Hb Electrophoresis Patterns

Diagnosis Hb A Hb S Hb F
Normal ~99% 0% <1%
Sickle Cell Disease 0% 85-95% 5-15%
Sickle Cell Trait 50-60% 35-45% <2%
Hydroxyurea
Decreases freq of vasooclussive pain crises
Increases amt of HbF in circulation (> 15%)
This diluts # sickled cells in circulation which:
 Reduces vaso-occlusive episodes
 Need for transfusions
 Episodes of acute chest syndrome
- Major Adverse effects
 MYELOSUPPRESSION
o Neutropenia
o Thrombocytopenia
o Anemia

Acute Severe Anemia in Sickle Cell Disease

Cause Reticulocytes Key Features
- transient arrest of erythropoiesis
Aplastic Crisis DEC - 2’ to infection
 Ex. Parvovirus B19
Splenic - splenic vaso-occlusion  rapidly enlarging spleen
INC
Sequestration - occurs in children prior to autosplenectomy

Aplastic Crisis
Acute drop in Hb +
Low reticulocyte count (<1%) +
NO splenomegaly
#1 MC = Parvovirus B19
Cause
 < 15 yo
Sudden halt in RBC production
Characterized
Severe anemia (< 6)
by
WBCs and PLTs Normal
- Pallor
- Weakness
Presentation - Fatigue
- Functional Systolic Murmur
 Bc hyperdynamic blood flow
Treatment Transfusions
DDx of BONE PAIN in SICKLE CELL DISEASE
Cause Vaso-Occlusive Crisis Osteomyelitis Avascular Necrosis
Clinical Features - Acute, severe pain - Acute or SUBACUTE - Chronic,
- Pain > 1 site pain worsening pain
 Ex. Dactylitis - Focal pain at 1 site  Ex. Femoral
- +/- Low grade fever  Ex. Long bone head
- Erythema AND - Prolonged fever - Absence of fever
warmth - Erythema and - Absence of
- May be preceded by warmth warmth or
trigger - Positive Blood erythema
 Ex. dehydration Culture
About Earliest manifestation # 1 MCC = S aureus in Infarcytion of
of vaso-occlusion in ALL patients articular cartilage
SCD w SCD and bony surface
INC risk Salmonella after chronically
decreased blood
flow
Presentation 6 mo – 4 years Swelling Chronic,
Acute Fever progressive hip
SYMMETRIC SWELLING Pain pain w weight
of hands and feet Localized to 1 single bearing
bone
NOT bilateral
Radiographs INITIALLY
- reveal soft tissue
swelling
RECURRENT Episodes
- can reveal mottle
appearance of bone
Treatment Hydration
Pain control
 Sickle Cell Trait 3967
Hb AS
ABout MC Africa, Middle East, Mediteranean countries
AAs and Hispanic individs
Usually asymp
Clinical Features
No change in overall life expectancy
Normal Hb, reticulocyte count, RBC indices, and morphology
Lab Findings
HB Electrophoresis: HbA > HbS
PAINLESS Hematuria/Papillary necrosis
Splenic Infarction (esp at higher altitudes)
Complications Venous Thromboembolism
Priapism
Exertional Rhabdomyolysis
Painless Hematuria
- likely due to renal papillary ischemia or necrosis
- pap necrosis -> HEMATURIA
Renal Medullary CA
UTIs
Renal Complications
Inability to concentrate urine
- vasa rectae damage w inability to maintain concentrated medullary
gradient
Distal renal tubular acidosis
- tubular damage w impaired H+ secretion

Osteomyelitis
Kids w SICKLE CELL dz - Salmonella spp (enteritidis) - As above (anti staph ex. Ox,
(2/3) vanco)
- Staph Aureus (1/4) PLUS
- 3rd Gen Ceph
 Ceftriaxone
 Cefotaxime

Sickle Cell Anemia

B-globin mutation
Pathophysiology
AR
Hemolytic anemia
Clinical Features
Vaso-occlusive pain crises
Functional Asplenia
Other - intermittent sickling of RBCs in spleen leads to splenic infarction and
then fxnal asplenia
 HIGH risk of SEPSIS
- Strep pneumo #1 MCC Sepsis in SCD
 Usually from non-vaccne serotypes
- H flu
- N meningitidis
- DEC Hct
- INC Reticulocytes
Lab findings - Periph Blood Smear
 d RBCs
Howell-Jolly Bodies
MAINTENANCE
- vaccination
- prophylactic penicillin (until age 5)
- folic acid supplementation
Management
ACUTE PAIN CRISES
- hydration
- analgesia
- +/- Transfusion
#1 MCC Strokes in PEDS
STROKES
- MRI for confirmation
DIagnosis Hb Electrophoresis