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
What Hgb is the last one to start developing in utero but lasts into adulthood? How are thalassemia's definitively Dx?
D Electrophoresis
26
What type of Hx will PTs w/ thalassemia have? What type abnormal RBC morphologies may be seen on smears?
FamHx Hx of microcytic anemia Microcytes Acanthocytes- Greek, thorn Codocytes- target cells
27
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
AA/AA Normal AA/A- Trait AA/-- Homozygous A-/A- Heterozygous
28
What would Chromosome 16 look like in a PT w/ A-thalassemia Intermedia What form of A-Thalassemia mainly affects Asians or Blacks?
Hgb H: A-/-- Heterozygous A-/A- Asian Homozygous AA/-- Black
29
What is the life expectancy for PTs w/ A-thalassemia minor trait How are PTs w/ Hgb H Dz managed
Normal Daily folate, avoid iron Transfusion during hemolytic exacerbations
30
What drug classes do PTs w/ Hgb H Dz need to avoid Since B-thalassemias have impaired B production, what substitutions can occur?
Anti malaria bacteria Sulfa Analgesis TB Excess A chains can pair w/ D chains (HbA2) or G chains (HbF)
31
What happens to the A-globins in cases of B-thalassemia What PTs have more severe cases of B-thalassemias?
Unstable and damage RBC membranes Homozygous impaired B-globin synthesis
32
What two types of B-Thalassemia have basophilic stippling What form of B-Thalassemia has nucleated RBCs?
B minor/trait B intermedia Major- Cooley's Anemia
33
What form of thalassemia appears abnormal on electrophoresis Only ? thalassemia has heterozygous genes?
B, due to G/D globin chains G= Hgb F D= Hgb A2 B-Thalassemia minor
34
B thalassemia intermedia globin combos B thalassemia major globin combos
B+/B+ Bo/Bo or, B+/B+
35
? Thalassemia is transfusion dependent and is a majority of HgF How is A-Thalassemia trait or mild B-thalassemia Tx
B-Thalassemia Majors None, identify to avoid future eval/Tx for Fe deficiency Hgb H- Folic acid supplements and avoid Fe/Oxidative drugs
36
How is B-Thalassemia intermedia/major Tx What can develop if these PTs are over Tx?
Transfusions Folate Splenectomy Marrow Transplant- TxOC for Major Too many transfusions= iatrogenic Fe overload syndrome
37
What lab result is Dx of Sideroblastic anemia What causes this d/o
Bone marrow Fe >10% ringed sideroblasts Serum Fe is avail but isn't utilized by mitochondria= RBCs w/ iron granule in mitochondria
38
What lab method is used to visualize ringed sideroblasts Why are stippled basophils also seen in this lab test?
Prussian blue (Perl's reaction) shows ring of blue granules Indicative of disrupted erythropioesis
39
How is sideroblastic anemia Tx How does lead poisoning cause anemia
``` Support Hgb <7= PRBC transfusion PO Pyridoxine (B6) ``` Impairs heme synthesis Inc RBC destruction
40
What lab result is used to differentiate lead poisoning as a recent or chronic exposure How is lead poisoning anemia Tx
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)
41
What are the 4 conditions that present w/ basophilic stippling? What do the lead panel results look like for results w/ stippling in them?
B minor/intermediate Lead poisoning Sideroblast Inc TIBC Fe Low Ferritin
42
What are the two types of Macrocytic anemias due to?
Mega: B12/Folate deficient, Drugs ``` Non-mega: AHEAD MS Alcohol Hypothyroid Emphysema Accelerated erythopoiesis Drug induced Marrow d/o Surface area, inc on RBC ```
43
To initiate hematopoiesis, EPO stimulates ? cell Megaloblastic anemia has their etiology starting w/ ? cell
Proerythroblasts Erythroblastic stage
44
If hypersegmented neutrophils are seen on a peripheral smear, this indicates? What is the criteria for hypersegmented neutrophil?
Megaloblasts in marrow Neutrophil nuclei w/ +6 lobes or, +3% neutrophils w/ five nuclear lobes
45
How is it brought into the GI system for use
HCl releases pepsin, seperates B12 from foods Parietal cells release IF, absorbs B12 in terminal ileum TC2 from WBCs carries B12
46
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 ?
