Iron deficiency anemia and porphyria

35 Pages·2016·0.93 MB·English

by  Wolff Fleur

Iron deficiency anemia and porphyrias
Fleur Wolff , Frédéric Cotton¹, Axelle Gilles²
¹
¹Department of clinical chemistry, Hôpital Erasme, ULB
²Department of hematology, Hôpital Erasme, ULB
BHS, November 2015
PLAN
1. Iron deficiency anemia
1.1. Introduction
1.2. Biochemical markers
1.3. Causes
1.4. IRIDA
1.5. Take home message
2. Porphyrias
2.1. Introduction
2.2. Type of porphyrias
2.3. Acute porphyrias
2.4. Cutaneous porphyrias
2.5. Take home message
OBJECTIVES
1. Iron deficiency anemia
 Utility of hepcidin determination in diagnosing IRIDA
Porphyrias
2.
 Overview of porphyrias
 Appropriate tests for the diagnose of porphyrias
1. IRON DEFICIENCY ANEMIA
1.1. Introduction
 Iron → multiple functions
 Absolute iron deficiency → body iron stores exhausted
 Prevalence
(Lopez A et al, Lancet 2015)
1. IRON DEFICIENCY ANEMIA
 Iron homeostasis:
PietrangeloA, N Engl J Med 2004
1. IRON DEFICIENCY ANEMIA
 Hepcidin: main regulator of systemic iron metabolism
→Controlled iron absorption
→Controlled recycling
→Controlled storage
Ganz T, Physiol Rev 2013
1. IRON DEFICIENCY ANEMIA
 Stimuli affecting hepcidin transcription
Polin V et al, Dig Liv Dis 2013
1. IRON DEFICIENCY ANEMIA
 Iron status and hepcidin regulation
 Regulation by circulating iron
and by iron stores
 In case of iron overload
Ferritin
TfS
Rischi G et al, 2015
1. IRON DEFICIENCY ANEMIA
 Iron status and hepcidin regulation
 In IDA
Ferritin
Rischi G et al, 2015
1. IRON DEFICIENCY ANEMIA
1.2. Biochemical parameters
Ferritin:
 <30ng/mL ; sensitivity of 98% and specificity of 92% for ID (Mast et al, 1998)
 Acute phase protein
 Hepatic cytolysis
Soluble receptor of transferrin sTfR (sTfR/log ferritin):
 Not affected by inflammation
 Concentration raised with increased erythropoiesis
 Absence of standardized cut-offs