April 12 2014 Virginia Harrod MD PhD Pediatric Hematology Oncology Overview Definition of anemia Review of CBC values Review of Hemoglobin Types of Anemia Microcytic macrocytic normocytic ID: 914378
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Slide1
AnemiaDell Children’s Medical CenterApril 12, 2014
Virginia Harrod, MD, PhD
Pediatric Hematology Oncology
Slide2Overview Definition of anemiaReview of CBC values
Review of Hemoglobin
Types of Anemia
Microcytic, macrocytic, normocytic
Diagnostic Evaluations and Workup
When to consult or refer to Hematology
Slide3Definition of Anemiaa condition in which the blood is deficient in red blood cells, in hemoglobin, or in total volume
http://www.merriam-webster.com/dictionary/anemia
Slide4What is in a CBC?WBC (white blood cell)
Differential (
neutr
,
lymphs
,
monos
,
eos
,
baso
)
Hemoglobin (RBC,
Hct
, MCV, MCHC)
Platelets (MPV)
Slide5What is missing from a CBC?Reticulocyte count
Gives an indication if the body “knows” something is wrong and if it can respond appropriately
Peripheral smear
Gives us insight into processes
Slide6Definition of Anemia
Age (years)
Hemoglobin (g/
dL
)
Mean (low limit)
Hematocrit
(%)
Mean (low limit)
0.5-1.9
12.5 (11.0)
37 (33)
2-412.5 (11.0)38 (34)5-713.0 (11.5)39 (35)8-1113.5 (12)40 (36)12-14 Female Male13.5 (12.0)14.0 (12.5)41 (36)43 (37)15-17 Female Male14.0 (12.0)15.0 (13.0)41 (36)46 (38)18-49 Female Male14.0 (12.0)16.0 (14.0)42 (37)47 (40)
Nathan and
Oski’s
Hematology of Infancy and Childhood 6
th
Ed., Ch10, pp409
Slide7HemoglobinStructure
Synthesis
Function
Structure of Hemoglobin
http://themedicalbiochemistrypage.org/hemoglobin-myoglobin.php
Slide8HemoglobinStructure
Synthesis
Function
a2b2
Structure of Hemoglobin
http://sickle.bwh.harvard.edu/hbsynthesis.html
Slide9HemoglobinStructureSynthesis
Function
Carry oxygen from lungs to body and carry CO2 from body to lungs
Found in Red Blood Cells
97% of the RBC is hemoglobin
Structure of Hemoglobin
en.wikipedia.org
American Society of Hematology Image Bank Online
Slide10Classifications of AnemiaBased on RBC size (MCV)
Microcytic (hypochromic)
Macrocytic
Normocytic (normochromic)
Based on Physiology
Production problem
Maturation problem
Destruction problem
Slide11Macrocytic Anemias
Megaloblastic
marrow changes
Vitamin B12 deficiency
Folic acid deficiency
Hereditary
oroticaciduria
Without
megaloblastic
changes
Liver disease
Hypothyroid
Bone marrow failureAplastic anemiaFanconi’s anemiaDiamond Blackfan anemiaMyelodysplasia
Slide12Macrocytic Anemias
Megaloblastic
marrow changes
Vitamin B12 deficiency
Usually associated with pernicious anemia
Loss of gastric parietal cells/intrinsic factor required for absorption of
vit
B12 in ileum.
Otherwise rare except some at risk groups:
Lower socioeconomic group, infants, elderly
Folic acid deficiency
Hereditary
oroticaciduria
Slide13Macrocytic Anemias
Megaloblastic
marrow changes
Vitamin B12 deficiency
Folic acid deficiency
Natural food sources
Dark green leafy veggies, fruit, nuts, beans, dairy, liver
01/1998 – US Government (FDA) – requiring supplementation
Breads, cereals, pastas, rice, grains
Uncommon (poor diet, alcoholism,
malabsorptive
d/o)
Am J Clin Nutr. 2013 Oct;98(4):1042-7. doi: 10.3945/ajcn.113.059683. Epub 2013 Aug 14.Near-elimination of folate-deficiency anemia by mandatory folic acid fortification in older US adults: Reasons for Geographic and Racial Differences in Stroke study 2003-2007.Odewole OA1, Williamson RS, Zakai NA, Berry RJ, Judd SE, Qi YP, Adedinsewo DA, Oakley GP Jr.