2-5000mcg >3yrs after absorption ceases Used for converting m-CoA into s-CoA in mitochondria Methylmalonic acid
47
What is the most sensitive test for B12 deficiency at the cellular level The lack of B12 for DNA synthesis causes ? to be formed
MMA test Hypersegmented neutrophils
48
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?
Strict vegan no dairy, meat, fish Only breast fed baby of vegan mother NO- present w/ spinal degeneration Imerslund Grasbeck Syndrome
49
What are the biggest 3 Sxs that present w/ B12 anemia in order of presentation Why does B12 deficiency cause neuro Sxs?
Peripheral neuropathy: paresthesia of hands Ataxia Dementia Involved w/ myelin production
50
What is the '5 P's of B12 Deficiency" mnemonic?
``` Pancytopenia Peripheral neuropathy Papillary atrophy (atrophic glossitis) Posterior column neuropathy Pyramidal tract signs ```
51
What is the only lab result that differs between B12 deficiency anemia and pernicious anemia? How is B12 deficiency anemia Tx
IFBA/Serum Gastrin tests Genetic cause: lifetime Tx Reversible: until corrected and Sxs are gone Vit B12 but NOT nasal route
52
Pts w/ B12 Deficiency anemia but no Sxs are Tx w/ ? What PT population may need more aggressive repltion and how is that done?
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 ```
53
How is Pernicious Anemia Tx How are PTs w/ Lichtheims Dz that have B12 and folate deficiency Tx
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
54
What is the sequence of response to Tx for B12 deficiency Where is folate acid normally found, absorbed and how can it be destroyed?
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
55
Body normally stores about ? folate which is a ? supply What meds were highlighted as causing folate deficient anemia
5000mcg 2-3mon Anti convulsants ABX- sulfas HIV meds
56
How is folate deficient anemia Tx
PO 1000mcg PO per day x 1-4mon or until complete hematological recovery
57
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
Combo: Dec B12, Inc MMA Oval macrocytes Inc RDW
58
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?
No Aplastic CLDz ETOH Unexplained megalo or, Myelodysplasia suspected Megaloblastoid RBC precursors (proerythroblasts) w/ nuclear chromatin
59
? anemia has inc RBC surface area ? meds can cause this type of anemia w/ inc surface area
Non megalo macro- inc deposition for cholesterol/lipids Benzenes
60
# Define MDS What are 3 causes
Marrow d/o of ineffective erythropoiesis, mutated menatopoietic stem cell Chemo/Rad Tx Benzene meds
61
PTs w/ MDS are at risk of developing ? type of leukemia How is this Dx fore shadowed?
AML Pre-leukemia syndrome, precedes Dx by 10yrs
62
How do PTs w/ MDS present in clinic? What lab report is Dx
Fatigue out of proportion to anemia Dec Hct Inc B12 and RDW All other values Norm
63
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
MDS- inc RDW Oval macrocytosis Peripheral smear Unilateral marrow biopsy HIV B12/Folatedeficient
64
How is MDS Tx
``` Support/Sxs Transfusion Hgb <7 Platelet transfusion if <50K before surgery <20K and bleeding <10K and ASx Chemo/Marrow transplant ```
65
Can you use MCV for Dx B12 deficiency? What are the 4 causes of normocytic anemia
No, lacks sensitivity/specificity for cobalamin deficiency Chronic Dz Aplastic/Hemolytic anemia Acute blood loss
66
What are the 3 causes of hemolytic anemia What is the 2nd MC anemia
Hereditary Spherocytosis G6PD Sickle Cell Normocytic Anemia, ACD
67
What is the pathophysiological reason behind normocytic anemia, ACD?
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
68
When marrow erythropoiesis fails, what organ picks up 25% of the extramedullary work load? How is Normocytic ACD Tx
Liver ``` Fe supplements (unless no deficiency) Transfusions ESA w/ anemia Sxs AND Hgb <10g: CA on chemo CKD GFR <30 HIV Tx w/ myelosuppression ```
69
What is the goal of Tx for Normocytic ACD What lab result is not used for routine measurement
Hgb 11 and Hct >33% Serum EPO
70
# Define Aplastic Anemia What are the two types of Aplastic?
Hypoproliferative anemia due to marrow failure, leading to pancytopenia Primary: IgG against marrow Secondary: acquired
71
What is the #1 cause of aplastic anemia What metals can cause it? What toxins can cause it? What viruses can cause it?
