Anemia

Erika Franz-O’Neal
PGY-2 Pediatric Resident
Definition of Anemia
• Hgb < 13.5 within the first month of life
• Anemia worse than what you would expect with physiologic
nadir (hgb <9)
• After 1 year of age, Varies with age and ethnicity

Due to copyright some images have been removed.

See normal values first year of life in healthy term infants

UpToDate: Approach to the child with anemia.
Patient Z
• 4 yo previously healthy female presents to the ED with facial
swelling and fatigue. She is found to have a Hgb of 6.
Labs: CBC and Other
• CBC, what’s important? • Other labs?
• MCV: represents the mean • Retic count
volume of the individual RBCs • ARC= percent reticulocytes x
• Microcytic, normocytic, RBC/L
macrocytic • Normal high is 100 K/mcL
• RDW: measure of the variability • Blood smear
in size (anisocytosis)
• MCHC: mean corpuscular hgb
concentration
(MCHC=HGB/HCT)
• hyperchromic, normochromic,
hypochromic anemia
• WBC, Platelets
• High WBC?
• High Platelets? Low platelets?
• Pancytopenia?
Patient Z labs
• CBC:
• Hgb 6, MCV 84, normal RDW and MCHC
• Platelets 150,000 (borderline)
• WBC normal
• Retic count 9%, ARC of 211 (H) ….Normocytic anemia with
• Blood smear: pending… reticulocytosis

• What else do you want to know from history/physical?

• Blood loss (no obvious signs)
• Hemolysis (no jaundice, dark urine, or hepatosplenomegaly)
• Medication (no meds)

• Differential Diagnosis?
• Additional Labs?
Labs
• Frac Bilirubin: total bili 3.0, indirect bili 2.2
• LDH: >1000
• Haptoglobin: <8 (not reliable in infants < 18 months)
• Plasma free hgb: not done
• CMP:
• Na 138
• K 4.5
• Cr .7
• BUN 26
• LFTS WNL
• Protein 6 (low)
• UA: 3+ protein, large hgb
• UR protein/Cr ratio 5.8 mg/mg
• No diarrhea, could not get GI PCR
• Blood smear: normocytic, normochromic anemia with frequent
schistocytes
Kidney biopsy
THROMBOTIC MICROANGIOPATHY.
ACUTE TUBULAR INJURY.
FOCAL TUBULAR ATROPHY AND SLIGHT TO MODERATE
INTERSTITIAL FIBROSIS.
Patient Z
• Most likely typical HUS
• Did have diarrheal illness that resolved 1 week before
hospitalization
• Could not rule out atypical HUS so will need long term follow
up
• Platelets were confusing so obtained renal biopsy
• Got RBC transfusion and did great! Went on vacation 2 days
later after 3 days in the hospital
Due to copyright images were removed. See overview of
hemolytic anemias in children at UpToDate.

CAUSES OF HEMOLYTIC ANEMIA IN

CHILDREN
HUS
• Microangiopathic hemolytic anemia, thrombocytopenia, AKI
• Etiologies
• Typical/Primary (diarrhea-positive)
• Infection: STEC (90%), Strep penumo, HIV
• Drug toxicity (cancer patients or solid organ transplant recipients)
• Rarely in SLE, pregnancy

• Atypical/Secondary (diarrhea negative)

• Complement mediated (5-10%): gene mutations and antibodies to
complement factor H
• Mutations in coagulation pathway
• Errors of Vit B12 metabolism leading to homocystinuria and
merthylmalonic acidemia
STEC-HUS Pathophysiology
• Shiga toxin has two subunits, A and B
• Subunit B binds to Gb3 receptors which are highly expresses
on kidney tubular cells, brain and gut endothelial cells
• Endocytosed and inactivates ribosomes causing cell death
STEC-HUS
• Clinical diagnosis
• Microangiopathic hemolytic anemia with hgb < 8 with a negative
coombs test and peripheral smear showing schistocytes
• Thrombocytopenia below 140,000, no correlation with severity of
disease
• AKI
• Ranges from proteinuria and hematuria to severe renal failure and
oligoanuria.
• Renal failure requiring dialysis occurs in about 50% of cases
• Hypertension is common
• Other organ involvement
• Central nervous system (significant mortality), GI tract, pancreas,
liver
• Shiga-toxin genes in stool
Differential Diagnosis
• DIC
• Abnormal coag studies and D-dimer
• Seriously ill
• TTP
• Present at birth
• Low ADAMTS13 activity
• Systemic vasculitis
• Arthralgia, rash
Treatment Recommendations
• Mainly Supportive
• Avoid antibiotics or antimotility agents
• Early IV hydration to avoid renal hypoperfusion
• RBC transfusions (goal hgb of 8-9)
• Platelet transfusion only if actively bleeding (HLA-antibodies)
• Monitor for electrolyte and metabolic derangements
• Dialysis
• Manage hypertension, calcium channel blockers in acute
phase
• If severe neurologic involvement, can use Eculizumab (binds to
complement factor C5 to block complement activation)
• Plasma exchange debated
Prognosis/Follow-up
• Mortality rate below 5%
• Risk of renal failure 20 years after recovery warrants long term
follow up
• In renal transplantation, recurrence of HUS is rare
• Yearly evaluations with BP measurements, UA, and serum Cr
• ACEI after the acute phase in patients with renal sequelae for
renoprotective effect
THANK YOU!
• Resources
• UpToDate
• Salvadori, M., & Bertoni, E. (2013). Update on hemolytic uremic
syndrome: Diagnostic and therapeutic recommendations. World
journal of nephrology, 2(3), 56-76.