way compression
Compartment syndrome
Retroperitoneal
CNS bleeds

PTT
Stims of Factor 12
Detects issues of 1 2 5 8 9 10 11 12 and Fibrinogen

129
Q

Normal PTT= ?

PTT >100sec = ?

Normal aPTT is ?

A

60-70sec

Spontaneous bleeds

25-40sec

130
Q

How is the extrinsic pathway tested?

What does the INR test

A

Thromboplastin to plasma, wait for fibrin formation
Detects issues of 2 5 7 12 and fibrinogen
Norm= 10-14sec

Extrinsic path based on PT
0.8-1.2

131
Q

What is the target INR for PTs w/ DVTs, PEs, AFib or valve HDz?

What is the target range for PTs w/ mechanical valves or recurrent MIs?

A

2-3

2.5-3.5

132
Q

Hemophilia ? is only in males due to it being passed by ?

Hemophilia ? is in either gender equally

A

A and B, X-linked

C

133
Q

How do cases of hemophilia present

What will lab results show

What are the 3 classifications

A

hemarthrosis/Arthropathy
OOP bleeding during surgery

aPTT prolonged

Factor activity of-
Severe: <1%
Mod: 1-5%
Mild: >5%

134
Q

Severe hemophilia classification

A 
2-4 times/mon
Factor activity <1%
Spontaneous hemorrhage from infancy
Frequent hemarthrosis/muscle bleeds
Dx by 2y/o
135
Q

Moderate hemophilia classification

A 
4-6x/year
Factor activity 1-5%
Hemorrhage due to trauma/surgery
Occacsional spot hemarthrosis
Dx by 5-6y/o
136
Q

Mild hemophilia classification

A 
Uncommon bleeds
Factor activity >5%
Bleeds due to trauma/surgery
Occasion spot hemorrhage
Dx later in life
137
Q

How is hemophilia Tx

What Tx method is avoided in PTs w/ factor concentrations starting in childhood

A

Replace clotting factors
DDAVP for mild Hem A

Hemophilic arthropathy

138
Q

What are the 3 types of VWDz

A

1: partial quant of vWF
2: qual of vWF
A absence
B binds w/ GP1b
M dec platelet dependent function (weak bonds)
N doesn’t carry Factor 8
3: complete quant deficient vWF

139
Q

How is VWDz Tx

Circulating antigoagulants usually neutralize ? clotting factors

When is the Dx considered

A

DDAVP for Type 1, 2a, 2b
Recombinant vWF for Type 2 and 3
No FFP (no/low vWF)

2 5 8 9

PTs w/ inc PTT or PT that doesn’t correct w/ repeated mixing test

140
Q

Pts w/ circulating anticoagulatns and Hemophilia are Tx w/ ?

If PT doesn’t have hemophilia they’re Tx w/ ?

A

Recombinant Factor 7

Cycophosphamide
CCS
Rituximab

141
Q

During coagulopathy of Liver Dz Tx, all factors are dec except?

If this factor is dec, ? dx is considered

A

8

DIC

142
Q

As the severity of a liver Dz inc, what lab value also inc?

What is the primary lesion in DIC

A

PTT
Mild: Inc PT, N PTT
Severe: Inc PT and PTT

Excess tissue factor causes overwhelming formation of thrombin/fibrin

143
Q

What are the two phases of DIC

What types of cells are seen in DIC?

A

Ischemia to Hemorrhagic

Schistocytes

144
Q

What is the natural blood thinner of the body that will be dec in disseminated DIC

A DIC score of _ or more is indicative of higher mortality

A

Anti thrombin 3

5 or higher

145
Q

How is Disseminated DIC Tx

When is anticoagulation therapy recommended?