Idiopathic Gold Arsenic Glue Benzene Solvents Seronegative non A-G hepatitis HIV Other HSV EBV
72
What is the typical bimodal presentation of Aplastic Anemia What are the Sxs What are the Signs
15-25 and >60y/o Thrombocytopenia Anemia Neutropenia Pallor Purpura Petechiae
73
What PE findings are absent in aplastic anemias? What lab result is the hallmark of aplastic? How is a Dx confirmed?
Hepatosplenomegaly Lymphadenopathy Bone tenderness Inc fat, dec stem cells= Pancyotpenia Hypocellular bone marrow
74
What does the term 'non-clonal' mean? What are two non-clonal d/os causing aplastic anemia?
Not bone marrow Hypersplenism Lupus
75
How is aplastic Tx What are the intrinsic etiology of hemolytic anemia
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
What are the extrinsic factors causing hemolytic anemia
External factors Immune: AutoImm, drugs Microangiopathic- TTP HUS Cardiac valve hemolysis Infection- Borrelia Malaria Clostridium
77
What are the two types of schistocytes seen in hemolytic anemia What will lab analysis show?
Bite cell Helmet cell ``` Dec haptoglobin Inc: Indirect bili Hemoglobinuria LDH RTC ```
78
What is the MC intrinsic hemolytic anemia What does this issue become evident
Hereditary spherocytosis- abnormal spectrin/actin (RBC membrane structure) Less deformable and can't pass through spleen= rupture and trapped
79
What type of hereditary passage does hereditary spherocytosis have? What lab results are indicative of a Dx
Auto Dom- abnormal gene from one parent= Dz Inc MCHC Neg Coombs test= not AutoImm Inc osmotic fragility test
80
How is hereditary spherocytosis Tx What is the MC enzymatic d/o of RBCs
Support/Transfuse Hgb <7 Folic supplements Splenectomy- rarely needed G6PD- RBCs hemolysis when under oxidative stress
81
What type of hereditary passage does Sickle have? What PT population does it affect most?
X linked recessive SE Asia Mediterranean- severe, chronic hemolytic anemia AfAM
82
# Define Homo/Hetero/Hemizygous When radicals are formed during oxidative stress they attack ?
Homo: two identical alleles Hetero: two different alleles Hemi: only one allele DNA Lipids Proteins
83
# Define Favism What type of cell is Dx of G6PD
All PTs w/ favism= G6PD Not all G6PD= favism Heinz bodies- seen w/ crystal violet stains Causes bite cells during spleen passage
84
What 2 blood d/os have bite cells? What will lab analysis of G6PD show
Normo, hemolytic anemia Normo, G6PD Dec: haptoglobin G6PD enzyme Inc: Indirect bili LDH RTC
85
When are splenectomys recommended for G6PD PTs What genetic d/o causes sickle cell?
Severe episodic/chronic hemolysis Auto Recessive- point mutation on 6th AA of B-globin gene HbA, HbS= trait HbS, HbS= Dz
86
How/why is Sickle Cell Dx so early The sickling of RBCs is sensitive to ?
Hgb F drops off= Hgb S Intracorpuscular concentration of deoxygenated hgb
87
When is sickling of RBCs increased? What are PTs w/ Sickle Trait at risk for?
Dehydration Changes to oxygen dissociation curve: hypoxia acidosis altitude Sudden cardiac death Rhabdo
88
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?
Ulceration on lower anterior tibia Spine Kidneys Long bones Lungs
89
# Define a sickle Acute Chest Syndrome If Sickle PT has osteomyelitis, ? is the microbe
Angina Hypoxemia Pulm infiltrates on CXR Salmonellae
90
What will be seen on lab analysis of Sickle? What is the hallmark of hyosplenism in sickle cell
Inc WBC, indirect bili Dec Hct Howell Jolly bodies Target cells
91
How are sickle cell PTs managed for Tx When do these PTs need transfusions?
``` 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
When are Sickle PTs admitted How can this Dz be definitively Tx
Acute chest syndrome Aplastic crisis Unresponsive Pain crisis Stem cell transplant before end organ damage
93
How/why does acute blood loss lead to anemia
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
# Define Massive Transfusion Doing these procedures can cause ? metabolic shifts/issues
>50% of PTs volume in 24hrs >10 PBRC units in 24hrs ``` Metabolic acidosis Hypo Ca/thermia HyperK Coagulopathy Dilutional thrombocytopenia ```
95
What is the MC transfusion reaction How can the chances of this reaction be reduced How is it Tx
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
What causes acute hemolytic transfusion reactions What is the MC cause of this reaction When do the most severe reactions occur
Mismatched ABO/Rh blood= massive intravascular hemolysis Clinical error Surgery under general anesthesia
97
What are the classic S/Sxs of AHTR PTs under anesthesia can't/wont present with classic signs of AHTR but will present w/ ?
