Anemia and Cell Histology Vocabulary

Vocabulary flashcards related to Anemia and Cell Histology

Hemoglobin A

Normal adult hemoglobin, consisting of 2 alpha chains and 2 beta chains, making up 98% of adult hemoglobin.

Hgb A2

Hemoglobin consisting of 2 alpha and 2 delta chains, making up 2% of adult hemoglobin.

Hgb F

Fetal hemoglobin, consisting of 2 alpha and 2 gamma chains.

present to 4-6 months of life.

allows transfer of O2 from maternal to fetal in utero

present in thalassemia major

Bohr Effect

The effect where O2 dissociates from hemoglobin more readily when the pH is lowered (acidic conditions).

increased O2 demand due to increased metabolism

increased metabolism → more CO2 → intracellular pH lowers resulting in enhanced oxygen release from hemoglobin, facilitating oxygen delivery to tissues.

Left Shift

Hgb-O2 affinity increases

lower co2

higher pH

low temp

Right Shift

Hgb-O2 affinity decreases

higher co2

lower pH

higher temp

acidosis

sickling can occur

EPO (erythropoietin)

Kidneys produce this to stimulate RBC production.

RBC

Life cycle of ~120 days; lack nucleus and mitochondria

Globin and Heme

Macrophages break down RBC into

Haptoglobin

Protein that binds to free hemoglobin, and then the complex is removed by RES.

hemolysis causes increased hemoglobin → more haptoglobin binding → decrease free haptoglobin levels

Ferroportin

Allows Fe export out of macrophages and into circulation.

Hepcidin

Blocks ferroportin so Fe stays within macrophage.

high wen iron stores are high and during inflammation

low during IDA

prevents iron from being used

Transferrin

Binds to and transports iron throughout circulation.

TIBC

Total Iron Binding Capacity – “how many seats are available on the bus” Indirect measurement of transferrin

% Saturated

How many seats are taken on the bus

UIBC

“How many seats are remaining on the bus” indirect measure of how many transferrin are not bound to iron

Anemia

Anemia: reduction of RBC, hemoglobin (hgb) or hematocrit (HCT)

MCV

Mean corpuscular volume – the size of the RBC

MCH

Mean corpuscular hemoglobin – average hemoglobin content (color of the RBC)

RDW

Red cell distribution width – variation in RBC size

Reticulocyte count

Immature RBCs - rate of RBC production

Normocytic

MCV 80-100

Microcytic

MCV <80

defect in cellular hemoglobin synthesis

mostly iron deficiency

thalassemia, sideroblastic

Macrocytic

MCV >100

asynchronous maturation of nuclear chromatin

rate of cell division is reduced

B12,folate, copper deficiency

myelodysplastic syndrome

HPLC (High performance liquid chromatography)

genetic newborn screening for common hemoglobinopathies

Thalassemia

Reduced Alpha chains – alpha thalassemia; in utero or at birth

Sickle Cell

Valine substituted for glutamic acid on beta chains; 4-6 months

Hemolysis

Breakdown of RBC – many causes including genetic, acquire, infectious

Intravascular

Within blood vessels

Extravascular

Within tissue. Typically RES (spleen) causing sequestration

Anisocytosis

Increase in RBC that vary in size

poikilocytosis

Increase in RBC that vary in shape.

Reticulocytosis

Increase in RBC production.

Band cell

Immature Neutrophil

hemoglobin structure

4 heme molecules & 4 iron

how many hemoglobin per RBC?

280 million

what hemoglobin is present in thalassemia?

A2

where do all cells come from?

pleuripotent cell

erythropoiesis fetal

liver, spleen, lymph nodes

erythropoiesis infant

bone marrow of all bones

erythropoiesis adult

ribs, sternum, vertebrae, pelvic bones

liver function in erythropoiesis

stores elements and synthesize proteins necessary for formation of RBC, EPO production, RBC breakdown

Cirrhosis/Hepatitis/Inflammation à decreased synthesis of proteins for RBC production, reduced storage ability or sequestered stores, decreased ability to process bilirubin

GI function in erythropoiesis

intrinsic factor, absorption of necessary components of RBC production: iron, vitamins, amino acids

gastric bypass à gastric cells that produced intrinsic factor do not encounter food à inability to absorb B12 à pernicious anemia

inflammatory disease à inability to adequately absorb nutrients à ineffective RBC production

lungs in erythropoiesis

oxygenation can influence EPO production

hypoxia stimulates epo and RBC production

lung disease (COPD, etc) hypoxic environment increased EPO and increased RBC

High RBC & High hematocrit = High viscosity and High risk for stroke

What does glucose oxidation capacity do?

