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86 Cards in this Set

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what does the RCDW measure

red cell distribution width. a coefficent of variation in red cell volume

what are some symptoms that are characteristic of anemia

weakness, malaise, easy fatigability, and mild dyspnia on exertion



more extreme cases: fatty change of liver, myocardium and kidney

how long from the onset of hypoxia and the release of erythropoietin from the kidney does it take CFU-E proenitors to mature and appear in the blood

5 days. they appeat as reticulocytes

leukocytosis, reticulocytosis, and thrombocytosis are often associated with

blood loss anemias

shortened blood cell lifespan (<120 days), elevated EPO levels, and accumulation of hemoglobin breakdown products are all hallmark signs of __________________

hemolytic anemia

what is the difference between intravascular and extravascular hemolysis

extravascular hemolysis occurs when RBCs are destroyed within phagocytes, can lead to splenic and phagocytic hyperplasia, also jaundice and reduced haptoglobin



intravascular hemolysis is caused by mechanical injury, compliment fixation, and parasites, this is less common

what rises as haptoglobin is used up?

free hemoglobin in the blood is oxidized to methemoglobin (Fe+++ hemoglobin)



free iron is converted to hemosiderin and can accumulate in the kidneys



bilirubin accumulates and can cause jaundace

is splenomegaly seen in extravascular hemolysis


is excess serum bilirubin conjugated


no and no

defecits in ankryin, band 3, spectrin, and band 4.2 cause what disorder?

hereditary spherocytosis

what anemia would you suspect if a patient presented with spleomegaly, and jaundace, as well as small dark staining hyperchromic red cells

hereditary spherocytosis

what can cause a sudden worsening of spherocytosis

parvovirus infection

what painful complication is associated with excessive bilirubin released into the GI tract

gallstones derived from heme pigments

what x linked recessive deficency associated with the hexose monophosphate shunt or glutathione metabolism reduces the ability of red cells to protect themselves from oxidative injuries

Glucose 6 phosphate deficency



reduction of glutathione (which eliminates H2O2 radicals) is dependent of G6P

What would worsen the anemia of a patient with G6P deficency

increased oxidative stress, like that assiciated with infections

what is a heinz body? what is a bite cell?

dark inclusions within red cells associated with G6PD



macrophages in the spleen try to get the heinz bodies and take a bite out of the affected cells

what causes the deficit that is responsible for sickle cell disease?

point mutation (glutamate switches to valine) in the beta globin gene which causes polymerization of deoxygenated hemoglobin

what is the composition of Hemoglobin A

2 alpha and 2 beta chains

what is the composition of HbS

2 alpha chains and 2 beta-s chains

what is the effect of increased HbF on sickle cell patients

milder disease because HbF prevents sickling of HbS

what is the effect on dehydration on MCHC in sickle cell patients? what does this cause?

higher MCHC, more sickling

how does the bhor effect relate to sickle cell disease?

lower pH (as in higher CO2) causes lower affinity of hemoglobin for oxygen. this increases fraction of deoxy HbS and increases sickling

why is the spleen a common site for sickle cell aggregation

sluggish blood flow

what is responsible for the most serious clinical features of sickle cell disease

microvascular occlusions which cause hypoxia, further sickling, pain and infarct (vaso-occlusive crisis)



hand foot syndrome and acute chest syndrome are specific types of vaso occlusive crises

why is hydroxyurea affective in treating sickle cell disease

1. increases HbF


2. has an antiinflamatory effect


what mutations are most often associated with B+ thalassemia

splicing mutations

what mutations are most commonly associated with B0 thalassemia

chain terminator mutations (frameshifting) prevent any functional synthesis of hemoglobin B

what would you see in a CBC of a patient with B-thalassemia

hypochromic, microcytic red cells and less HbA due to imbalance in a/b globin synthesis

what role does hepticidin play in thalassemia syndromes

hepticidin is a negative regulator of iron absorbtion. ineffective erythropoesis results in excessive iron accumulation

what genotypes are associated with transfusion dependent B thalassemia major

B+/B+


Bo/Bo


B+/Bo

what genotypes are associated with the beta thalessemia trait (minor)

Bo/B


B+/B

in what poplations is B thalassemia major the most common

mediterranean, africa, SE asia

when does b-thallasemia manifest

6-9 months when HBF production swithes to HbA

how many alpha globin genes are usually present?

