HEMA LEC DISORDERS OF IRON AND HEME METABOLISM

Iron deficiency anemia

Anemia of Chronic inflammation

Sideroblastic anemias

Iron overload

Disorders of Heme and Iron metabolism (4)

Inadequate intake

Increased need

Impaired absorption

Chronic blood loss

Iron deficiency anemia common causes (4)

Stomach acid reducers

Medication that can inhibit iron absorption by decreasing gastric acidity

Functional iron deficiency

iron stores are adequate but the iron is not available to support normal erythropoiesis, what deficiency?

(iron is available but not utilizied)

1mg of iron

Approximately how much iron is lost from the body everyday?

Non-tropical sprue

Celiac disease

Tropical sprue

Bacteria

Occult blood

blood that cannot be seen in the stool but is positive on a fecal occult blood test

Chronic blood loss

Iron deficiency developed with repeated blood donations, chronic hemorrhage, or hemolysis

Paroxysmal nocturnal hemoglobinuria

Deficiency developed due to loss of iron in hemoglobin passed in the urine

Inadequate intake

most common cause of iron deficiency

Storage compartment

Transport compartment

Functional compartment

3 compartments of iron deficiency in order

Ferritin

Storage compartment of iron

Transferrin

Transport compartment of iron

Hemoglobin

Myoglobin

Cytochromes

Functional compartment of iron (3) (HMC)

Storage iron depletion

Stage 1 iron deficiency (asymptomatic)

Stage 1 of iron depletion

Hemoglobin: Normal

Serum iron: Normal

TIBC: Normal

Ferritin: Decreased

Which stage of iron depletion?

Transport iron depletion

Stage 2 iron depletion

Stage 2 of iron depletion

Hemoglobin: Normal

Serum iron: Decreased

TIBC: Increased

Ferritin: Decreased

Which stage of iron depletion?

Functional iron depletion

Stage 3 iron deficiency

Stage 3 of iron depletion

Hemoglobin: Decreased

Serum iron: Decreased

TIBC: Increased

Ferritin: Decreased

Which stage of iron depletion?

Total iron binding capacity

indirect measurement of transferrin

Frank anemia

symptomatic anemia, Stage 3, uses functional compartment mainly the Hb

Normal blood smear

Normal blood smear or iron deficiency anemia?

Central pallor enlarged

In iron deficiency anemia, the central pallor is ________________

Microcytic, hypochromic

Red blood cells of a person with IDA

Glossitis

Angular cheilosis

Koilonychia

Pagophagia

Symptoms of Iron deficiency anemia (4)

Glossitis

inflammation of tongue

Angular cheilosis

cracking of sides of lips

Koilonychia

spooning of fingernails

Pica

craving of non food items, severe IDA wherein mental is affected

Pagophagia

craving for ice

Menstruating women

IRON DEFICIENCY ANEMIA

Epidemiology

Group of individuals especially at high risk for IDA.

Their monthly loss of blood increases their routine need for iron

1200mg of iron

IRON DEFICIENCY ANEMIA

Epidemiology

If women of childbearing age do not receive proper iron supplementation, pregnancy and nursing can lead to a loss of nearly how much iron?

Iron supplementation

IRON DEFICIENCY ANEMIA

Epidemiology

Even though breast milk is a better source of iron than cow's milk, it is not a consistent source.

Therefore, what is recommended for breastfed infants after 6 months of age?

Men and postmenopausal women

IRON DEFICIENCY ANEMIA

Epidemiology

Iron deficiency is relatively rare in ____ and _____ because the body conserves iron so tenaciously, and these individuals lose only about 1 mg/day.

Necator americanus and Ancylostoma duodenale

IRON DEFICIENCY ANEMIA

Epidemiology

The worm attaches to the intestinal wall and literally sucks blood from gastric vessels. What are these worms? (2)

Trichuris trichiura

Schistosoma mansoni

Schistosoma haematobium

IRON DEFICIENCY ANEMIA

Epidemiology

Which parasites are the cause of heme iron lost from the body as a result of intestinal or urinary bleeding? (3)

Acidic environment

What kind of environment does iron like?

March hemoglobinuria

IRON DEFICIENCY ANEMIA

Epidemiology

Exercise induced hemoglobinuria, also called (?) develops when RBCs are hemolyzed by foot-pounding trauma and iron is lost as hemoglobin in the urine.

Screening

Diagnostic

Specialized

3 categories of testing in laboratory diagnosis:

No stainable iron

Bone marrow iron stain (Prussian blue reaction) in IDA

None

Sideroblasts in BM in IDA

Ferrous sulfate 3 times per day

If in stage 3 iron depletion (Iron deficiency anemia), what should you take and how many times per day?

65mg of elemental iron

1 tablet of ferrous sulfate contains how many milligrams?

Every morning when you wake up with an empty stomach

When should you take ferrous sulfate?

