Hematology 1-5

Medicine Dit Class with Meyer at University of Chapel Hill

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By: John Meyer
Created: 2012-05-15
Size: 79 flashcards
Views: 16

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Heparin, antidote

Protamine sulfate(bc it has a positive charge and binds negatively-charged heparin)

Toxicities of heparin

bleeding, HIT, osteoporosis, and drug-drug interactions

HIT, what is mechanism

heparin binds to platelet factor IV, and this causes auto-antibodies to this complex.

binds and activates platelets -> thrombocytopenia but hypercoagulable state

tx for HIT

Stop heparin then give direct thrombin inhibitor(Bivalirudin or Lepirudin)

5 hereditary thrombosis syndromes

1. Factor V Leiden
2. Prothrombin mutation(G20210A mutation)
3. Antithrombin deficiency
4. Protein C deficiency
5. Protein S deficiency

heparin and warfarin, which crosses placenta

Warfarin does cross the placenta and is teratogenic, so don't give it during pregnancy

Heparin is fine during pregnancy

Warfarin overdose tx

Fresh frozen plasma + vitK

Effects of bradykinin on body

Increased vessel permeability, vasodilation, and causes pain

Kallikrein, relation to coag cascade and what it goes on to activate?

Factor XIIa is needed to convert Prokallikrein to Kallikrein

goes on to make Bradykinin and Plasmin
Important link between inflammation and coagulation

Effect of citrate on coag cascade?

It is a Ca++ binder, so it would make coagulation less effective

Fetal erythropoiesis

"Young Liver Synthesizes Blood"

1st(at 3wks) Yolk sac makes blood
Liver is most important for fetus
Spleen
Bone marrow

which bones make blood

In kids, flat bones and long bones(sternum, pelvis, ribs, cranial bones, vertebrae, tibia, femur)

long bones become quiescent in adolescents, and so adults only have bones of axial skeleton involved in blood formation

HbF

Lower affinity for 2,3BPG, so higher affinity for O2.

Gets O2 for fetus bc maternal Hb has less affinity for O2 so HbF can grab it, forming the shunt needed to oxygenate baby

poikilocytosis

varying shapes

RBCs seen with liver dz

Acanthocytes and Target Cells

Irregular spikes on the RBCs

acanthocytes

Liver dz and abetalipoproteinemia

Basophilic stippling

Mainly think Lead poisoning

Also seen with Thalassemias, Anemia of chronic dz, and Iron deficiency

"TAIL"

What are Heinz bodies

Denatured hemoglobin (due to oxidation of iron from ferrous to ferric form)

Damage RBC membrane and leads to bite cells

Ringed sideroblasts

found in bone marrow

Sideroblastic anemia

Target cells

HALT
HbC dz, Asplenia, Liver dz, and Thalassemia

Basophilic nuclear remnants found in RBCs

Howell-Jolly bodies

seen in pts with functional hyposplenia or asplenia (think sickle cell, ITP or trauma pts)

RBCs in pt with no spleen

Target Cells

Howell Jolly bodies

Causes of inappropriate absolute Polycythemi

Renal Cell Carcinoma, Hepatocellular Carcinoma, Wilms' tumor, cyst, hydronephrosis

all produce ectopic epo

tx for lead poisoning in an adult

Dimercaprol

Tx for lead poisoning in kid

succimer

HbC

hemoglobin C dz

beta chain problem, lysine substitutes for glutamate

LC (leucine in hbC)

Hb Bart's

hydrops fetalis = dz

4 gamma chains, no alpha chains due to 4gene deletion

HbH

due to severe alpha-thalassemia or HbH dz.

4 beta chains and no alpha chains due to 3 gene deletion

Lead inhibits what

Ferrochetalase and ALA dehydrase

also inhibits rRNA degradation -> basophilic stippling

headache, memory loss, demyelination

Lead poisoning in an adult

might also get colicky abdominal pain, wrist/foot drop, etc

tx for acute intermittent porphyria

heme(bc it inhibits ALA synthase) and glucose

Skin problems, tea-colored urine, excessive hair growth

what dz, enzyme deficiency, and associations(2)

Porphyria cutanea tarda

Uroporphyrinogen decarboxylase
hepC and alcoholism

red-wine colored urine, painful abdomen, polyneuropathy, psych issues

what dz, enzyme deficiency

Acute Intermittent porphyria

Porphobilinogen deaminase (aka uroporphyrinogen-1-synthase)

rate-limiting enzyme of heme synthesis, what does it need

aminolevulinic acid synthase(inhibited by glucose and heme)

needs vitB6

what is the alpha-globin chromosome

alpha-thal trait

2 gene deletion

no significant anemia

dx of beta-thal minor

usually asymptomatic, so what you look for is increased HbA2 on electrophoresis(>3.5%)

beta-thal major tx

requires blood transfusions, but have to be careful of secondary hemochromatosis

why you might use iron-chelating agent deferoxomine

tx for sideroblastic anemia

vitB6. works bc it is a cofactor for ALA synthase

what is an important lab that can help you distinguish folate from B12 deficiency

methylmalonic acid(increased with B12 def, and it is normal with folate def)