Fever/rigor HOTN HA/back pain Oliguria Bleeding
98
How long for DHTR to present w/ ? unique Ab is seen in this type of reaction What is the name of the reaction
5-10 days Alloantibody- produced against foreign tissues Anamnestic responses- enhanced immune response
99
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?
Gram neg- platelets not refrigerated Gram Pos= Fever, Bacteremia Rarely sepsis Most: Hep B Least: HTLV
100
How does kidney damage occur from blood product contamination? Fresh Whole Blood
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
PRBC Define CMV negative, leukocyte reduced filtered RBC
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
When are CMV neg, WBC reduced RBCs used How long can frozen RBCs be stored?
Hx febrile non-hemolytic transfusion reactions Cardiovascular surgery Transplant candidates Chronically transfused PTs 10yrs- expensive, reserved for rare blood types
103
# Define Irradiated RBCs What part of blood contains coagulation factors
Completely removes WBCs Used for ImmComp PTs Plasma
104
When is FFP used 1 unit of apheresis platelets= + units of whole blood platelets
PTs w/ active bleeding or at high risk for bleeds 6 units 1 unit= inc platelets by 5-10K w/in 1hr
105
Define Platelet refractoriness
Platelets don't inc w/in 1hrs after transfusion Non-Imm MC cause: sepsis Imm: Abs against HLA Ags are primary cause
106
What is the use of Freeze Dried Plasma Who is universal donor and recipient
Only avail for SF, sterile water reconstitute D: O- R: AB+
107
Erythroblastosis Fetalis How is it prevented
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
What are the 3 steps of forming the initial platelet plug This process requires ? two phases to work togeth
Adherence Activation Aggregation Vascular Platelet
109
# Define Secondary Hemostasis What are the 3 phases
Cascade of activating clotting factors to form fibrin clot Vascular Platelet Coagulation cascade phase
110
What happens during vascular phase of hemostasis What happens during platelet phase of hemostasis What happens during coagulation phase?
Spasms of smooth muscle, lasts 30min Endothelial cells release vWFs, results in platelet adhesion Enzyme thrombin activates fibrinogen to form fibrin= clot
111
# Define vWF This cell carries ? clotting factor
Blood glycoprotein used as bridge for platelet aggregation Factor 8
112
Steps of platelet adhesion
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
? binds fibrinogen and is considered the final common location for platelet to platelet aggregation What activates the intrinsic pathway This in turn activates ?
Gp1b/3a Trauma exposing blood to subendothelail collagen Extravascular/tissue trauma phase
114
What is the only clotting factor used in all of the pathways? There is no Factor _
Factor 4: Ca ions, accelerate clotting pathway 6- prothrombinase, combo of 5 and 10 factors
115
Coagulation Cascade
``` Xa + Va= prothrombinase 2 to Thrombin Thrombin to 1 Fibrin + stabilizer Cross linked fibrin- Factor 8 Clot ```
116
What do blood banks add to Ca2 during clot formation Thrombin is needed to activate ? factors
Citric acid 8 11 5 13
117
Fibrin stabilizer is factor ? What is it's function
13 Combos w/ thrombin and Ca= Factor 13a- caused cross link fibrin
118
What clotting factors are vital to the common pathway? Factors _ + _= Activated 10,a
1 2 4 5 10 13 8a + 9a Thrombokinase
119
Where is the intrinsic pathway found? What is the most important part of the extrinsic pathway?
In vasculature system Tissue factor TF + 7a= 10a
120
What lab test is used to identify issues in the intrinsic, extrinsic pathway What are the Vit K dependent factors
In: PTT Ex: PT 2 7 9 10 Protein C, S
121
Vit K deficiency= ? Coumadin is a ?