Anemia of chronic inflammation

most common anemia among hospitalized patients

Sideropenia

lack of iron in the blood

Sideropenia in the face of abundant iron stores

Central feature of anemia of chronic inflammation

Acute phase reactants

any substance that increases during inflammation

Hepcidin

Lactoferrin

Ferritin

3 APRs that are the culprits of ACI

Hepcidin

Prevents release of iron bound by ferritin - anemia of chronic disease.

a hormone produced by hepatocytes to regulate body iron levels, particularly absorption of iron in the intestine and release of iron from macrophages and hepatocytes.

Ferroportin

What does hepcidin degrade which exports iron from enterocytes into the blood, thus reducing the amount of iron absorbed into the blood from the intestine?

Lactoferrin

Prevents the phagocytized bacteria from using intracellular iron for their metabolic processes

Lactoferrin

Iron prefers (Lactoferrin/Transferrin)

9-11g/dL w/o reticulocytosis

Lab diagnosis for chronic inflammation. What is the Hb conc.?

Normocytic, normochromic

In ACI, red blood cells appears as ______ and _______

Leukocytosis and thrombocytosis

Indication of ACI

There are already iron present but iron is trapped in the cells

Transferrin will not be created in the liver if __________ and that's why TIBC is decreased in ACI

Treat first inflammation

Where to start when treating ACI?

Sideroblastic anemia

heme production is abnormal because iron and PPIX cannot combine

Microcytic, hypochromic

Appearance of red blood cells in sideroblastic anemias

Iron

In sideroblastic anemias, what is abundant in the BM?

Prussian blue stain

shows normoblasts with iron deposits in the mitochondria

Ringed sideroblasts

Hallmark of sideroblastic anemia

X-linked and Autosomal mutation

Hereditary sideroblastic anemia (2)

Primary and secondary

Acquired sideroblastic anemia (2)

Refractory

Primary sideroblastic anemia is _________ which means cause is unknown

Secondary sideroblastic anemia

sideroblastic anemia caused by drugs and bone marrow toxins

Antitubercular drugs

Chloramphenicol

Alcohol

Lead

Chemotherapeutic agents

5 causes of secondary sideroblastic anemia (ACALC)

Lead poisoning

a cause of secondary anemia which affects CNS and hematologic systems

______ interferes with porphyrin synthesis

Paints

Lead poisoning is often due to expose to:

Porphobilinogen synthase

Ferrochelatase

2 enzymes inhibited by lead poisoning in porphyrin synthesis

Hypoplasia

Sideroblastic anemia leads to incomplete development of the BM, otherwise known as:

Basophilic stippling

Classic finding in sideroblastic anemia

pyrimidine 5'-nucleotidase

Lead inhibits _____, an enzyme involved in the breakdown of ribosomal ribonucleic acid (RNA) in reticulocytes.

Porphyrias

impaired production of heme, porphyrin accumulates

leads to accumulation of products which will be deposited in body tissues

Single enzyme deficiency

Porphyrias is either acquired through lead poisoning, or passed down by:

Photosensitivity

Porphyrias in skin leads to:

Fluorescence of developing teeth and bones

Porphyrias in children leads to:

Down

Down or normal

Up

Down

Up

No stainable iron

None

Iron deficiency anemia lab diagnosis

Serum ferritin: ?

Serum iron: ?

TIBC: ?

Transferrin sat: ?

FEP/ZPP: ?

BM iron: ?

Sideroblasts in BM: ?

Up or normal

Down

Down

Down or normal

Up

Up or normal

None/Very few

Anemia of Chronic Inflammation lab diagnosis

Serum ferritin: ?

Serum iron: ?

TIBC: ?

Transferrin sat: ?

FEP/ZPP: ?

BM iron: ?

Sideroblasts in BM: ?

Up

Up

Down or normal

Up

Up

Up

Up (ring)

Sideroblastic anemia lab diagnosis

Serum ferritin: ?

Serum iron: ?

TIBC: ?

Transferrin sat: ?

FEP/ZPP: ?

BM iron: ?

Sideroblasts in BM: ?

Congenital erythropoietic porphyria

Deficiency of uroporphyrinogen III synthase.

Erythropoietic protoporphyria

Ferrochelatase deficiency

Iron overload

Body's rate of iron acquisition exceeds the rate of loss

Transusion-related hemosiderosis

Acquired iron overload through repeated transfusions

Hemochromatosis

Hereditary iron overload through gene mutations

Skin

Liver

Pancreas

3 organs commonly affected by iron overload

Golden color

Deposition of hemosiderin in skin gives the skin what color?

Cirrhosis-induced jaundice

Deposition of hemosiderin in liver

Diabetes mellitus

Deposition of hemosiderin in pancreas

Bronzed diabetes

Iron overload

Pathogenesis

The traditional characterization

of hemochromatosis is "____"

Serum iron concertation/TIBC x 100

Transferrin saturation formula

Withdrawal of blood by phlebotomy

IRON OVERLOAD Treatment