both have elevated homocysteine

specific neuro findings w B12 deficiency

1. peripheral neuropathy
2. posterior columns (vibration/proprioception)
3. Lateral corticospinal (spasticity)
4. Dementia

causes of nonmegaloblastic macrocytic anemias

alcoholism/liver dz, 5-FU, AZT, hydroxyurea

nonhemolytic normocytic anemias

anemia of chronic dz, aplastic anemia, and kidney dz

hypocellular bone marrow w fatty infiltration

aplastic anemia

Causes of aplastic anemia

Radiation and drugs(chloramphenicol, alkylating agents, benzene), viral agents (parvovirus B19, EBV, HIV), and Fanconi's anemia(DNA repair defect)

other sxs that are a hint for aplastic anemia

purpura(thrombocytopenia) and infections(leukocytopenia)

anemia of chronic dz, mechanism and labs

IL-6 stimulates hepcidin release(inhibits iron transport).

Leads to decreased release of iron from macrophages

decreased iron, TIBC, and increased ferritin

HIV positive pt w macrocytic anemia

Zidovudine

Microcytic anemia reversible w vitB6

sideroblastic anemia

elevated jaundice with a hemolytic anemia

extravascular hemolysis(like sickle cell anemia or heriditary spherocytosis)

decreased haptoglobin with a hemolytic anemia

intravascular hemolysis (like with mechanical destruction or paroxysmal nocturnal hemoglobinuria)

paroxysmal nocturnal hemoglobinuria (intra or extravascular hemolysis? unique lab? complication?)

Complement-mediated RBC lysis (impaired synthesis of DAF).

intravascular hemolysis
increased urine hemosiderin
Complication = thrombosis

red cell lysis at low pH

Ham's test

classic test for Paroxysmal Nocturnal Hemoglobinuria, but it's a little old, now we do flow cytometry

what precipitates sickling and what complications is this related to in the dz

low O2 or dehydration

painful crisis
renal papillary necrosis(due to low O2 in papilla)

does hereditary spherocytosis have a positive Coombs' test?

no, negative Coombs

+ osmotic fragility test

Cold agglutinins, occur regularly in infections with...

mycoplasma pneumo, EBV, malignancies

Warm agglutinins, almost always seen with...

viruses(EBV, HIV, etc), SLE, malignancies, congenital immune abnormalities

IgG

Platelet plug formation, 3 steps

adhesion, activation, aggregation

where does vWF come from and what does it bind during injury?

Comes from the damaged endothelial cells and binds to the newly-exposed collagen

Adhesion of plts

Plts bind vWF via Gp1b

Plt activation

Change of shape to allow tighter binding

also secretion of ADP, PDGF, thromboxane A2, thrombin and calcium, among other things

Aggregation of plts

ADP had upregulated GpIIb/IIIa and that is how aggregation occurs

Only platelet disorder with normal platelet count

Glanzmann's thrombasthenia

Cause of ITP

Anti-GpIIb/IIIa antibodies -> platelet destruction

cause of bernard-soulier dz

decreased Gp1b -> defect in platelet to collagen adhesion

Glanzmann's thrombasthenia, cause

decreased GpIIb/IIIa -> defect in platelet to platelet aggregation

no platelet clumping on blood smear

TTP, cause

Deficiency of ADAMTS 13(vWF metalloprotease) -> lack of degradation of vWF multimers.

You get large vWF multimers -> increased platelet aggregation and thrombosis

TTP pentad and how is HUS different w presentation

"Nasty Fever Torched His Kidneys"
1. Neuro sxs 2. Fever 3. Thrombocytopenia 4. Hemolysis 5. Kidney Failure

HUS is just without neuro szs and fever

tx of vWF

DDAVP which is synthetic, strong form of ADH. Releases vWF stored in endothelium

causes of DIC

STOP Making New Thrombi

sepsis, trauma, obstetric, pancreatitis, malignancy, nephrotic syndrome, Transfusion

labs for DIC

schistocytes, increased fibrin split products(D-dimers), decreased fibrinogen(bc it's all being used up making clots), and decreased Factors V and VIII(also being used up

Aspirin toxicity

gastric ulceration, hyperventilation(leading to resp. alkalosis), Reye's syndrome, tinnitus

streptokinase, tPA

clot-busters.

Convert plasminogen to plasmin

thrombolytic toxicity, what is the tx

aminocaproic acid(an inhibitor of fibrinolysis)

Clopidogrel, ticlopidine

Irreversibly block ADP receptor

this prevents glycoprotein IIv/IIIa expression

can cause neutropenia

Aptifibatide and Tirofiban

block glycoprotein IIb/IIIa

Abciximab

monoclonal antibody that binds to IIb/IIIa and prevents aggregation

microcytic anemia reversible w vitB6

Sideroblastic anemia

HIV + pt w macrocytic anemia

Zidovudine use

About this deck

By: John Meyer
Created: 2012-05-15
Size: 79 flashcards
Views: 16

About StudyBlue

“I have been getting MUCH better grades on all my tests for school. Flash cards, notes, and quizzes are great on here. Thanks!”
Kathy