Prolonged PT, extrinsic Vit K antagonist Inhibs 2 7 9 10 Protein C, S
122
Why is coumadin bridged w/ heparin? Factor 1
Coumadin causes transient hypercoagulable state during Protein C decreases faster than coagulation factors Fibrinogen- activated to form fibrin
123
# Define DIC What carries Factor 8
Depleted fibrinogen and fibrin (1A) levels vWF
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A deficiency of vWF can lead to ? What factors are involved w/ Hem A, B and C
Defected platelets and coagulation= Von Willebrand Dz Hem A: Factor 8 Hem B: Factor 9 Hem C: Factor 11
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Platelet problems usually present as ? What is LESS severe, platelet of coagulation d/o
Mucous membrane bleeds Platelet
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Platelet count below ?K puts PTs at risk for spontaneous massive hemorrhages Coagulation d/os are usually associated w/
<10K ``` Large vessel bleeds- Hemarthrosis Hemotoma Ecchymosis Excessive bleeds ```
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What is the hallmark red flag to bleeding Define Menometrorrhagia
Hemarthrosis causing pain Irregular prolonged and heavy bleeds
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What are 4 types of life threatening hematomas How are issues w/ intrinsic pathway tested for?
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
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Normal PTT= ? PTT >100sec = ? Normal aPTT is ?
60-70sec Spontaneous bleeds 25-40sec
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How is the extrinsic pathway tested? What does the INR test
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
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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?
2-3 2.5-3.5
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Hemophilia ? is only in males due to it being passed by ? Hemophilia ? is in either gender equally
A and B, X-linked C
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How do cases of hemophilia present What will lab results show What are the 3 classifications
hemarthrosis/Arthropathy OOP bleeding during surgery aPTT prolonged Factor activity of- Severe: <1% Mod: 1-5% Mild: >5%
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Severe hemophilia classification
``` 2-4 times/mon Factor activity <1% Spontaneous hemorrhage from infancy Frequent hemarthrosis/muscle bleeds Dx by 2y/o ```
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Moderate hemophilia classification
``` 4-6x/year Factor activity 1-5% Hemorrhage due to trauma/surgery Occacsional spot hemarthrosis Dx by 5-6y/o ```
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Mild hemophilia classification
``` Uncommon bleeds Factor activity >5% Bleeds due to trauma/surgery Occasion spot hemorrhage Dx later in life ```
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How is hemophilia Tx What Tx method is avoided in PTs w/ factor concentrations starting in childhood
Replace clotting factors DDAVP for mild Hem A Hemophilic arthropathy
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What are the 3 types of VWDz
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
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How is VWDz Tx Circulating antigoagulants usually neutralize ? clotting factors When is the Dx considered
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
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Pts w/ circulating anticoagulatns and Hemophilia are Tx w/ ? If PT doesn't have hemophilia they're Tx w/ ?
Recombinant Factor 7 Cycophosphamide CCS Rituximab
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During coagulopathy of Liver Dz Tx, all factors are dec except? If this factor is dec, ? dx is considered
8 DIC
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As the severity of a liver Dz inc, what lab value also inc? What is the primary lesion in DIC
PTT Mild: Inc PT, N PTT Severe: Inc PT and PTT Excess tissue factor causes overwhelming formation of thrombin/fibrin
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What are the two phases of DIC What types of cells are seen in DIC?
Ischemia to Hemorrhagic Schistocytes
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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
Anti thrombin 3 5 or higher
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How is Disseminated DIC Tx When is anticoagulation therapy recommended?
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)
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What is the sequence of platelet development What are the granules inside of platelets
``` Myeloid Progenitor Megakaryoblasts Megakaryocytes Platelets in plasma membrane ``` ADP, Thromboxane A2
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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
EDTA tube- sodium citrate (light blue) Platelet clumping Quant: VWDz Acquired d/o Congenital
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What is the MC cause of immune thrombocytopenia What are secondary causes of Immune Thrombocytopenia What is the time difference between chronic and acute
ITP AutoImm HIV/HCV Drugs Acute <6mon (kids, 2-10yr) Chronic >6mon (adults w/ HIV AutoImm APS or Hep B/C
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How does ITP present What PE finding is considered uncommon
Mucosal bleeds Splenomegaly
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What is the sequence of bleeding in ITP as platelet numbers fall? What does the absence of cutaneous petechia mean in this condition?
