Delayed Diagnosis of Thrombotic Thrombocytopenic Purpura (TTP)

M Chadi Alraies MD, Abdul Hamid Alraiyes MD,

Richard Christie MD1, Emmanuel Elueze MD, FACP2
Internal Medicine Residency Program, Department of Internal Medicine
St. Vincent Charity Hospital - Case Western Reserve University

THE CASE THE EVENT WHY DID IT HAPPEN?

Chief complaint: Eight hours later, patient’s blood pressure was still 200/112 and the patient became febrile 1. Lack of medical knowledge.
“Worst headache in my life” (39.5C), unresponsiveness for 1 minute with spontaneous regaining of consciousness, i. TTP criteria
followed by agitation, confusion, bizarre movement of extremities, and was unable to follow ii. Blood smear reading
commands. Patient was transferred to the ICU and repeated labs showed platelets of 4 iii. Work up of thrombocytopenia
History of present illness
x103. She received 6 units of platelets because of this acute drop in platelets and risk of iv. Severe headache as neurological deficit of TTP
A 57 year old African American lady with past medical history significant for
spontaneous bleeding. Hematology team evaluated the patient in the next morning and the v. Missing the “big picture” by managing each medical problem separately.
hypertension and bronchial asthma presented to our emergency department at 10:00
peripheral smear which showed schistocytes (>1%) and polychromatophilic red cells 2. System failure
pm with headache and uncontrolled blood pressure for one day. This headache was
(reticulocytes) suggesting Microangiopathic hemolytic anemia (MAHA) . TTP (Thrombotic i. Overwhelmed night float team
localized to the left side of her head, gradual in onset, started at 9:30 am when she
thrombocytopenic purpura) was suspected in this patient and the plan was to stop the ii. Lack of experts
went to work, dull in nature, 8/10 in severity and without particular radiation. There
transfusion, start Nitroglycerin IV drip, Haldol IV and get the patient transferred to a tertiary 3. Clinical decision making experience
were no alleviating factors but the pain worsened with head movement. On arrival at
center for plasmapheresis. However, patient became hypotensive and unresponsive and 1. Wrong treatment
the ED, the pain was still present. Patient claimed she was compliant with her
arrested in ICU. 45 min of resuscitation was attempted without success and patient was 2. Delayed follow up labs
medication and never had such headache before. This pain was associated with left
announced dead 12 hours after admission.
eye blurriness, seeing white spots, dark vision, but no photophobia. She claimed
weakness and pain in the right arm (transitory) lasted for 20 minutes in the morning.
Her medications included: Losartan, Amlodipine and Clonidine for BP, Fluticasone
250 mcg/Saleterol 50mcg and Albuterol MDI for asthma. She had no allergies and
worked as a clerk, lived alone, and denied any history of smoking, ETOH or illicit drug WHAT HAPPENED? How to prevent a similar event from happening?
abuse.

Physical Examination 1. Awareness of the diagnostic criteria for TTP.

Vitals on arrival were 36.8oC, BP 210/125, 18, 98, 98% room air. Middle aged, MISSED DIAGNOSIS 2. Have a broad differential diagnosis for thrombocytopenia including TTP.
obese, mildly anxious African American female, in no acute distress. Skin exam 3. Reading blood smears in any case of thrombocytopenia and anemia.
showed: a large, hot, tender ecchymosis on the left lower leg and bilateral pitting 4. “Lab error”
edema of lower extremities. Examination of the chest was normal, CVS examination i. Repeat if in doubt.
was regular rate and rhythm and wasn’t significant for any gallops, murmurs, or rubs. LATE MANAGEMENT ii. Do not delay work up.
Abdominal examination was normal. CNS examination: Awake alert oriented x3 5. “Big picture” approach.
female. Power, tone and deep tendon reflexes were normal. Fundoscopic 6. Early expert opinion.
examination was not done. 7. Critical lab result alert

Laboratory result on admission: PATIENT DIED

Complete blood count and complete metabolic profile showed mild anemia (Hgb
10.3mg/dl and Hematocrit 25%) with baseline hemoglobin of 12.9 mg/dl 3 months
ago, thrombocytopenia (45x103) and renal failure (Creatinine of 2.7) with a baseline
creatinine of 0.9 mg/dl 3 months ago. Urinalysis was significant for proteinuria,
microscopic hematuria - 15-20 RBC/hpf. CT of the head was negative for acute References:
bleeding or ischemia. Chest X-ray showed cardiomegaly, otherwise normal. 2. George, JN. Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354:1927.
3. Kojouri, K, Vesely, SK, George, JN. Quinine-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome:
Frequency, clinical features, and long-term outcomes. Ann Intern Med 2001; 135:1047.
4. George, JN, Gilcher, RO, Smith, JW, et al. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: Diagnosis and
Assessment and Plan: management. J Clin Apheresis 1998; 13:120.
14. Hypertensive emergency secondary to medication non compliance. Plan is to start 5. UpToDate 16.1
6. Agency for Healthcare Research and Quality (AHRQ) website. www.ahrq.org
Labetalol 10 IV q4h PRN for SBP > 180, Acetaminophen 650 mg PO Q4 hours PRN
and Tramadol 50 mg PO Q6hr PRN for headache. Six hours later, the BP was still
elevated at 200/110 and an extra dose of Labetalol IV was given. 1
Dr. Richard Christie, MD is program director of internal medicine residency program, Case Western reserve University / St. Vincent
15. Thrombocytopenia secondary to laboratory error or idiopathic thrombocytopenic Charity Hospital, Cleveland, OH
purpura (ITP) or a lab error. Plan to repeat platelets in 8 hours, hematology consult 2
Dr Emmanuel Elueze, MD, PhD, FACP is an associate program director of internal medicine residency program, Case Western
and 6 units of platelets were put on hold. reserve University / St. Vincent Charity Hospital, Cleveland, OH
Microangiopathic smear Normal peripheral blood smear