Disorders of Iron Kinetics and Heme Metabolism

Example of a physiological loss of iron

Menstruation

Example of pathological loss of iron

Chronic blood loss

How do we get Iron?

Sourced from food

Years when you need more iron in your system

Adolescent years

Substance that regulates ferroportin levels

Hepcidin

Substance that regulates iron into circulation

Ferroportin

In 1 day, approx. how much iron is lost

1mg

TRUE OR FALSE: Iron should not be increased nor decreased and should always be at a normal level

TRUE

What parameter must be measured to know the levels of iron in the body?

Ferritin

Disease associated with the mutation of the matriptase-2 protein

Celiac disease

Disease associated with malabsorption of iron through the enterocytes in the blood

Celiac disease

Chronic gastrointestinal bleeding from ulcers is associated with

H. pylori

Parasite to keep in mind for this topic

Hookworms

Hematocrit of acute blood loss

Normal

Hematocrit of chronic blood loss

Decreased

Stage 1

Storage Iron Depletion

Stage 2

Transpor Ion Depletion

Stage 3

Functional Iron Depletion

Lab test values top to bottom

Hemoglobin

Serum iron

TIBC

Ferritin

STFR

Hemoglobin content of retic

Stage 1 lab test values

Ferritin decreases

STFR increases

Stage 2 lab test values

Serum iron decreases

TIBC increases

Ferritin decreases

STFR increases

Hemoglobin content decreases

Stage 3 lab test values

Hemoglobin decreases

Serum iron decreases

TIBC Increases

Ferritin decreases

STFR Increases

Hemoglobin content decreases

Iron storage compartment

Ferritin

Iron transport compartment

Transferrin

Functional iron compartment

Hemoglobin

When do we usually do bone marrow examinations?

Identify different types of Leukemia

Symptom referred to as inflamed cracksat the corners of the mouth

Angular cheilosis

Symptom referred to as spooning of the fingernails

Koilonychia

Cells that become active in chronic infections

Macrocytes, T lymphocytes

Substances that increase in chronic infections

TNFa, INF-y, IL 1, IL 6

Diseases associated with TNFa

Chronic infection, Malignancy, Autoimmune dysregulation

Where is iron deposited?

In bone marrow

Where are macrophages deposited?

Liver

Serum iron of Iron Deficiency Anemia and Anemia of Chronic Inflammation

Significant decrease; Decrease

Serum Ferritin of Iron Deficiency Anemia and Anemia of Chronic Inflammation

Decrease; Normal-Increase

TIBC of Iron Deficiency Anemia and Anemia of Chronic Inflammation

Increase; Decrease

STFR of Iron Deficiency Anemia and Anemia of Chronic Inflammation

Increase; Normal

Saturation of Iron Deficiency Anemia and Anemia of Chronic Inflammation

Decrease; Decrease

Ferritin levels interpretation: Normal range

30-100ng/mL

Ferritin levels interpretation: <30ng/mL

Iron deficiency anemia

Ferritin levels interpretation: >100ng/mL

Anemia of inflammation

Ferritin levels interpretation: 30-100ng/mL ; >2

Anemia of chronic inflammation with true iron deficiency

Ferritin levels interpretation: 30-100ng/mL ; <1

Anemia of inflammation

Anemia associated with Hereditary, X-linked, Autosomal

Sideroblastic anemia

Secondary Sideroblastic anemia is caused by

Drugs, tumors, or fibrosis

Two diseases often similar

Sideroblastic anemia and Lead poisoning

Acquired Sideroblastic anemia specific causes

• Antitubercular drugs

• Chloramphenicol

• Alcohol

• Lead

• Chemotherapeutic drugs

Enzyme affected in Congenital Erythropoietic Porphyria (CEP) for Erythropoietic Porphyrias

Uroprohyrinogen III synthase deficiency

Enzyme affected in Erythropoietic Protoporphyria for Erythropoietic Porphyrias

Ferrochelatease deficiency

Enzyme affected in X-linked Erythropoietic Protoporphyria (XLEPP) for Erythropoietic Porphyrias

ALA-synthase 2

Only X-linked dominant among erythropoietic porphyrias

X-linked Erythropoietic Protoporphyria (XLEPP)

Only microcytic anemia where STFR increases

Iron deficiency anemia

Best way to differentiate Sideroblastic anemia and Lead poisoning

Presence of sideroblasts in BM or Basophilic stiplings for lead

Only microcytic anemia where Serum Ferritin is decreased

Iron deficiency anemia