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Radiology Tumors > Hematological Diseases > Flashcards

Hematological Diseases Flashcards

1
Q

what is the etiology of sickle cell anemia

A

chronic, congenital, and hereditary hemolytic disease

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2
Q

sickle cell anemia affects what population

A

black individuals

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3
Q

what are the symptoms of sickle cell anemia

A 
episodic abdominal crisis 
jaundice 
bone pain 
dactylitis 
splenomegaly 
osteonecrosis 
bone deformities
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4
Q

sickle cell anemia predisposes the patient to what

A

salmonella osteomyelitis

secondary to ischemic injury of bone and GI tract - high incidence of infarct beds with GI flora

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5
Q

sickle cell anemia causes early death due to what

A

infection
visceral infarcts
cardiac decompensation

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6
Q

vascular compromise in sickle cell anemia causes an __ appearance

what is this appearance due to

A

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

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7
Q

H vertebrae occurs in what conditions

A

sickle cell anemia
thalassemia
gauchers disease

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8
Q

sickle cell anemia dactylitis common occurs when? what is dactylitis? what aggravates the sludging?

A

during infancy

painful swellings of hands and feet due to infarction or infection

cold weather aggravates the sludging

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9
Q

sickle cell dactylitis is also called

A

hand foot syndrome

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10
Q

bone infarction is difficult to distinguish between what

whats more common

A

osteomyelitis

osteomyelitis > bone infarction

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11
Q

what are the xray findings of bone infarction caused by sickle cell?

what is the preferred method to visualize these bone infarcts?

A

periostitis
osteopenia

patchy subchondral sclerosis indicates prior infarcts

MR

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12
Q

what is preferred imaging for bone infarction due to sickle cell?

high signal on what?

what is the most sensitive?

A

MR

high signal on T2 and SPIR - spectral presaturation inversion recovery

MR T1 with contrast**
- most sensitive

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13
Q

how do femoral heads appear in sickle cell anemia

A

femoral head ischemic changes in 8-20% of patients

AVN tends to be bilateral

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14
Q

repeated episodes of splenic infarctions occur in what disease

what does this do to the spleen size

whats the end stage

A

sickle cell anemia

decrease in size - could be normal but nonfunctional

end stage - spleen contains perivascular fibrosis with deposition of hemosiderin and calcium

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15
Q

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 __

A

hemosiderin and calcium

autosplenoectomy

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16
Q

advanced dactylitis due to sickle cell causes what radiographic findings

A

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

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17
Q

hair on end appearance is seen in what condition

what causes this

A

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

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18
Q

what does osteonecrosis look like in sickle cell

A

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

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19
Q

what are the bone deformities seen in sickle cell anemia

what are these bone deformities due to

A

shortening of 3rd and 4th metacarpals and phalanges

early fusion of growth plates

osteonecrosis in infancy

20
Q

what is thalassemia also called

A

cooleys anemia

mediterranean anemia

21
Q

what is the etiology of thalassemia

A

hereditary disorder of hemoglobin synthesis

22
Q

what are the 3 forms of thalassemia

which one is of greatest severity and worst prognosis

A

major ** - worst prognosis - death in infancy is common

minor

intermedia

23
Q

in less severe forms of thalassemia, what is the typical end road in disease

how does it occur

A

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
Q

what are common characteristics of thalassemia

A

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
Q

in children, thalassemia affects __

A

the entire skeleton

26
Q

what are the radiographic findings of thalassemia

A

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
Q

what are the growth disturbances seen in thalassemia

complications

A

erlenmeyer flask deformity

premature fusion of growth plates

fracture, avn, hemochromatosis, arthropathy

28
Q

how do you tell the difference between sickle cell and thalassemia on xray skull

A

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
Q

what causes intramedullary hematopoiesis

what is this

A

thalassemia

masses noted in chest/ribs and paraspinal muscles

30
Q

what is the clinical presentation of thalassemia

A

appear first 2 years of life

fatigue
pale skin or jaundice
protruding abdomen with enlarged spleen and liver

31
Q

what is hemophilia

A

deficiency in specific clotting factors - coagulopathies

32
Q

what are the 2 most common bleeding disorders with skeletal abnormalities

A

(classic) hemophilia A

(christmas) hemophilia B

33
Q

what is the etiology of hemophilia

who does the disease manifest in

A

sex linked recessive

females are carriers for the gene

hemophilia manifests only in males

34
Q

when do hemorrhages occur in hemophilia

A

hemorrhages occur spontaneously or after minor trauma that can cause skeletal changes within or around joints/bones

35
Q

what is a intraosseous hemorrhage

where does it most commonly occur

what joints is this seen in

A

destructive expansile lesion - pseudotumor of hemophilia

MC in ilium and femur

weight bearing joints - knees hips

36
Q

what are some characteristics of hemophilic arthropathy

A

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)

37
Q

what does hemophilia arthropathy resemble

A

charcotjoint

neurotrophic arthropathy

38
Q

what can intraosseous hemorrhage lead to

A

neoplasm in 1-2% of hemophiliacs

39
Q

CT of hemophilia pseudotumor will appear like what

A

large expansile lesion with inhomogeneous internal attenuation

40
Q

what is leukemia

A

myeloproliferative malignant disease of the marrow

proliferating WBCs

41
Q

what are the different populations and types of leukemia

A

childhood - acute

adult - chronic

42
Q

what is the most common childhood malignancy

A

acute leukemia

43
Q

what are the radiological findings of leukemia

A

osteopenia

radiolucent submetaphyseal bands - leukemic lines

bone destruction

periostitis

44
Q

what is the clinical presentation associated with leukemic lines

A

pain in the legs or refusal to walk associated with leukemic lines

45
Q

when children are over the age of __, bilateral lucent bands called __ are indicative of leukemia

A

2

leukemic lines

Radiology Tumors (11 decks)

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