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Flashcards in Hematological Diseases Deck (45)
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1

what is the etiology of sickle cell anemia

chronic, congenital, and hereditary hemolytic disease

2

sickle cell anemia affects what population

black individuals

3

what are the symptoms of sickle cell anemia

episodic abdominal crisis
jaundice
bone pain
dactylitis
splenomegaly
osteonecrosis
bone deformities

4

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)

5

sickle cell anemia causes early death due to what

infection
visceral infarcts
cardiac decompensation

6

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

7

H vertebrae occurs in what conditions

sickle cell anemia
thalassemia
gauchers disease

8

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

9

sickle cell dactylitis is also called

hand foot syndrome

10

bone infarction is difficult to distinguish between what

whats more common

osteomyelitis

osteomyelitis > bone infarction

11

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

12

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

13

how do femoral heads appear in sickle cell anemia

femoral head ischemic changes in 8-20% of patients

AVN tends to be bilateral

14

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

15

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

16

advanced dactylitis due to sickle cell causes what radiographic findings

lytic process in 1st and 5th metacarpals
and
periostitis in the 3rd metacarpal

17

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

18

what does osteonecrosis look like in sickle cell

flattening of the femoral heads with a mixture of sclerosis and lucency

19

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

20

what is thalassemia also called

cooleys anemia
mediterranean anemia

21

what is the etiology of thalassemia

hereditary disorder of hemoglobin synthesis

22

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

23

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

24

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

25

in children, thalassemia affects __

the entire skeleton

26

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

27

what are the growth disturbances seen in thalassemia

complications

erlenmeyer flask deformity

premature fusion of growth plates


fracture, avn, hemochromatosis, arthropathy

28

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

29

what causes intramedullary hematopoiesis

what is this

thalassemia

masses noted in chest/ribs and paraspinal muscles

30

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