Slide14Macrocytic Anemias
Megaloblastic
marrow changes
Vitamin B12 deficiency
Folic acid deficiency
Hereditary
oroticaciduria
Without
megaloblastic
changes
Liver disease
Hypothyroid
Bone marrow failureAplastic anemiaFanconi’s anemiaDiamond Blackfan anemiaMyelodysplasia
Slide15Bone Marrow FailureFanconi’s
anemia
Diamond
Blackfan
anemia
Aplastic anemia
Myelodysplasia
Characteristics
Smaller than average
Usually presents before
teen years
Developmental defects (60%): thumb/armDevelopmental delaysGI disturbanceshttp://radiopaedia.org/articles/fanconi-anaemia
Slide16Bone Marrow Failure
Fanconi’s
anemia
Diamond
Blackfan
anemia
Aplastic anemia
Myelodysplasia
Characteristics
Craniofacial
malformations
Usually presents in infancyDevelopmental defects (60%): thumb/armCardiac, GU malformationsLow birth weight, growth delayshttp://dbafoundation.org/testimonials/DBA Foundation Support
Slide17Bone Marrow Failure
Fanconi’s
anemia
Diamond
Blackfan
anemia
Aplastic anemia
Myelodysplasia
Characteristics
Present similar to leukemia
Pancytopenia:
fever, bruising, preceding illnessesNormal MarrowAplastic Anemia
MDS
ASH
Image Bank Online
Slide18Microcytic AnemiasUsually represents impaired synthesis
Heme
Assembly line is interrupted
Iron
Lead
Globin
Thalassemias
Slide19Iron Absorption
New Human Physiology, 2
nd
Ed.,
Paulev
and
Zubieta
, 2004
Slide20Iron Absorption
New Human Physiology, 2
nd
Ed.,
Paulev
and
Zubieta
, 2004
Slide21Iron Absorption
New Human Physiology, 2
nd
Ed.,
Paulev
and
Zubieta
, 2004
Calcium ?
Slide22Iron Absorption
New Human Physiology, 2
nd
Ed.,
Paulev
and
Zubieta
, 2004
Calcium ?
Lead
tox
Slide23Iron Deficiency AnemiaLow MCVLow
Retic
Low ferritin
Common clinical scenarios:
Pale child, high milk intake, “picky eater”, clinically appears well (not fatigued, no fevers,
etc
)
Slide24Microcytic AnemiasUsually represents impaired synthesis
Heme
Iron
Lead
Globin
Thalassemias
ASH
Image Bank Online
Slide25ThalassemiaAlpha Decreased production of alpha globin chains
Beta
Decreased production of beta globin chains
Beta major (
b0),
beta
intermedia
(b+)
Typically autosomal recessive, but some not
Slide26Microcytic AnemiasUsually represents impaired synthesis
Heme
Iron
Lead
Globin
Thalassemias
Basophilic
stippling
ASH
Image Bank Online
Slide27Microcytic AnemiasUsually represents impaired synthesis
Heme
Iron
Lead
Globin
Thalassemias
ASH
Image Bank Online
Slide28Microcytic Anemia Workup
http://
www.aafp.org
/
afp
/2009/0815/p339.html
Mentzer
Index
Not reliable
Just measure ferritin
Slide29Normocytic anemias
Hemolysis (destruction)
External factor mediated
Antibodiy
mediated,
microangiomathic
, toxins, infectious,
hypersplenism
Membrane disorders of RBC
Spherocytosis,
elipotocytosis
, PNH (paroxysmal nocturnal
hemoglobinuria)Hemoglobinopathies (sickle cell disease)Hb S, Hb CEnzyme disordersG6PD, PK defHemorrhageSome production disorders:Red cell aplasia, TEC, marrow replacement (leukemia)
Slide30Antibody Mediated Auto-immune hemolytic anemia (AIHA)
Idiopathic or possibly infection, drugs,
etc
Warm and Cold antibodies
Diagnostic indicators:
Direct coombs
Reticulocyte counts
Microangiopathic
/infectious
Slide31Membrane DisordersSpherocytosis
Defect in
spectrin
Autosomal Dominant
Often present with “newborn jaundice” (first day of life)
Family history of
splenectomies
Slide32Membrane Disorders
ASH
Image Bank Online
Slide33Sickle Cell AnemiaSubstitution on 6
th
position beta chain (
val
for glutamic acid)
Hemoglobin forms long polymers under deoxygenated (hypoxic) states
Shortened RBC life span
Microvascular
obstruction/ischemia/infarction
10% African Americans carry trait, other populations too
Slide34Sickle Cell AnemiaLife threatening concerns
Splenic sequestration
Infection (encapsulated organisms, functional
asplenia
)
Other concerns
Aplastic crisis (
parvo
)
Vaso
-occlusive episodes (acute chest)
Slide35Sickle Cell Anemia
http://en.wikipedia.org/wiki/Sickle-
cell_disease
ASH online image bank
Slide36Anemia Workup Algorithm
Janus, et al., Evaluation of Anemia in Children, Am
Fam
Physician 2010 Jun 15; 81(12): 1462-1471
Slide37TreatmentsAll
anemias
Fix the problem
Deficiencies – give iron, vitamin,
etc
Sickle cell/
thal
Control symptoms
Hydroxyurea
Transfusions
Destruction issues
Splenectomy
, treat infectionMarrow failure/leukemiaImmune suppression, chemotherapy, marrow transplant
Slide38Consult Heme?
All “genetic” conditions
Iron deficiency anemia that doesn’t ‘get better’
Anemia resulting in transfusion or hospitalization
Phone consults are welcome for quick review and ‘next step’ thoughts
Slide39In Review Definition of anemiaReview of CBC values
Review of Hemoglobin
Types of Anemia
Microcytic, macrocytic, normocytic
Diagnostic Evaluations and Workup
When to consult or refer to Hematology