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48 Cards in this Set

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Anemai with elevated reticulocytes tell you that the problem does not stem from the ____
bone marrow

(bone marrow is normally producing RBC @ ^ rate but somethings is destroying the RBCs)
Evidence of hemolysis
Jaundice (skin & sclera)

Pallor (bc anemic)

Dark urine (Hb & hemosiderin in urine)

^ Reticulocytes

^ LDH (enzyme present in RBCs)

^ indirect/unconjugated bilirubin (bc ^ breakdown of heme, liver is overwhelmed)
Name the 4 morphological hallmarks of Hemolysis
1)Polychromasia - immature cell showing tha tBM is working harder; larger than RBC & bluish in color

2)Spherocytes - more round (not biconcave), more Hb crammed into a small space -> darker appearance; classic for autoimmune hemolytic anemia & hereditary spherocytosis

3)Schistocyte - fragmented cells that are ripped apart in circulation; fibrin deposition in small BV, sharp edges; seen in microangiopathic hemolytic anemias, heart valves, DIC

4)Nucleated RBC - easily recognizable; suggests that BM is working hard
RBC sole source of energy?
Glycolysis - remember RBC lack mitochondrea

also NO NUCLEUS or RIBOSOMES - do not make protein, just carry O2 and Hb
What does RBC need ATP for (6)?
1) Na/K pumps
2) Ca pumps
3) Needs energy to maintain biconcave disc shape
4)Regulation of phospholipid in bilipid membrane so that negative are insede and positive outside (prevents coagulation)
5)Reducing equivalents - keep reduced glutathione to prevent oxygen damage
6)Keep sulfhydryl groups present (maintain ferrous form)
Through what biochemical pathway does RBC get its reducing equivalents (GSH)?
Hexose monophosphate shunt

10% of glucose goes through (90% through glycolysis)
Key enzyme in Hexose monophosphate shunt?
Glucose-6-phosphate dehydrogenase (G6PD)

G6PD converts NADP+->NADPH; Glutathione reductase then uses this NADPH to make glutathione

ABSOLUTELY have to have this and Hexose monophosphate shunt to keep RBC healthy
T/F G6PD Deficiency is mostly suffered by men?

T/F G6PD Deficiency is a chronic hemolytic anemia?
T, X-linked dz

F, just episodic hemolysis..there is a Shortened RCB survival, in general affected people have a normal Hb and normal life
G6PD Def can cause what in neonatal period?
Common cuse of neonatal Kernicterus and Jaundice (lead to mental retardation)
T/F Old cells have more G6PD than young cells?
**F, young cells usualy have more G6PD ("important test point")**

degree of deficiency of old cells PREDICTS the severity of anemia
Describe the mechanism of hemolysis by G6PD Def
Oxidants can damage any protein with sulfhydryl group, particularly Hb b/c it ahs oxygen & is easily damaged when not much glutathione around

Denatured, oxidized Hb can precipitate and form HEINZ BODIES
Heinz bodies affects of RBC (2)?
1) very toxic to cell; adhere to RBC membreane making it stiffer and more difficult to transverse microcirculation and spleen

2)easily lysed -> v RBC survival (frank hemolysis)
T/F Neonatal jaundic enot actually a hemolytic problem?
T, G6PD Def comvined iwth Gilbert's disease (deficiency of bilirubin conjugation enzyme) can result in very high bilirubin levels and may -> kernicterus -> mental retardation
Phagocytosed Heinz bodies called?
Bite cells, usually formed in spleen

Both 1)Bite Cells & 2)Heinz bodies used for diagnosis of G6PD deficiency
T/F ther is no clinical significance wtih G6PD A+
T, normal G6PD activity, just migrates differently on electrophoresis gel
Normal type of G6PD is?
G6PD B (bulk of world's population
MC form of G6PDD in america
G6PD A-

Moderate to severe hemolysis w/ stress

Cells decay faster so there are more cells w/ v G6PD

Esp larger % in blackies ;)
One benefit seen by ALL G6PD genetic defects?
Malaria resistance
Southeast Asian form of G6PD defect?
G6PD Canton
More severe type of G6PD defect?
G6PD B-

Sever hemolysis after stress

Lower activity level, decays even faster (bc less cellular protective effects)

Can be associate with favism (damn you fava beans!!)
Give some examples of oxidant drugs
Antimalarias - primaquin, chlorquin

Sulfa drugs - sulfonylamide

Nitrofurantoin


All make you esp vulnerable when suffering from G6PDD
Clinical Presentation of G6PDD
1)Acute hemolysis of oxidant stress (ie Infections, Diabetic ketoacidosis, Surgery, Oxidant drugs)

