RBC
Qinshi Pan
Hematopoiesis: SC  RBC/WBC/platlets
-can increase 4-5 fold in 7-10 days
-usually in BM, can also be in liver, spleen, LN
RBCs in intravascular space (not stored)
O2 in kidneyEPO(peritubular caps)  SC (clusters of dark
cells) RBC 40% of blood
RBC: transport O2 and CO2 for 100-120 days
Aging: smaller, less elastic, spherical (removed in
spleen)
4 factos:
1. Normal vs impaired Hb
2. Acute vs Chronic loss
3. Extent of RBC volume loss
4. Indirect effects (Fe, spleen, etc)
Anemia: Hb or Hct <2.5 percentile after being
adjusted for age, sex, machine, altitude [therefore
clinical definition]
4 approaches: Etiology, RBC size/morphology,
frequency of occurrence, practical (Fe/B12 shot)
Clinical Measurements:
Hematocrit: RBC mass as % of blood volume
(high as baby, dips at few months then men:
38.8-50/ female: 34.9-44.5)
Hemoglobin: Total Hb/ volume blood
Hct/3=Hgb
Red Cell Distribution (RDV): measure of
anisocytosis
Low Hb and Hct (amt of RBC)= anemia
Low MCV (size)= microcytosis
Low MCH (color)= hypochromia
Hyperchromic does not exist
Haptoglobin: protein that binds plasma
circulating heme, in hemolysis taken out of
circulation (acute phase reactant) therefore
hemolysis= less haptoglobin
Hemolysis:
-Intravascular (sudden, catastrophic): destruction of
RBCs in BVs  schistocytes, anemia, Hb goes up,
Haptoglobin goes down, bilirubin increases,
hemoglobinemia, hemoglobinuria  Renal failure, DIC
-- Immunehemolytic RBC, C’ mediated, severe osmotic
stress
- Extravascular (slow): chronic, enhancement, amp, of
normal physiologic removal of RBCs  anemia,
elevated EPO, BM hyperplasia (rxn to decreased RBCs)
Normal
Rouleux
Echinocytes: may be
artifact from storing
Heinz body:
Denatured
hemoglobin
Anisocytosis (size)
Poikilocytosis (Shape)
Basophilic
Stippling: RNA
Stomacytosis
Pappenheimer
body: Iron
Spherocytosis
Teardrop
Elliptocytosis
Howell-Jolly:
DNA
Etiology:
Blood Loss
- Acute: trauma (normal hgb/hct)
10-15% loss in <1hr: S&S from defect in
vascular volume not lack of O2 carrying
capacity
20% loss in <1 hr: shock, postural hypoTN
- Chronic: GI/Gyn: gradual b/c BM can
increase production by 4-5 fold. S&S if
Hb<7
Increased Destruction of RBCs
- Intrinsic (Defective RBC):
-Hereditary: spherocytosis, G6PD
thalassemia, sickle cell anemia
- Acquired: paroxysmal nocturnal
hemoglobinuria
- Extrinsic (Normal RBC): AIHA,
mechanical trauma, Pb poisoning
Impaired Red Cell production
Intrinsic Defects
Peripheral Blood
Congested Spleen
Hereditary Spherocytosis (memH): usu AD
Ankryn, can also be spectrin. Unstable
membrane  shear stress in circulation 
loss of fragments
S&S: 6-9 Hb, spleno, cholelithiasis
Dx: osmotic fragility test Rx: Splenonectomy
Sequelae: parvo infection  aplastic crisis
Paroxysmal Nocturnal Hemoglobinuria (memA):
GPI anchor mut less CD55/59/C8BP  C’
med chronic hemolysis WITHOUT severe
hemoglobinuria acute at night
S&S:dark urine (morning), hemolysis and
hemoglobinuria
Ass: venous thrombosis, AML
Intrinsic Defects
G6PD Deficiency (metabH): XR 
can’t protect as well from free
radicals
GDPD A-: blacks, moderately reduced
GDPD Mediterranean: Middle east,
Fava bean  hemolytic episode
Both protect against Malaria.
Patho: oxidative stress  acute
intravascular hemolysis  anemia,
hemoglobinuria, hemoglobinemia 
removed extravascularily
PB: Heinz bodies and bite cells
Episodes are self-limited if stress is
removed
Sickle Cell Disorders (Qualitative): 6 position of
beta GluVal (Hetero: trait, Homo:Disease)
5-6mo (no HbF)  Sickle crisis (severe
bone/lung/liver/brain/penis pain)/
Autosplenectomy (esp with S pneumo and H.
Flu), also see gallstones, skin ulcers
50% to 50yo
Dx: Hb electrophoresis, DNA testing, HbS
solubility
Treat with hydroxyurea
Thalassemia (Quantitative):
2 beta and 2 alpha globin make 1 Hb
PB: anisocytosis, microcytosis, Crewcut radiograph
Alpha: SE Asia
Chrom 16 (a1-4):
-/a a/a: silent carrier, asymptomatic
-/- a/a or -/a -/a: asympt like Bminor
-/- -/a: HbH disease, severe like Binter
-/- -/-: Hydrobs Fetalis: die in utero
Beta: mediterranian
Chrom 11 (B1-2): B- (normal) B0/B+ (mutation)
B0/B0 or B+/B+ or B0/B+: Major, Severe, transfuse
Variable: Intermediate, severe but don’t need to
transfuse
B0/B-, B+/B-: Minor, asymptomatic
Spleen cong.
