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Hematologic Disorders

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Alicia Yochum

on 12 November 2013

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Transcript of Hematologic Disorders

Hematologic Disorders
Alicia M. Yochum RN, D.C.
Diagnostic Imaging Resident
Logan College of Chiropractic

Thalassemia
Hemophilia
General Considerations
Clinical/Pathologic Features
Radiologic Features
Radiologic Features
Clinical Features
Pathologic Features
General Considerations
General Considerations
Clinical Features
Radiologic Features
Sickle Cell
Congenital
Black Population
Types
Hb SS- Homozygous, symptoms
Hb SA- Sickle Cell Trait
Hb SC- Sickle Cell Hemoglobin
Abnormal Molecular structure
Sickling Phenomenon: Normal RBC transformed to sickle shaped RBC under hypoxic conditions
Increased: Fragility, Viscosity, Stasis
Decreased: pH
Marrow Hyperplasia
Generalized osteopenia
Sparce coarse trabecular pattern (honeycomb)
Cortical thinning
Large Vascular channels
Wide Medullary cavity
Loss of diaphyseal constriction
Adult: General sclerotic pattern EXCEPT spine
Hb SS are symptomatic
Weakness
Pallor
Abdominal Crisis
Jaundice
Acute Bone Pain
Dactylitis (hand and foot syndrome)
World's most common hereditary disorder
Abnormal hemoglogin synthesis
Types
Major-Homozygous, Cooley's Anemia,
Minor- Heterozygous, Mediterranean Anemia
Intermedia
Arhtralgia 30%
Low back pain 25%
Locomotor System 60%
Scoliosis 40%
Labs: hypochromic microcytic anemia, reticulocytosis,
increased bilirubin
Physical: pallor, lethargy, retarded growth,
hepatosplenomegaly, rodent facies, inhibited sexual
development
Imbalance in globin chain production produced ineffective
hematopoesis, hemolysis and anemia
Overgrowth of marrow tissue

Young: entire skeleton because of red marrow predominance
Older: regression in peripheral skeleton to fatty/yellow
marrow

Extramedullary Hematopoesis: RBC production in places other than the marrow
Hepatosplenomegaly
Masses in posterior mediastinum
General Features
Coarsened trabecular pattern (honeycomb)
Cortical thinning
Expanded bone caliber
Osteoporosis
Vascular channel enlargement
Widened medullary cavity
Epiphysis spared frequently
Blood coagulation disorders
Types
Classic Hemophilia (A)- Factor VIII
Christmas Disease (B)- Factor IX

Female is carrier and male usually presents with disease
Spontaneous bleeding from minor injures
Hematomas in muscles
Intraosseous-destructive expansile lesion
Articular/hemarthrosis- permanent joint changes
Common
Bone
Growth abnormalities (epiphysis)
Osteoporosis
Pseudo-Tumors
Subchondral bone cysts
Joint
Dense joint effusion
Destruction
Erosions
Cardiac: Tricuspid Regurg-Cardiomegaly
Pulmonary: Hypertension
GI: Gallstones, Mesenteric Vascular Thrombosis
GU: Renal Failure
Brain: Infarction
Marrow Hyperplasia
Osteomyelitis
Infarction
Osteomyelitis

Salmonella is common 100x's the incidence
Infarction to the intestine predisposes the pathway for entry
Femur, Tibia, Fibula, Humerus, Radius, Ulna, Spine
Bilateral and Symmetric
Infarction
Cortical splitting (bone in bone)
Disturbed bone growth
Epiphyseal necrosis (humeral and femoral heads)
Linear diaphyseal periostitis
Organized metaphyseal medullary infarcts
Patchy areas of lucency and sclerosis
Soft tissue swelling
Femoral Head
Hb SS 8-20% ischemic changes on radiographs

Crescent Sign: subchondral fracture of weight bearing surface

Step Defect: sharp depression of articular surface

Bite Sign: flattening or irregularity of weight bearing surface
9yo with hip pain
14 yo pain in same hip
Same patient post core decompression
4 year follow up
Dull ache with ambulation
Decreased rotation
Special Features
Erlenmeyer flask deformity
Hair-on-end appearance (skull)
Persistent spine and pelvis changes with age
Paraspinal extramedullary hemopoiesis
Marrow Hyperplasia
Pathologic Features
Clinical Features
Absence of clotting factors
Repeated hemarthrosis precipitates:
Synovial proliferation(pannus)
Fibrosis
Bone erosion
Osteoporosis
Growth disturbance
Late cartilage degeneration
Uncommon
Chondrocalcinosis
Ectopic ossification
Fractures
Ischemic Necrosis
Septic Arthritis
Bones and Joints are affected
Joints- almost always affected in adult life
MC: Knee, ankle and elbow

Stages of Hemophilic Arthropathy:

I- Soft tissue swelling
II- Osteoporosis
III- Osseous lesions
IV- Cartilage destruction
V- Joint disorganization
Knee
Enlarged epiphysis
Widened intercondylar notch
Squared inferior patella
Ankle
Tibiotalar slant deformity
Elbow
Enlarged radial head
Widening of the radial and ulnar notches
Hypertrophy of radial head causing impingement (arrow)
Yochum T, Rowe L: Essentials of Skeletal Radiology, 3rd ed, Lippincott Williams and Wilkins 2005.

http://www.jaaos.org/content/12/4/234/F6.expansion

http://radiopaedia.org/cases/haemophilia-2

veria.com

emedicine.medscape.com

gait.aidi.udel.edu

studydroid.com

ajronline.org
References:
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