heme Flashcards

(86 cards)

1
Q

major functions of blood

A

transport (O2, CO2, nutrients, hormones)

immunity (WBCs)

hemostasis (stop bleeding)

homeostasis (provide optimal environment to
maintain steady state (pH, ions, temp, nutrient supply)

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2
Q

main role of RBCs

A

transportation of oxygen

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3
Q

how long do RBCs survive?

A

4 months (120 days)

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4
Q

precursor RBC in bone marrow

A

erythroblast

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5
Q

what are platelets?

A

essential for blood coagulation
much smaller than leukocytes
represent bits of the cytoplasm of megakaryocytes
largest precursor cells in the bone marrow
short survival, about 10 days

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6
Q

most numerous of the WBCs in an adult

A

neutrophils

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7
Q

what are bands?

A

slightly less mature neutrophils that have a non-segmented or band-shaped nucleus - neutrophils which have just been released from the bone marrow

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8
Q

sites of hematopoiesis

A

yolk sac
liver and spleen
bone marrow

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9
Q

what is a granulocyte?

A

WBC that is filled with microscopic granules
little sacs containing enzymes that digest microorganisms

innate immune system

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10
Q

role of basophil

A

secretory cell which mediates the hypersensitivity reaction

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11
Q

what is the largest cell in the peripheral blood?

A

monocytes

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12
Q

which WBCs are referred to as mononuclear?

A

monocytes and lymphocytes

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13
Q

what is the flexible inner protein fiber in RBCs?

A

spectrin

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14
Q

hematopoiesis occurs in the red bone marrow of what?

A

axial skeleton and girdles

epiphyses of the humerus and femur

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15
Q

Erythropoietin (EPO) release by the kidneys is triggered by:

A

Hypoxia due to decreased RBCs
Decreased oxygen availability
Increased tissue demand for oxygen

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16
Q

where does the body get iron from?

A

stores iron in Hb, the liver, spleen, and bone marrow

intracellular iron is stored in protein-iron complexes such as ferritin and hemosiderin

circulating iron is loosely bound to the transport protein transferrin

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17
Q

where does RBC breakdown occur?

A

spleen - dying RBCs are engulfed by macrohages. heme and globin are seperated and the iron is salvaged for reuse

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18
Q

what is heme degraded into?

A

Fe recycled ot ferritin or hemosiderin

yellow pigment called bilirubin

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19
Q

what is hematocrit?

A

portion of total blood volume that is RBC

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20
Q

what is MCV?

A

mean corpuscular volume - average volume/size of a single RBC

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21
Q

abnormal variation in cell volume

A

anisocytosis

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22
Q

when will you see target RBCs?

A

iron deficiency
liver disease
spleen removal
thalassemia

*densely stained central area with a pale surrounding area

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23
Q

what are platelets?

A

cytoplasmic fragments of megakaryocytes - in adults megakaryocytes are exclusively in the bone marrow

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24
Q

what is thrombopoeitin?

A

promotes the grown of Meg-CFC
increases the rate of endomitosis
inhibits apoptosis
stimulates magakaryocyte maturation

