Hematological Diseases Flashcards
what is the etiology of sickle cell anemia
chronic, congenital, and hereditary hemolytic disease
sickle cell anemia affects what population
black individuals
what are the symptoms of sickle cell anemia
episodic abdominal crisis jaundice bone pain dactylitis splenomegaly osteonecrosis bone deformities
sickle cell anemia predisposes the patient to what
salmonella osteomyelitis
secondary to ischemic injury of bone and GI tract - high incidence of infarct beds with GI flora
sickle cell anemia causes early death due to what
infection
visceral infarcts
cardiac decompensation
vascular compromise in sickle cell anemia causes an __ appearance
what is this appearance due to
H vertebrae
or
fish vertebrae
ischemia/osteonecrosis of the central endplate due to vascular compromise - less blood vessels in the middle so the middle thins quicker
H vertebrae occurs in what conditions
sickle cell anemia
thalassemia
gauchers disease
sickle cell anemia dactylitis common occurs when? what is dactylitis? what aggravates the sludging?
during infancy
painful swellings of hands and feet due to infarction or infection
cold weather aggravates the sludging
sickle cell dactylitis is also called
hand foot syndrome
bone infarction is difficult to distinguish between what
whats more common
osteomyelitis
osteomyelitis > bone infarction
what are the xray findings of bone infarction caused by sickle cell?
what is the preferred method to visualize these bone infarcts?
periostitis
osteopenia
patchy subchondral sclerosis indicates prior infarcts
MR
what is preferred imaging for bone infarction due to sickle cell?
high signal on what?
what is the most sensitive?
MR
high signal on T2 and SPIR - spectral presaturation inversion recovery
MR T1 with contrast**
- most sensitive
how do femoral heads appear in sickle cell anemia
femoral head ischemic changes in 8-20% of patients
AVN tends to be bilateral
repeated episodes of splenic infarctions occur in what disease
what does this do to the spleen size
whats the end stage
sickle cell anemia
decrease in size - could be normal but nonfunctional
end stage - spleen contains perivascular fibrosis with deposition of hemosiderin and calcium
the end stage of sickle cell anemia ends with the spleen becoming perivascular fibrosis with deposition of __ and __ that has been termed the process of __
hemosiderin and calcium
autosplenoectomy
advanced dactylitis due to sickle cell causes what radiographic findings
lytic process in 1st and 5th metacarpals
and
periostitis in the 3rd metacarpal
hair on end appearance is seen in what condition
what causes this
sickle cell anemia and thalassemia
hair on end appearance - in the skull
expanded marrow cavity due to marrow hyperplasia - trabeculae are oriented perpendicular giving it its appearance
what does osteonecrosis look like in sickle cell
flattening of the femoral heads with a mixture of sclerosis and lucency
what are the bone deformities seen in sickle cell anemia
what are these bone deformities due to
shortening of 3rd and 4th metacarpals and phalanges
early fusion of growth plates
osteonecrosis in infancy
what is thalassemia also called
cooleys anemia
mediterranean anemia
what is the etiology of thalassemia
hereditary disorder of hemoglobin synthesis
what are the 3 forms of thalassemia
which one is of greatest severity and worst prognosis
major ** - worst prognosis - death in infancy is common
minor
intermedia
in less severe forms of thalassemia, what is the typical end road in disease
how does it occur
repeated transfusions result in hemochromatosis and heart failure
death by 2nd or 3rd decade
dilated and restrictive cardiomyopathy due to iron deposits in cardiac tissue
what are common characteristics of thalassemia
retarded growth - due to anemia
hepatosplenomegaly - due to hemolytic anemia
mongoloid facies - ineffective erythropoiesis and marrow hyperplasia
maxillary overgrowth - rodent facies
inhibited sexual development
in children, thalassemia affects __
the entire skeleton
what are the radiographic findings of thalassemia
expansion of medullary space due to marrow hyperplasia
thinning of cortices and loss of tubulation
expansion of bone diameter - erlenmeyer flask deformity
osteoporosis
resorption of fine trabeculae with thickening of remaining
honeycomb trabeculae - coarse trabeculae pattern
squaring of metacarpals
what are the growth disturbances seen in thalassemia
complications
erlenmeyer flask deformity
premature fusion of growth plates
fracture, avn, hemochromatosis, arthropathy
how do you tell the difference between sickle cell and thalassemia on xray skull
both have hair on end appearance
thalassemia has obliterated sinuses and rodent facies - due to stem cells in sinus converting to marrow to produce new rbcs
what causes intramedullary hematopoiesis
what is this
thalassemia
masses noted in chest/ribs and paraspinal muscles
what is the clinical presentation of thalassemia
appear first 2 years of life
fatigue
pale skin or jaundice
protruding abdomen with enlarged spleen and liver
what is hemophilia
deficiency in specific clotting factors - coagulopathies
what are the 2 most common bleeding disorders with skeletal abnormalities
(classic) hemophilia A
(christmas) hemophilia B
what is the etiology of hemophilia
who does the disease manifest in
sex linked recessive
females are carriers for the gene
hemophilia manifests only in males
when do hemorrhages occur in hemophilia
hemorrhages occur spontaneously or after minor trauma that can cause skeletal changes within or around joints/bones
what is a intraosseous hemorrhage
where does it most commonly occur
what joints is this seen in
destructive expansile lesion - pseudotumor of hemophilia
MC in ilium and femur
weight bearing joints - knees hips
what are some characteristics of hemophilic arthropathy
repeated intra-articular bleeding that leads to joint effusion from hemosiderin/iron deposits - increases density of soft tissues around joint
intraosseous hemorrhage
osteoporosis and epiphyseal growth from hyperemia
subchondral cysts
bone erosion
widening of intercondylar notch from cartilage destruction (pannus)
what does hemophilia arthropathy resemble
charcotjoint
neurotrophic arthropathy
what can intraosseous hemorrhage lead to
neoplasm in 1-2% of hemophiliacs
CT of hemophilia pseudotumor will appear like what
large expansile lesion with inhomogeneous internal attenuation
what is leukemia
myeloproliferative malignant disease of the marrow
proliferating WBCs
what are the different populations and types of leukemia
childhood - acute
adult - chronic
what is the most common childhood malignancy
acute leukemia
what are the radiological findings of leukemia
osteopenia
radiolucent submetaphyseal bands - leukemic lines
bone destruction
periostitis
what is the clinical presentation associated with leukemic lines
pain in the legs or refusal to walk associated with leukemic lines
when children are over the age of __, bilateral lucent bands called __ are indicative of leukemia
2
leukemic lines