2)Appropriate ethnic or gender backgound

3)Jaundice, pallor, dark urine
T/F Onset of G6PDD is usually gradual
F, onset usually SUDDEN (hours to days after insult)

many times presents with BACK Pain and RETROPERITONEAL symptoms
Something to beware when using enzyme assay to maeasure NAPDH production to test G6PDD
NAPDH production may be NORMAL during ACUTE G6PDD episode (false negative!)
2nd MC hemolyic RBC enzyme disorder but much less common than G6PDD? Mech?
PK deficiency

v ATP -> cannot mantain gradients -> water loss -> cell SHRINKS and becomes RIGID

Cells cannot traverse spleen or microciculation, resulting in CHRONIC hemolysis
Clinical Features of PK Def?
Homozygotes are anemic and jaundiced at birth

Bc of ongoing meolysis, prone to bilirubin GALLSTONES and SPLENOMEGALY bc the spleen is full of dead and dying RBCs

usually heterozygous & heterozygots are asymptomatic

LIFELONG transfusion requirements
Biochemical deficiency that is involved in the breakdown of RNA @ reticulocyte stage and congenital ongoing hemolysis?
Pyrimidine 5'-nucleotidase deficiency

causes damage to RBC membrane and thus congenital ongoing hemoysis

Also seen in LEAD poisoning & THALASSEMIAS
The process of maturation fo a mature RBC from a erythroblast takes place ____ until the reticulocyte stage, and then goes to the ____ ?
first BM, the to peripheral blood

lose organells and nuclear material & gain Hb with RBC maturation
How can you tell the maturity of a RBC through histology?
by the nuclear material that it contains (the more the less mature)
RBC takes how long for maturation?
8-12 days
MC form of anemia?
Hemorrhage
Rapid Hemorrhage defined as
loss of more than 20% of blood volume in less than 1 hour

caused by: extravasularly by trauma & intravascularly by G6PD Def & acquired hemolytic anemia
Patient can tolerate up to ___% loss of blood volume as long as it is not acute.

Compensatory mechs?
70% volume tolerance by:

1)^ BP, HR, CO
2)shuntivnng of blood from peripherial system
3)increased 2,3-DPG levels
End form of all anemias lead to?
Tissue anoxia and/or cell death
Describe stages of incerased RBC destruction continuum
Anemia -> Hemoglobinuria/emia -> Jaundice (from excess Hb)-> Cholelithiasis (gall stones; trying to excrete excess bilirubin) -> Aplastic anemia -> Hemolytic crisis
What enzyme is markedly elevaed in MEGALOBLASTIC anemia vs other types
Serum LDH increases a lot
Again, the 4 distinctive cell signs on blood smear indicating severe hemolysis?
Polychormasia, Spherocytes, Schistocytes (Fragmented cells) & Nucleated RBCs
Conplications of ^ RBC sequestration (vaso-occlusion)
1)Autosplenectomy

2)Renal papillary necrosis - from distal capillary beds clogged

3)Dactylitis (abnormal growth of digits)

4)Sequestration crisis (v ability to oxyginate -> Acute splenic & pulmonary crisis (both often seen with Sickle Cell Dz)

5)Leg ulcers
Abnormal growth/assymetry of digits due to the inability to oxygenate the tissues
Dactylitis
4 Manifestations for Erythroid Hyperplasia?
1)Hypercellular marrow (>95% cellularity; normal cellularty for an adult is 100 minus age)

2)Extramedullary hematopoisesis (can occur in spleen, liver, etc)

3)Expansion of marrow space

4)Bone "mass' formation and deformities
Intravascular or Extravascular hemolysis leads to what 3 things?
1)System iron overload

2)Renal Failure

3)Liver/congestive Heart failure (tiger striping of heart due to lipid accumulation)
A Sickle Cell Dz patient with watch will show severe cytopenia, anemia & hypocellular bone marrow with GIATN ERYTHROID PRECURSORS
Parvo virus
A clonal red cell abnormality that has proteins that are susceptible to complement destruction called?
Paroxysmal Nocturnal Hemoglobinuria (PNH)
In Sickle Cell Dz, osteomyelitis is classically caused by what?
Salmonella
Bone marrow is devoid of hematopoietic elements called?
Aplastic anemia
Large Erythroid precursors think..
Parvo virus
Reticulocyte normally spends _____ in BM and _____ in peripheral blood b/f teh rest of the nuclear materials are excluded?

Difference in crisis?
one & one

in crisis, reticulocytes shift from the BM into the blood and maturation time in blood increases to 1.5-2.5 days, depending on severity of anemia
Reticulocyte Production Index (RPI) formula:
RPI = (Hct/45) * Reticulocyte%/Correction factor