A

If precipitated by sepsis- Activated Protein C (regulated activity of thrombin)
Hemostatic therapy- platelets and FPP

Arterial thromboemboli w/ mural thrombus
Migratory thrombophlebitis w/ Trousseau syndrome ( 2nd leading cause of death in CA PTs)

146
Q

What is the sequence of platelet development

What are the granules inside of platelets

A 
Myeloid
Progenitor
Megakaryoblasts
Megakaryocytes
Platelets in plasma membrane

ADP, Thromboxane A2

147
Q

If attempting to r/o pseudothrombocytopenia, what color tube is used for the draw?

This pseudo condition is AKA ?

Platelet d/os of ? etiology are more likely to be overlooked

A

EDTA tube- sodium citrate (light blue)

Platelet clumping

Quant:
VWDz
Acquired d/o
Congenital

148
Q

What is the MC cause of immune thrombocytopenia

What are secondary causes of Immune Thrombocytopenia

What is the time difference between chronic and acute

A

ITP

AutoImm
HIV/HCV
Drugs

Acute <6mon (kids, 2-10yr)
Chronic >6mon (adults w/ HIV AutoImm APS or Hep B/C

149
Q

How does ITP present

What PE finding is considered uncommon

A

Mucosal bleeds

Splenomegaly

150
Q

What is the sequence of bleeding in ITP as platelet numbers fall?

What does the absence of cutaneous petechia mean in this condition?

A

Skin- petichae/purpura
Mucous membranes
Viscera

Low chance of inracranial hemorrhage and there for, less severe case of ITP

151
Q

What criteria are used for drug induced ITP Dx

How is it Dx

What are the 4 scenarios to do this Dx step

A

Severe thrombocytopenia
Mucotaneous bleeds x 7-14 day
after start of new drugs

Marrow biopsy

> 50y/o to exclude MDS
Unexplained cytopenia
Therapy failure
Prior to splenectomy fo Dx confirmation

152
Q

What is the mainstay of ITP Tx

How is relapsed/persistent ITP Tx

How is relapsed/worsening ITP Tx

A

REduce bleeds w/
CCS +/- IVIG or anti-D
If severe hemorrhage present- platelet transfusion

Monoclonals Rituximab or
Anti-D or
IVIG or
TPO

Splenectomy

153
Q

When is serial platelet counts used for monitoring ITP Tx

PTs w/ acquired HIT are at risk for ?

A

ASx and Platelets above 30K

Art and Ven thrombosis for wk/mon after heparin d/c

154
Q

How does HIT develop

How is a Dx confirmed

When is Tx started

A

IgG against Platelet Factor 4

PF4 Ab enzyme linked ELISA
+= confirm w/ SRA

Immediately when Dx is SUSPECTED

155
Q

When is a HIT Dx suspected

A

5-14d post heparin exposure
50% decline from baseline
Necrotic skin at site
Thrombosis

4Ts w/ 8pts max:
Low: 3 or less
Int: 4-5
High: 6 or more

156
Q

How is this condition scored?

A

2pts for
Platelets dec x 50% and 20K or higher

Clear onset between days 5 and 10 or,
Platelets fall under one day

New thrombosis/necrosis or systemic reaction

157
Q

How is HIT Tx

A

Direct thrombin inhib- Argatroban Bivalirudin Fondaprinux until +100K
Warfarin c/i until platelets at 100K
INR goal 2-3
Duplex doppler LE to r/o DVT

158
Q

How long is anticoagulation Tx post-HIT maintained

What refereal is placed for these PTs

A

Thrombosis= 3-6mon after platelet recovery
No thrombosis= 30 days after platelet recovery

ALL PTs w/ HIT see a hematologist

159
Q

Define TMA

This condition includes ?

What findings are indicative of TMA Dx

A

Vascular injury leading to thrombi in microvasculature

TTP HUS

MAHA- schistocytes
Thrombocytopenia

160
Q

Define TTP in a TMA case

What is the pentad of TMA

What part is most associated w/ TTP

A

AutoAbs against vWF cleave protease ADAMTS-13

Fever Anemia Thrombocytopenia Renal Dz *Neuro complications

161
Q

Renal Dz in TMA is most associated w/ ?

