(Ch22/23) Vascular occlusion and purpura

Question 1

aPL antibody-negative group
Sneddon As/w

Answer 1

1. ↑larger-pattern livedo reticularis with rings >1 cm
2. ↓Seizures
3. ↓mitral regurgitation by echocardiography or clinically audible
4. ↓thrombocytopenia (<150000/mcl)

Question 2

APLS + lupus As/w

Answer 2

1. ↑arthritis
2. livedo reticularis,
3. thrombocytopenia
4. Leukopenia

Question 3

APL♀ As/w

Answer 3

1. ↑ arthritis
2. Livedo reticularis
3. migraine

Question 4

APL♂ As/W

Answer 4

1. ↑ MI
2. epilepsy
3. arterial thrombosis in lower legs & feet.

Question 5

aPL antibody-mediated thrombosis via

Answer 5

✗ production & release of prostacyclin
✗ protein C / S pathways
✗ antithrombin III activity
✗ prekallikrein activation to kallikrein
✗ endothelial plasminogen activator release

Question 6

Systemic Sx of APL

Answer 6

DVT/PE
CNS disease

Question 7

Catastrophic ALPS
Risk factors

Answer 7

1. surgeries,
2. drugs (sulfur-containing 3. diuretics, captopril, OCP)
3. D/C of anticoagulant
   infections

Question 8

Catastrophic ALPS systemic sx

Answer 8

renal & acute respiratory distress syndrome

Question 9

in APL if typical abs -ve what abs test to order

Answer 9

prothrombin antibodies
If standard screening tests are -ve

Question 10

lupus + APLS
treatment helps with anticoagulation

Answer 10

⚑ Antimalarial Rx protects against thromboses.

Question 11

Feature of Sneddon

Answer 11

Overlap with AR adenosine deaminase 2 deficiency

Triad of
1. Widespread livedo reticularis/racemosa (‘broken’ livedo)
2. Labile hypertension
3. Cerebrovascular disease

Question 12

lab abnormality in Cholesterol emboli

Answer 12

Eosinophilia

Question 13

MOA of Cholesterol emboli

Answer 13

1. Spontaneous
2. Arterial or coronary Cath
3. Acute thrombolytic Rx for MI or stroke
4. Prolonged anticoagulation
5. Heimlich maneuver

Question 14

Renal injury causes in Cholesterol embolus

Answer 14

Renal injury 2/2 NLRP3 inflammasome

Question 15

causes of 2° or acquired oxalosis:

Answer 15

1. ↑ intake of oxalate precursors
2. ↑ dietary absorption of oxalate
3. Pyridoxine (Vit B6) deficiency
4. ↓Renal excretion
5. IBD.

Question 16

Causes of 1° hyperoxaluria

Answer 16

due to enzyme deficiencies:

1. ↑ oxalic acid accumulation (type 1)
2. ↑ production (type 2)

Question 17

sign of oxaluria on on path

Answer 17

Yellow–brown, birefringent crystals in rectangular or

Question 18

Lucio phenomenon vs EN-leprosum

Answer 18

No fever
No leukocytosis
No tenderness
Poor response to thalidomide
ONLY in diffuse non-nodular lepromatous leprosy.

Question 19

mention 4 risk factors for developing APS

Answer 19

I. Hypercoagulable state:Factor V Leiden
II. Connective tissue disease:APS
III. Altered fibrinolysis or platelet activation
IV. Dysproteinemia: cryoglobulinemia

Question 20

erythromelalgia due to ET vs other causes/1ry?

Answer 20

i. Unilateral
ii. Progress to ischemic necrosis
iii. Respond to ASA