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25
does thrombopoeitin promote platelet release?
no
26
what inhibits platelet aggregation?
cyclic AMP | - Cyclic AMP increased by prostoglandins
27
what do anti-platelet drugs do?
interfere with prostaglandin synthesis Aspirin, NSAIDS - COX1 inhibitor Celebrex - COX 2 inhibitor interfere with cyclic AMP, ADP binding, and inhibition of the GPIIb/IIIa receptor
28
acquired platelet dysfunction is from what?
``` liver disease cadiopulmonary bypass uremia dysproteinemia myeloproliferative disorders (bone marrow) alcohol toxicity ```
29
inherited platelet dysfunction
``` giant platelet disorders Wiskott-aldrich syndrome storage pool disorders glanzmann thrombasthenia platelet release disorders ```
30
what is thrombocytopenia?
reduced platelet count in peripheral blood
31
what causes thrombocytopenia?
increased destruction** decreased production abnormal pooling or distribution within the body artifact
32
what is pseudothrombocytopenia?
conformational change in plt membrane Immune clumps the platelets cold dependent clumping *evaluate with smear
33
what is ITP?
immune thrombocytopenic purpura also called idiopathic autoimmune thrombocytopenic purpura occurs in both children and adults caused by platelet specific antibodies which bind to the platelets
34
this mediates adhesion of platelets to the collagen exposed on endothelial cell surfaces
von willebrand factor - acts as a bridge between a specific glycoprotein complex on the surface of platelets and collagen fibrils
35
vWF is required to bind to and stabilize coagulation factor what?
VIII
36
what is aggregation?
binding to other platelets via fibrinogen
37
aggregation is mediated by what?
fibrinogen receptors (GPIIb/IIIa)- undergo conformational change to active state
38
goal of clotting cascade
form fibrin
39
this is initiated when contact is made between blood and exposed negatively charged surfaces
intrinsic cascade
40
this is initiated upon vascular injury which leads to exposure of tissue factor
extrinsic pathway
41
what pathway does aPTT (activated partial thromboplastin time) evaluate?
intrinsic
42
what pathways doe PT/INR (prothrombin time) evaluate?
extrinsic
43
factors determining hemoglobin values
``` age gender race degree of sexual maturation altitude heredity ```
44
what does a high retic count indicate?
bone marrow is trying to compensate for anemia
45
symptoms of iron deficiency anemia
``` asymptomatic weakness, fatigue, SOB, pallor, dizziness, irritability, decreased exercise tolerance pica - severe state (ice craving) palor koilonychia tachycardic glossitis ```
46
what is sideroblastic anemia?
iron fails to bind to pophyrin ring in the erythroblast to produce heme iron is retained in the mitochondria of the cell ringed sideroblasts (normoblast with perinuclear halow of blue-stained material) seen on bone marrow smear with prussian blue staining
47
what is sideroblastic anemia characterized by
anemia low retic count ineffective erythropoiesis with distinctive mophology
48
treatment of sideroblastic anemia
remove offending agent | trial of pyridoxine to correct nutritional deficience
49
what are folate and B12 required for?
DNA synthesis and regulation
50
B12 and folate deficiency anemia results in what?
megaloblastic anemia due to reduced DNA replication
51
clinical manifestations of folate deficiency
jaundice due to ineffective erythropoiesis | smooth tongue
52
lab findings for folate and cobalamin deficiency
``` macrocytosis howell-jolly bodies relatively low retic count mild thrombocytopenia and/or neutropenia hypersegmentation of neutrophils megaloblastic changes in marrow ```
53
will magaloblastic anemia of B12 deficiency respond to folic acid in high doses?
yes, but neurologic deficits do NOT respond
54
what is anemia of chronic disease?
occurs in setting of chronic disease states associated with inflammatory cytokines or impaired marrow production induce production of hepcidin - blocks iron absorption from gut
55
causes of anemia of chronic disease
``` acute and chronic infections inflammatory and autoimmune diseases (T-cell mediated) cancer CKD liver disease MDS (myelodysplastic syndromes) aging ```
56
what is aplatic anemia
failure of bone marrow due to injury or abnormal expression of pluripotent hematopoietic stem cell hallmark: bone marrow shows hypocellularity and hypoplasia of all cell lines
57
most common cause of non-immune hemolytic anemia
