Morning Report Danielle Behrens D.O. PGY2 August 18, 2009
Chief Complaint Bilateral Leg pain left > right
History of Present Illness • 47 year old female presents to the ED with a chief complaint of bilateral leg pain L>R. • States pain began 2 months ago- LLE calf pain and great toe numbness, worse with ambulation; improved with rest. • Pt was seen at OLOL ED 1 month ago- had Duplex u/s –negative. Pt took Ibuprofen and pain improved. • Pt then developed progressive pain in b/l feet with numbness- still worse with ambulation and improved with rest- saw PCP- no studies done at that time. • Pain has been getting progressively worse over last 3 days- now not relieved by rest
History of Present Illness • Pt went to ED at large inner city hospital 5 days prior- was discharged from ED- told to follow-up with PCP. Also given referral to Neurologist. • Pt again presented to ED at large inner city hospital 2 days prior with worsening pain-Was given Percocet and discharged home. • Pt states pain is persistent- worse with exposure to cold;
PMHx: • Asthma • Uterine Fibroids • GERD • Seasonal Allergies • PSHx: • Tubal Ligation • Social Hx: • 15 pack year smoking history ( ½ ppd x 30 years) • Occasional ETOH • Denies IVDA/ + cocaine use 30 years ago • Works as a teacher’s assistant/bus aide
Family Hx: • Non Contributory • ROS: • b/l LE pain • Paresthesias b/l feet • Cold intolerance b/l LE
Physical Exam • VS:T: 99.5 HR: 114 RR: 18 BP: 138/78 O2 • Gen: AAO x 3, Uncomfortable • HEENT: NCAT, EOMI, PERRLA • CV:tachycardic RR no murmurs, rubs gallops • Lungs: CTA b/l no wheezes, rales, rhonchi • Abd: soft, NT/ND BS + 4, no pulsations • Back: No CVA tenderness • Ext………
…Feet hypersensitive to touch L>R, L foot pale and cool. Toes on R foot purple color with cap refill 3-4 secs. PT pulse recorded on doppler. Photo from Oncology Nursing Society www.ons.org
CT with contrast: • L common artery thrombus without complete obstruction.
Tx to CUH ICU • IV aorta with runoff- • Large ovoid mobile thrombus located withinn the distal abdominal aorta, left eccentric- which extends into the left common iliac artery. • Single vessel runoff on the left with presumed distal embolization of proximal peroneal and posterior tibial arteries.
Thrombectomy done by vascular surgery • Remained in ICU- • Left foot progressively more ischemic/necrotic • Underwent Left BKA
2 D echo: • Normal LV size and wall thickness. LVEF: 60-65% • Normal RV size and function • Normal LA; Normal RA; Normal Interatrail septum. • Negative Bubble study • No masses seen.
Results: • Factor V Leiden- negative • Prothrombin gene mutation- negative • AT III- 67 • Homocysteine: 9.6 • Protein C: 27.4 • Protein S: 58 • Fibrinogen: 492 • B2 Microglobulin I: Neg IgA, IgM & IgG
Results: • Anti-Cardiolipin Ab: POSITIVE • IgA, IgM, IgG NEGATIVE • Lupus Anticoagulant: • dRVVT: **61.4 sec** (28.8-42.0) • Hex Phase: ** 59.7 ** (<8.0)
Antiphospholipid Ab Syndrome • Disorder of coagulation associated with arterial and venous thrombosis • Also associated with recurrent fetal loss
Antiphospholipid Ab Syndrome APS Criteria- revised 2006 *Must have 1 clinical criteria and 1 lab criteria for dx* 1. Vascular thrombosis 2. Pregnancy morbidity - Three or more SABs <10 wks gestation - One or more SAB >10 wks gestation - One or more premature births <34 weeks gestation assoc. with Preeclampsia/eclampsia or placental insuffuciency. 3. Presence of anticardiolipin, Lupus anticoagulant or Anti- B2 Glycoprotein antibodies
Antiphospholipid Ab Syndrome • Primary APS- APS without Rheumatologic Disease • Secondary APS- APS in the presence of Rheumatologic Disease • Ex: SLE with Lupus anticoagulant • ** Important- Pt can have Lupus anticoagulant without SLE**
Antiphospholipid Ab Syndrome • Cardiolipin- mitochondrial membrane phospholipid • B2 Glycoprotein I- phospholipid binding protein • Lupus anticoagulant- antibodies that prolong the coagulation time.
Antiphospholipid Ab Syndrome • Anticardiolipin antibodies are more sensitive • Lupus anticoagulant is more specific
Catastrophic Antiphospholipid Ab Syndrome • Multiple thrombi in 3 or more organ systems over days to weeks • Mortality rate 50% • Death occurs from multiorgan failure • Kidneys are most affected followed by lungs, CNS, heart, skin
Treatment • Aimed at : • Prophylaxis • Treatment of thrombi • Prevention of future thromboemboli • Management in pregnancy
References • “The Antiphospholipid Syndrome”Jerrold S. Levine, M.D., D. Ware Branch, M.D., and Joyce Rauch, Ph.D. The New England Journal of Medicine Volume 346:752-763 March 7, 2002 Number 10 Photo from Oncology Nursing Society www.ons.org