How is this condition defined

A

HUS

162
Q

TMA is a main cause of CKI in ? PTs

These PTs all present w/ ? Triad

A

Kids

Petechia HTN RF

163
Q

What typically precedes TMA HUS

? stool culture results may be seen here?

A

GE w/ E Coli O157 STEC

+ Ecoli 157
Shiga toxin

164
Q

What is the difference in lab results between acquired TTP and inherited

What is needed for a Dx of complement mediated HUS

A

Ac: dec ADAMTS12
In: absent

Mutated genes that encode complement

165
Q

How is TMA Tx

What is the exception

A

Immediate plasma exchange as soon as Dx is made
Platelet transfusion c/i
2nd line: if refractory; CCS Rituximab IVIG Cyclo Splenectomy
CKI= dialysis

Shiga/Ecoli origin= support Tx only

166
Q

? med can cause qual platelet d/os

Thalassemia Dx is only considered after ?

A

ASA

Exclusion of IDA

167
Q

PT w/ DM or GERD may develop ? type of anemia?

4 ABX that can induce oxidative stress

A

B12 deficient macro

Sulfonamide
Nitrofuranotoin
Quinolone
Dapsone

168
Q

Deterioration of ? Dz will worsen normocytic anemia

What miscellaneous things can cause aplastic anemia

A

CKDz

Paroxysmal nocturnal Hbguria
Preg Anorexia Thymoma

169
Q

3 times nucleated cells are seen?

Define Thrombus

Define Embolus

Define Embolism

A

WBCs
Cooleys, Sickle

Blood clot

Fragment of thrombus traveling

Lodged embolus

170
Q

Define Coach Class Syndrome

What are the ‘strong’ RFs for VTEs

A

Classic VTE Hx: travel surgery/trauma CaHx

Spinal cord injury
Hip/leg Fx
Hip/knee replacement
GenSurg
Major trauma
171
Q

? lab result has strong evidence against a VTE

What is the initial image of choice for VTEs

What test is used if CT is unavail

A

D-dimer <500ng via Rapid Quant ELISA

CT-PA

VQ scan

172
Q

What is the ToC for detecting proximal DVTs

What is an invasive Dx test that can be done

A

VUS

Pulm Angiography

173
Q

How are VTEs Tx

What is the safest anti-coag Tx for PTs w/ GFR <30

How is LMWH or Heparin reversed

A

LMWH- Enoxaparin
UFH

Warfarin

Protamine Sulfate

174
Q

Coumadin is c/i in ? PTs

Heparin affects ? factors

Warfarin affects ? factors

Dabigatran affects ? factors

Factoa Xa inhibitors -ban affect ? factors

A

Pregnant

2a 7a 9a 10a 11a

2 7 9 10

2a

10a

175
Q

Why are Factor Xa inhibitors used for VTE Tx

What are the risks

A

No Lovenox bridge needed
ARE-ban
No monitoring or daily injection

Irreversible binding
Can’t monitor PT compliance

176
Q

How long are VTE PTs on anticoag therapy

How long are PTs that have idiopathic VTE or have low/mod risks of bleeds on therapy

What regime are Ca PTs placed on

A

1st one: 3mon

No stop date
D-dimer repeat in 1mon

Extended w/ LMWH

177
Q

What PE measurement indicates DVT

What are the MC S/Sx of PE

A

Calf diameter >3 measured at 10cm below anterior tibial tuberosity

Sinus Tachy
Non-specific ST/T changes
S1Q3T3

178
Q

Well’s Score

A

S/Sx
PE likely

Tachy
Imm/Surg
Prior DVT

Hemptysis
CA current/Tx 6mon
>6 high <2 low

179
Q

When is a D-dimer taken w/ a Wells score

A

<4
Dimer<500= neg
>4
CTPA, no D-Dimer

180
Q

CHA2DS2VASc score

A

Annual risk for TIA/CVA
2 or more= anticoag

CHF HTN Age >75 DM Stroke TIE TE Vascular Dz Age 65-74 Sex, female
Max 9
<74, max 8

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