What are reasons cells wouldn't pass the flexibility test in the spleen?

-denatured hemoglobin

-have antibody attached

-have complement attached

-fragile, damaged cells

What does heme get turned into after iron is removed?

Increase in:

-bilirubin

-unconjugated bilirubin

-urobilinogen in urine

-decreased haptoglobin

-hemosiderin in kidneys (from reabsorption of free hemoglobin)

-later: hemoglobin in urine-when kidney if full

-when haptoglobin is exhausted; other plasma proteins carry hemoglobin to liver for processing

a Defect in the sodium/potassium pumps which leads to influx of sodium and influx of water

-rare autosomal dominant

-RBC loses K+ too fast causing dehydration of cells

-projections from rbc

-also: stomatocytes, target cells, macrocytes, abnormal hgb distribution

*disregulation of lipid metabolism

-irregular projections (pseudopods)

How is Acanthocytosis acquired?

-Vitamin E deficiency

-Liver disease (alcoholics)

-precipitates of denatured hemoglobin(heinz bodies)

-causes k and na to leak out(drying cell)

-COOKIE BITEs

-Loss of flexibility

Bite cells

low haptoglobin bc intravascular hemolysis

high bilirubin

high WBC

hemoglobinuria

heinz bodies

low G-6-P

-low O2-high altitude

-oxidizing drugs (methylene blue, antibiotics, antimalarial meds)

-Fava beans

-infections

if intravascular hemolysis occurs:

-dec haptoglobin

-inc bilirubin

-inc urobilinogen

-high 2,3 BPG

What is IgG and what kind of hemolysis does it start?

divalent antibody

reactive at 37C

Extravascular hemolysis

decavalent

35-37C

Activate COMPLEMENT

intravascular and extravascular hemolysis

1. widespread activation targets RBCs

2. Drugs cause complement to stick the RBC

Describe DAT:

1. Use anti-human antibody to detect IgG or Complement

--->if positive<----

RBC clumps together

2. Distinguish bw the 2: use specific anti-human serum

-autoimmune diseases

-leukemias/lymphomas

-viral infections

• episodes of JAUNDICE
• fever
• pale
• splenomegaly/hepatomegaly
• tachycardia

Immunosuppressive therapy

What are the 2 types of Cold Reactive Antibodies?

1. Cold Agglutinin Disease

2. Paroxysmal Cold Hemoglobinuria (episodic Hg in urine from cold)

-hemolysis all the time

-Complement fixed to cells 20-25C

• Vascular obstruction (gel forms)
• Raynaud's syndrome
• white, blue fingers

IgG mediated

therefore extravascular

Acute massive hemolysis after <15C cold exposure

when IgG attaches

• aching in back (from kidneys) and legs
• abdominal cramps
• headaches (dec O2)
• chills/fever
• hemoglobinuria

1. Transfusion Reactions
2. Hemolytic Disease of the Newborn

Hgb H (4xbeta)

Hgb Barts (4xgamma)

•  4 alpha mutations
• micro/hypo
• nRBCs
• Hgb Barts
• fetus survives, but is stillborn or dies shortly after birth

• Poikilocytosis (target cells)
• golfball from Brilliant cresyl blue stain

1. Knockouts
2. Frameshifts and other protein mutations
3. Reduced production bc promoter isn't strong

Beta Thal Major: LOW HGB

• dependent on transfusions and chelation therapy to prevent overload or bm transplant
• micro/hypo: stippled cells, nRBCs, low MCV, wrinkled cells
• BM in skull causes face weirdness

• mild/undectable bc make enough B
• moderately severe anemia
• exercise intolerance/ easy fractures

2 minor/one severe mutation

chelation therapy not transfusion

How can iron overload occur after hemolysis in Beta Thal intermedia even in absence of transfusion?

• inc absorption of iron out of plasma during erythropoesis
• cells have limited survival
• EPO signals GI tract to absorb more Fe3+

Thalassemia Minor

• micro/hypo
• poikilocytosis ***target cells***
• hepatosplenmegaly
• high Hgb A2, Hgb F