Hematological Diseases Flashcards
(45 cards)
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
