7. Hematologic Disorders Flashcards
Chronic, congenital, and hereditary hemolytic anemia almost exclusive in black individuals with episodic abdominal crises, jaundice, bone pain and dactylitis
Sickle Cell Anemia
Sickle Cell Anemia patients have a predisposition to
Salmonella osteomyelitis
Vascular compromise in sickle Cell Anemia results in
“H” or “fish” vertebra
Ischemia of the central endplate from
sickling crisis
bilateral hip pain showing bilateral
femoral head infarcts and osteonecrosis with subchondral collapse
of the superior femoral articular surface
Sickle Cell Anemia
Painful swellings of the hands and feet common in infancy, aggravated by cold weather.
Sickle Cell Dactylitis
Sickle Cell Dactylitis
Hand-Foot Syndrome
Individuals with sickle cell disease are predisposed
to _______ secondary to ischemic injury of both bone and GI tract
osteomyelitis
periostitis and osteopenia on radiograph
Osteomyelitis
The most sensitive study for sickle cell anemai is a
T1 fat saturation
study performed post gadolinium contrast
In SCA there will be ischemic changes to the ________ in
8-20% of patients
Femoral Head
Repeated episodes of splenic infarction causes the spleen to decrease in size
Autosplenectomy (from sickle cell)
Lytic processes are present at the metacarpals, along
with periostitis
Advanced Dactylitis from Sickle Cell Anemia
- H vertebra
- dactylitis
- splenomegaly
- hair on end
- osteonecrosis
- bone infarct
- osteomyelitis
- ## bone deformity
sickle cell anemia
Hereditary disorder of hemoglobin synthesis
Thalassemia
Cooley’s anemia, Mediterranean anemia
Retarded growth in thalassemia is due to
severe anemia
Hepatosplenomegaly in thalassemia is due to
severe hemolytic anemia
Mongoloid facies in thalassemia is due to
ineffective erythropoiesis and marrow hyperplasia
Maxillary overgrowth also called
rodent facies
- Expansion of medullary space
- Thinning of the cortices
- Expansion of bone diameter (Erlenmeyer Flask Deformity)
- Osteoporosis
- Resorption of fine trabeculae with thickening of the
remaining trabeculae - Honeycomb trabeculae
Radiographic Findings of Thalassemia due to marrow hyperplasia
Erlenmeyer flask deformity and Premature fusion of growth plates
Thalassemia Growth Disturbances
In thalassemia the hyperplasia of bone
marrow results in
cortical thinning, loss of tubulation, and coarse trabeculae
Thalassemia
The radiating spicules of bone (“Hair-On-End) is due to
erythroid hyperplasia
Coarsened trabecular
pattern (honeycomb) and
Osteoporosis with
thinning of the cortices
Thalassemia
squaring of the
metacarpals
Thalassemia
Lobulated soft tissue opacities are noted overlying the ribs anteriorly and posteriorly
Extramedullary
Hematopoiesis in Thalassemia
two MC bleeding disorders with skeletal abnormalities which are both sex linked recessive
- Classic Hemophilia (hemophilia A)
2. Christmas Disease (hemophilia B)
Females are carriers for Hemophilia gene but manifested
only in
males
Intraosseous Hemorrhage in Hemophilia leads to
hemophilic arthropathy
Hemophilic Arthropathy Resembles
Neurotrophic Arthropathy (Charcot Joint)
Repeated intraarticular bleeding and dense joint effusion from hemosiderin which increases density of soft tissues surrounding joint
Hemophilic Arthropathy
Intraosseous hemorrhage may result
in expansile lesions simulating neoplasm
Hemophilic
Pseudotumor
malignant disease of marrow with proliferating WBCs
Leukemia
MC childhood malignancy
Acute leukemia
- Osteopenia
- Radiolucent submetaphyseal
bands - Bone destruction
- Periostitis
leukemia
Lucent metaphyseal band in a child who complained of pain in the legs
Leukemic Lines