4. thalassemia severity depends on number of genes that are defective. deletion of a single gene results in a silent carrier state

what are the genotypes associated with alpha thalassemia trait

a/a -/- , a/- a/-

which populations have a higher incidence of alpha thalassemia

asians

what is hemoglobin H disease

deletion of 3 alpha globin genes. causes formation of tetramers of beta globin which have a very high affinity for oxygen and are not effective for oxygen transfer

what is hydrops fetalis

deletion of all 4 alpha globin genes. results in a lifelong dependence on transfusion for survival, hematopoetic stem cell transplant can be curative

what is paroxysmal nocturnal hemoglobinuria

anemia associated with membrane compliment regulatory proteins (PIGA).



rbcs deficent in this are susceptable to lysis by compliment mediated processes especially the C5-9 membrane attack complex

what is warm antibody type immunohemolytic anemia

idiopathic hemolytic anemia characterized by IgG attack of RBCs



often due to presence of antigenic drugs. will manifest 1-2 weeks after dose of dru is applied

what is cold-agglutin type hemolytic anemia

IgM attack of RBCs in low temperature settings. presents like reynaud phenemenon

what is the most common hemolytic anemia associated with trauma to red cells

cardiac valve replacements can cause turbulent flow

what is a helmet cell

red cell fragment associated with trauma to red blood cells due to sheer stress

what are common themes aong megaloblastic anemias?

impaired DNA synthesis


ineffective hematopoesis


abnormally large erythroid precursors and red cells

what is the pathogenesis of pernicious anemia

autoimmune gastritis decreases intrinsic factor which is required for B12 absorption. Decreased B12 causes megloblastic anemia.



type 1 antibodies block b12 from binding to intrinsic factor



type 2 antibodies prevent b12-IF from binding to ileal receptors for uptake

what are the criteria for diagnosis of pernicious anemia

1. megloblastic anemia (moderate to severe)


2. leukopenia


3. hypersegmented granulocytes


4. elevated homocystene/methylmalonic acid

is anemia of folate deficency clinically different from B12 deficent anemia

no not really

what is the most common nutritonal deficency in the world?

iron deficency



more common in women paricularly of childbearing age

what carries iron in the blood?

transferrin

what levels of hepticidin do you expect to see in hemochromotosis

low

who is at highest risk for iron deficency

infants


the impoverished


older adults


teenagers

what is sprue

impaired absorption can cause iron deficency

what is the pathogenesis of iron deficency anemia

hypochromic, microcytic anemia


lower serum iron and transferrin saturation


increased erythroid activity


reduced serum heptacidin


what is the effect of sustained inflammation on anemia?

can lead to anemia


IL-6 stimulates an increase in heptacidin production which reduces transport of ron from storage pool to erythroid precursors

what is the most common cause of aplastic anemia

exposure to chemicals, drugs, and irridation

what is fanconi anemia

autosomal recessive d/o that is caused by a defect in the DNA repair



accompanied by kidnek and spleen hypoplasia

what is pancytopenia

anemia


neutropenia


thrombocytopenia

a patient presents with a moderate to severe anemia that is without splenomegaly. With only this information what type of anemia can you rule out

aplastic anemia is almost never associated with enlargment of the spleen

what is pure red cell aplasia and how does it differ from aplastic anemia

only erythroid precursors are affected



leukocytes and platlets are unaffected



what are acute and chronic causes of red cell aplasia

acute- parvovirus B19



chronic- thyroma, antibodies that act against EPO

what form of marrow failure is associated with space occupying lesions replacing normal marrow elements

myelophtisic anemia

what distinguishes absolute polycythemia from relative polycythemia

relative is due to deyhdration



absolute can be primary (increase in progenitors) and secondary (increase in EPO)

what is the most common cause of primary polycythemia

polychthemia vera



mutations cause EPO independent RBC development

what do PT and PTT measure?

