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Disorders of Heme & Iron Metabolism

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on 6 December 2013

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Transcript of Disorders of Heme & Iron Metabolism

Disorders of Heme & Iron Metabolism

Iron Overload
Joy P. Calayo, RMT, MSMT

Lab diagnosis of Hemochromatosis
Increased transferrin saturation (common screening test for hereditary hemochromatosis)

Transferrin saturation

Abnormal liver function tests

Genetic testing for mutations

Anemia of Chronic Inflammation (ACI)
Associated with chronic diseases (arthritis, chronic infections such as TB, HIV infection and malignancies

Feature: sideropenia but abundant iron stores

Etiology:
Hepcidin
Lactoferrin
Ferritin

Hepcidin

Low iron levels Low *Hepcidin *Inc. iron export to plasma by the enterocytes
Inc.iron release by macrophage



Inflammation Hepcidin increase *Decreased intestinal iron absorption
Dec. iron release by macrophage

Although there are abundant iron stores, they are still unavailable to the developing RBCs, because these iron stores are sequestered in the macrophages and hepatocytes. This sequestration reduces the amount of iron available to bacteria, therefore, bacterial cell death.
Lactoferrin

Inflammation Release of neutrophil lactoferrin in plasma

* Lactoferrin scavenges available iron at the expense of transferrin

When lactoferrin carries iron, it is bound to macrophage & liver cells that salvage iron
RBCs are deprived of iron because they do not have lactoferrin receptors


Ferritin

Inflammation Inc. ferritin

* Developing RBCs do not have ferritin receptors, thus, iron is unavailable for incorporation into the Hb.

Effects of Hepcidin, Lactoferrin & Ferritin
Show abundant iron stores in BM macrophages, but slowed release to developing RBCs

Iron stains will show iron stores in macrophages, but not in erythroblasts
Lab Diagnosis of ACI
*Mild anemia with Hb usually 9-11 g/dl, w/o reticulocytosis
*PBS: normocytic, normochromic; sometimes microcytic, hypochromic in patients with coexisting iron deficiency
*Leukocytosis, thrombocytosis, or both
See page 236, table 19-1
*BM: hypoproliferation of RBCs consistent w/ lack of reticulocytes in peripheral bld
Prussian blue stain: abundant Fe stores in macrophages but not in the RBC precursors
ACI vs IDA using STfRs
ACI= Normal
IDA= Increased
Sideroblastic Anemias
Hallmark: ringed sideroblasts shown in Prussian Blue stain
*Sideroblasts=iron-rich (in the mitochondria near the nucleus) nucleated RBC in the BM
Microcytic, hypochromic
See page 239, Box 19-1


Sideroblastic Anemia due to Pb poisoning. Note the punctate basophilic stippling of young RBCs & the microcytic, hypochromic mature RBCs.
Sideroblastic Anemia attributed to chronic alcoholism. Ringed sideroblasts are seen. The same can be observed in RA
Sideroblastic Anemias as diverse group of diseases
Hereditary
X-linked
Autosomal

Acquired
Primary SA (Refractory)
Secondary SA
Lead poisoning (drugs, heavy metals)= basophilic stippling (see page 224)
Porphyrias (see page 241-242)

Primary: Hereditary hemochromatosis

Secondary: to chronic anemias and their treatments

Etiology: deposition of hemosiderin in cells

Examples: as in repeated transfusions in Thalassemia patients
: as result of mutations

Increased hemosiderin imparts golden color of skin, liver cirrhosis or CA, bronzed diabetes, CHF
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