allows ATP production

why are RBCs phagocytized by macrophages in the reticuloendothelial system

so they can fit through the small capillaries in the spleen

Iron + transferrin

transported to the liver for storage

biliverdin

oxidized and reduced to bilirubin by biliverden reductase

hemolytic anemia

bilirubin + albumin

transported to liver for conjugation

what happens to the spleen after it has been overworked

it dies

ferritin

protein that contains iron in organs, can store 4500 Fe atoms

primarily stored in the liver

why is ferroportin increased during IDA

to maximize iron absorption and mobilization

type A blood

a antigen

compatible with A & O

type B blood

B antigen

compatible with B, O blood

AB blood

A and B antigens

compatible with A, b, ab, and O blood (Universal recipient)

O blood

no antigens

only compatible with O blood (universal donor)

iron levels in thalassemia

generally fine but hemoglobin chains are abnormal

High MCV

B12 or folate deficiency

low MCV

iron deficiency or thalassemia

RDW in anemia

high variation in cell size

labs in IDA

Low MCV

Low Iron

high TIBC

Low ferritin

low Transferrin

liver synthesizes more transferrin = increase TIBC

labs in thalassemia

Low MCV

High Iron

Low TIBC

High Ferritin

High Transferrin

labs for anemia of chronic disease

normal to low MCV

low iron

low TIBC

normal to high ferritin

normal to low transferrin

liver synthesize less transferrin

what should you do first for anemia

history and physical

what should you do second for anemia

the work up

CMP

peripheral blood smear

iron panel

vitamin

what if a CBC confirms microcytic anemia

order iron

what if a cbc confirms macrocytic anemia

order b12

IDA

B12 deficiency

IDA

hemolysis

row 1

normal

row 2

thalassemia trait

row 3

thalassemia major

row 4

Sickle cell trait

row 5

sickle cell anemia

what is IDA in older adults

colon cancer until proven otherwise

sideroblastic

Fe not incorporated normally

hgb S

valine substituted no beta chains

less soluble

Hgb C

lysine is substituted

Hgb D

glutamine is substituted

hgb H

beta chain tetramers

alpha thalassemia type where 3-4 alpha chains are missing

what causes sickling

1.  Exercise, etc → Increased O2 demand → localized or systemic effect

2.  Infection → Incr. metab → anaerob metab → lactic acid in plasma

Plasma pH decreases → RBC’s unload O2 more readily → sickling

3.  Fever → Increased metab → O2 unloads → sickling

Prolonged Fever/Infection → anaerobic metab → lactic acid → decr. pH → sickling

4.If the patient has another medical problem with acidosis risk – ie DM

when and what should SC patients be vaccinated

get more & more frequent vaccines

pneumococcus and meningococcus

effects of SC

renal failure

delayed puberty

skeletal abnormalities

functional asplenia

cholelithiasis (stones)

pulmonary HTN

hilar region

where vessels go to lung

dactylitis

vaso-occlusive crises that cause swelling in the hands and feet. repeated episodes of dactylitis will lead to a mottled appearance of the small bones

chronic osteomyelitis

happens in long bones

tibial infarctions

infection of infarcted bones

common organisms: salmonella species

frontal bossing

large and pronounced forehead

in any childhood anemia

sickle cell trait

heterozygous for HgB S

Hgb is still produced

rare sickling events

cells only sickle under severe hypoxic stress

adaptive for malaria

Thalassemia facial features

saddle nose

frontal bossing

maxillary expansion

A2 electophoresis

hgb F for beta thallassemia

consequences of hemolysis

Anemia → decreased O2 delivery →tissue ischemia

Anemia → decreased O2 delivery → increased cardiac demand

increased rbc synthesis→follate and Fe use→ deficiency

need for transfusions leading to fe overload

abnormal erythropoiesis

increase RBC production

howell jolly bodies

nuclear reminants not removed due to splenic dysfunction

functional asplenia

why could a patient have anemia and a normal reticulocyte count

ther is a problem with erythrocyte production

what effects size variation

MCV

what effects hemoglobin distribution

MCH

esr and rouleaux

high