PT measures extrinsic and common coagulation pathways (V,VII,X, PT, fibrinogen)



PTT measures intrinsic and common coagulation pathways (V,VIII,IX,X,XI,XII, PT, fibrinogen)

Scurvy and Ehlers-Danlos syndrome are both ossciated whith what type of anemia

hemmorhagic diatheses associated with microvascular bleeding

what serious hereditary blood disorder is associated with dilated, tortuous blood vessels with thin, easy bleeding walls and frequent nosebleeds

hereditary hemorrhagic telangiectasia

<100k platelets per microliter is associated with what bleeding dsorder,

thrombocytopenia


what an cause decreased platelet count

aplastic anemia, leukemia, drugs, HIV, alcohol

what is an alloantibody? how does it cause anemia?

can cross placenta and attack platelets in newborn causing thrombocytopenia

What causes chronic ITP? What are the clinical signs?

autoantibody mediated destruction of platlets



adult women <40yo pinpoint petechiae with occasional progression to ecchymoses



PT/PTT normal



tx with glucocorticoids

What is acute ITP who gets it

autoantibodies to platelets occuring in childhood usually self limiting



tx with glucocorticoids if thrombocytopenia is severe

What is HIT? Type 1? Type 2?

Heparin which is an anticoagulant can cause low platlets in 2 patterns



1. Type 1 occurs rapidly after onset of therapy


2. Type 2 occurs 5-14 days after initial treatment and can cause life threatening thrombosis due to antibodies that recognize heparin and platlet factor 4.

What is the most common hematologic manifestation of HIV infection

thrombocytopenia

A deficiency of plasma enzyme ADAMTS13 is associated with which bleeding disorder?

TTP



ADAMS13 degrades high molecular weight vWF to a less coaguable form. without it you see aggregates of vWF in the plasma which can trigger activation of platelets and microthrombi

What infection is associated with Hemolytic-Uremic Syndrome (HUS)

E coli. A shinga like toxin from E Coli can be absorbed from gastric mucosa and enter peripheral circulation where it promotes formation of microthrombi

what disorder causes abnormal platelet adhesion due to inherited deficits of glycoprotein complexes on the platelet membrane?

bernard souiler syndrome

what is a consequence of excess asprin and NSAID

asprin irreversibly binds COX which forms prostoglandins some of which are important mediators of platelet activation

what are the 2 most common inherited disorders of bleeding

hemophelia A and vWF disease

what does factor 8 do?

binds to vWF which stabilizes it and allows it to survive in the blood where it activates IX which activates X and propagates the clotting cascade.


What is type 1,2,3 vWF disease

1- dominant D/O, mild to moderate vWF deficit (70%) of cases



3- worse version of 1



2- qualaitative defects in vWF

how does vWF disease affect PT, PTT ?

PT normal


PTT prolonged

how would you distinguish hemophilia A from hemophilia B?

Trick question! VIII (A) activates IX (B) so they are virtually indistinguishable clinically



you will see prolonged PTT and normal PT in patients with A and B

release of tissue factor into circulation and widespread injury to endothelial cells are two triggering mechanisms for what life threatening disease?

DIC

What mediator of endothelial injury is often implicated in DIC occuring with sepsis

TNF which induces endothelial cells to express tissue factor, decrease thrombomodulin expression, also upregulates adhesion molecules on endothelial cells promoting leukocyte adhesion and ROS release

What is most often associated with DIC

obstetric complications, sepsis major trauma and certain cancers

patients with a deficit in ____________ are prone to severe potentially fatal allergic reactions with transfusions

IgA

What is TRALI?

transfusion related acute lung injury associated with transfusions triggering neutrophilia in the lung microvasculature