hereditary spherocytosis
58
manifestations of sickle cell disease
``` chronic hemolytic anemia pain dactylitis acute chest syndrome chronic organ damage (spleen, brain, kidneys) ```
59
most frequent reason for sickle cell hospitalization
vaso-occlusive pain crisis | severe, agonizing pain usually starting in limbs
60
transfusion indications in sickle cell
``` acute stroke or TIA acute chest syndrome priapism splenic sequestration pre-operative sympotomatic anemia aplastic crisis abnormal transcranial doppler ```
61
what is not indicated for a sickle cell transfusion?
pain crisis | anemia
62
neonatal factor assays for hemophilia
should be performed on male infants with a family hstory of bleeding problems or prolonged bleeding after circumcision hemophilia A - can be diagnosed from FVIII levels in CORD BLOOD collected immediately after delivery hemophilia B- requires blood to be taken after 6 months of age since FIX is vitamin-K dependent and all neonates have low levels of vitamin K and FIX
63
what should you avoid in patients with severe pain due to bleeding in joints?
aspirin and NSAIDS - can exacerbate bleeding
64
what is DIC characterized by?
clotting and bleeding systemic activation of coagulation system results in overactivation of the fibrinolytic system - clotting due to circulating thrombin - bleeding due to depletion of clotting factors and excessive fibrinolysis
65
why do you have platelet dysfunction in coagulopathy of liver failure?
splenomegaly due to portal hypertension - sequesters platelets ETOH cirrhosis- suppression of thrombopoeisis by toxicity of alcohol or concomitant folate deficiency
66
where are you clotting factors made?
liver - except VWF
67
what is the best prognostic indicator in Acute liver failure?
factor V level- helps determine need for urgent transplant in patient presenting with encephalopathy
68
treatment of coagulopathy in liver failure
not curative, only supportive novoseven (recombinant human factor VII) - off label use in liver disease but use is restricted due to limited data and excessive cost
69
what coag factors are dependent on vitamin k?
II, VII, IX, X
70
what is virchow's triad?
pathrogenesis of a thrombus endothelial injury abnormal bloodflow hypercoagulability (primary - genetic or secondary - acquired)
71
what signs/symptoms are indicative of a DVT?
painful, swollen, warm and plethoric extremity with reduced pulse volume
72
what signs/symptoms are indicative of a PE?
cough, SOB, hemoptysis, tachycardia
73
hypercoagulable state workup for primary
protein C activity - impaired inactivation of factors V and VIII -->unregulated fibrin production protein S activity - impaired inactivation of factors V and VIII -->unregulated fibrin production antithrombin III - major inhibitor of thrombin and fibrin formation factor V leiden
74
hypercoagulable state workup for secondary
anticardiolipin antibody lupus anticoagulant homocysteine
75
what is factor V leiden mutation?
activated protein C resistance point mutation in gene for Factor V renders it unable to be inactivated by protein C
76
primary HCS screening guidelines
VTE
77
primary HCS treatment
heparin drip until therapeutic with warfarin goals: 2-3
78
primary HCS duration of tratment
first VTE episode: 6-12 months | recurrent VTE: lifelong therapy
79
who is likely to have antiphospholipid syndrome
often associated with Lupus | recurrent pregnancy loss
80
what is antiphospholipid syndrome?
autoantibodies directed against phospholipids in cell membranes lining blood vessels induces endothelial cell damage, predisposing to thrombosis - starts coag cascade with no tissue damage
81
what should you suspect in patients with prolonged aPTT with no clinical bleeding?
lupus antigoagulant - clinically not a bleeding disorder. just an antibody that prolongs aPTT by binding to phospholipid in the lab assay
82
what are mixing studies used for?
performed on blood plasma used to distinguish factor deficiences from factor inhibitors
83
what are the primary lymphoid organs?
thymus and bone marrow
84
what are the secondary lymphoid organs
spleen MALT lymph nodes tonsils
85
what is the complication from tumor lysis syndrome (TLS)?
marked increase in uric acid excretion can result in ACUTE RENAL FAILURE - occurs with rapid release of intracellular potassium and phosphorous into blood stream
86
diagnostic tests for tumor lysis syndrome
``` LFT and LDH uric acid renal function test serum electrolytes EKG if hyperkalemic ```