Skin- petichae/purpura Mucous membranes Viscera Low chance of inracranial hemorrhage and there for, less severe case of ITP
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What criteria are used for drug induced ITP Dx How is it Dx What are the 4 scenarios to do this Dx step
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
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What is the mainstay of ITP Tx How is relapsed/persistent ITP Tx How is relapsed/worsening ITP Tx
REduce bleeds w/ CCS +/- IVIG or anti-D If severe hemorrhage present- platelet transfusion Monoclonals Rituximab or Anti-D or IVIG or TPO Splenectomy
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When is serial platelet counts used for monitoring ITP Tx PTs w/ acquired HIT are at risk for ?
ASx and Platelets above 30K Art and Ven thrombosis for wk/mon after heparin d/c
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How does HIT develop How is a Dx confirmed When is Tx started
IgG against Platelet Factor 4 PF4 Ab enzyme linked ELISA += confirm w/ SRA Immediately when Dx is SUSPECTED
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When is a HIT Dx suspected
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
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How is this condition scored?
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
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How is HIT Tx
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
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How long is anticoagulation Tx post-HIT maintained What refereal is placed for these PTs
Thrombosis= 3-6mon after platelet recovery No thrombosis= 30 days after platelet recovery ALL PTs w/ HIT see a hematologist
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# Define TMA This condition includes ? What findings are indicative of TMA Dx
Vascular injury leading to thrombi in microvasculature TTP HUS MAHA- schistocytes Thrombocytopenia
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# Define TTP in a TMA case What is the pentad of TMA What part is most associated w/ TTP
AutoAbs against vWF cleave protease ADAMTS-13 Fever Anemia Thrombocytopenia Renal Dz *Neuro complications
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Renal Dz in TMA is most associated w/ ? How is this condition defined
HUS
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TMA is a main cause of CKI in ? PTs These PTs all present w/ ? Triad
Kids Petechia HTN RF
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What typically precedes TMA HUS ? stool culture results may be seen here?
GE w/ E Coli O157 STEC + Ecoli 157 Shiga toxin
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What is the difference in lab results between acquired TTP and inherited What is needed for a Dx of complement mediated HUS
Ac: dec ADAMTS12 In: absent Mutated genes that encode complement
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How is TMA Tx What is the exception
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
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? med can cause qual platelet d/os Thalassemia Dx is only considered after ?
ASA Exclusion of IDA
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PT w/ DM or GERD may develop ? type of anemia? 4 ABX that can induce oxidative stress
B12 deficient macro Sulfonamide Nitrofuranotoin Quinolone Dapsone
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Deterioration of ? Dz will worsen normocytic anemia What miscellaneous things can cause aplastic anemia
CKDz Paroxysmal nocturnal Hbguria Preg Anorexia Thymoma
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3 times nucleated cells are seen? Define Thrombus Define Embolus Define Embolism
WBCs Cooleys, Sickle Blood clot Fragment of thrombus traveling Lodged embolus
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# Define Coach Class Syndrome What are the 'strong' RFs for VTEs
Classic VTE Hx: travel surgery/trauma CaHx ``` Spinal cord injury Hip/leg Fx Hip/knee replacement GenSurg Major trauma ```
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? 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
D-dimer <500ng via Rapid Quant ELISA CT-PA VQ scan
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What is the ToC for detecting proximal DVTs What is an invasive Dx test that can be done
VUS Pulm Angiography
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How are VTEs Tx What is the safest anti-coag Tx for PTs w/ GFR <30 How is LMWH or Heparin reversed
LMWH- Enoxaparin UFH Warfarin Protamine Sulfate
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Coumadin is c/i in ? PTs Heparin affects ? factors Warfarin affects ? factors Dabigatran affects ? factors Factoa Xa inhibitors -ban affect ? factors
Pregnant 2a 7a 9a 10a 11a 2 7 9 10 2a 10a
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Why are Factor Xa inhibitors used for VTE Tx What are the risks
No Lovenox bridge needed ARE-ban No monitoring or daily injection Irreversible binding Can't monitor PT compliance
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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
1st one: 3mon No stop date D-dimer repeat in 1mon Extended w/ LMWH
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What PE measurement indicates DVT What are the MC S/Sx of PE
Calf diameter >3 measured at 10cm below anterior tibial tuberosity Sinus Tachy Non-specific ST/T changes S1Q3T3
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Well's Score
S/Sx PE likely Tachy Imm/Surg Prior DVT Hemptysis CA current/Tx 6mon >6 high <2 low
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When is a D-dimer taken w/ a Wells score
<4 Dimer<500= neg >4 CTPA, no D-Dimer
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CHA2DS2VASc score
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