w/ sickle cells
Sickle
cell
Anemia caused by impaired RBC production
Extrinsic Defects
Decreased(Diminished) or Ineffective Erythropoiesis
Chemical Toxicity (Pb):
– Megaloblastic: B12 and folate deficiency
(nutritional)
Patho: Pb binds sulfhydryl groups in
ferrochelatase  displaces iron amd makes zinc
– Iron Deficiency (nutritional)
protoporphyrin/erytrocyte protoporphyrin
– Anemia of Chronic Disease (inhibits
PB: hypochro, microcytic anemia, basophilic
hematopoeisis)
stippling
– Aplastic anemia and pure red cell aplasia (destroy
Traumatic Hemolytic Anemia:
SCs)
Path: trauma  RBCs turn to fragments 
Marrow failure: replacement/displacement marrow
intravascular hemolysis
space
-Mechanical: prostethic cardiac valve
– Myelofibrosis, primary versus secondary
-Microangiopathic: TTP/HUS/DIC
– Space-occupying
Immunohemolytic:
• Hematologic malignancy
-Warm (IgG): idiopathic, SLE, drugs, CLL
• Metastatic non-hematologic malignancy
-Severe, life threatening extravascular
hemolysis (hard to treat b/c no compatible
blood)
-Cold (IgM):
-mycoplasma, mono (younger, abrupt, severe)
- idiopathic, Waldenstrom, CLL, DLBCL, splenic
lymphoma (older, mild, worse when cold)
-Cold (IgG): Cold hemolysin Hemolytic Anemia 
paroxysmal cold hemoclobinuria. RARE
Megaloblastic Anemia (B12 and Folate):
B12 Deficiency: elevated homocysteine and
methylmalonic acid (better sensitivity than
decreased serum cobalamin)
Pernicious Anemia: Patients have
antiparietal cell Ig  No IF  no Bwe
S&S: glossits, gastric atrophy,
neurological (can occur before B12
goes too low) cortex/lateral columns
Usually 60
Other causes: Nutritional, malsorption,
competitive uptake by
parasites, increased
requirement
(pregnancy/ hyperthyroidism)
PB: hypersegmented np with 6 lobes +
leukopenia, severe macrocytic anemia,
hyperbilirubinemia (extravascualr hemolysis)
BM: ineffective erythropoiesis
Dx: serum B12, MMA, homocysteine (also up in
B9 deficiency), Schillings test, pernicious needs
Ig test
Folic Acid: common in alcoholics, pregnant, and
those taking methotrexate.
Does not present with neurological symptoms
Iron Deficiency:
-insufficient intake (diet, malsorption, celiac, removal of
ileium (Chrons), secondary to systemic
- excessive loss (blood loss, hemodialysis)
- excessive use (growing children, pregnancy, lactation)
S&S: smooth tongue, spoon nail
PB: hypochromatic, microcytic RBC, low serum
ferritin/iron/transferrin saturation/stores, increased ironbinding capacity
Dx: Iron studies then look at reticulocytes to see if there’s
BM problem
BMBx: Prussian Blue stain for Iron
Anemia of Chronic Disease: Decreased EPO or can’t
move Fe to erythroid precursor, MCC in hospitalized
patients besides post-surgical and acute hemorrhage
-Chronic infections: osteomyelitis/endocarditis
-Immune (RA, Chrons)
-Malig: Hodkin, Carcinoma of lung/breast
High serum ferritin (Fe def has low)
Definitive: BM has prussian blue macrophage
Aplastic Anemia: idiopathic, chemo (alkylatin),
Chloramphenicol (ABX), Idiosyncratic, physical agents
(Hep, CMV, VZ, fanconi, telomerase)
FATTY BM- think back to WBC
Myelophthsic Anemia: like aplastic but instead of
messing with RBCs you have a mass in the bone
Polycythemia: RBC mass > 97.5 percentile
(remember to adjust for altitude, age, sex)
Erythrocytosis: Increased RBCs with no increase
in WBC or platelets
MCC: smoking, high alt., blue bloaters
Polycythemia rubra vera: see WBC notes (comes
with increase in WBC and platlets)
-- increased viscosity, CV complications,
thrombosis, hemmorrhage
Dx: phlebotomy
Blood Products
Platelets: replaces platlets
10,000/ml if stable, >20,000/ml if
unstable or > 50,000/ml in bleeding or
surgical patients
Frozen Plasma: replace coagulation factors
active bleeding or massive transfusions,
emergency reversal warfarin effect,
Cryoprecipitated antihemophilic factor
Replace fibrinogen and Factor XIII
Replace factor VIII or vWF (if factor VIII
and factor VIII/vWF
Cellular Therapy Products
White cells
Stem cells from bone marrow, cord blood,
or apheresis
Blood Testing
ABO and RhD
Hep B/C, HIV, HTLV, Syphilis
Acute hemolytic Anemia:
-Burning along vein, low back pain, chills, fever, shock,
increase pulse rate, DIC
-Lab: increased bilirubin and LDH
Febrile, Nonhemolytic Reaction
-Increase in temp by 1 degree, chills
Allergic Reaction:
-Urticaria, pruritis, facial/glottal edema
Anaphylactic Reaction:
-ANS dysregulation, dyspenea, pulmonary edema,
bronchospasm, hypotension
Transfusion Related Acute Lung Injury (TRALI)
-Acute respiratory distress in 6 hrs with hypoxemia and
bilateral pulmonary infiltrates
ABO Compatibility:
• O can only get O (universal donor)
• AB can get every type (universal acceptor/ blood
hogger)
• A can’t have B, B can’t have A