MOC June 2014: Young and confused

You just arrived for your 4pm shift, when you hear overhead, “Ambulance to Room 1 in 5 minutes, STAB team room 1 in 5 minutes”.  You head to the room to prepare.  39 year old female coming by EMS, syncopal episode with head trauma now confused, though airway intact, vitals stable.

EMS Arrives:  39 year old female with no significant PMHx, was at Pickerel Lake for a picnic with family when she stated she didn’t feel well, stood up and was walking towards the bathroom.  Was walking up a concrete staircase then syncopized, falling with impact to her head and left side.  +Brief LOC, no seizure activity per family, ambulatory shortly after, however seemed confused and not making sense.  Backboarded with C-collar at EMS arrival.  Accucheck 125.  Negative stroke screen, alert, but limited verbal output, complaining of headache unable to obtain further history.  12 lead with sinus tach.  T 37 C P 110s  BP 128/80  R 18  SpO2 100%.  1 P IV in place, no interventions en route.

HPI: Patient is intermittently moaning, speaks few words but cannot provide additional history.  No prior visits in EMR.

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1-- Should this patient be a trauma team activation?

United criteria for Level 2 trauma activation:

 - GCS 12-14 (not associated with alcohol or other substances)

 - >/= 20% BSA burns

 - Fall >20 feet or 2 times the height of child

 - Auto vs pedestrian with significant impact

 - Ejection from auto or other moving vehicle

 - High speed MVC with severe single injury or multiple system injuries

 - Death in same compartment

 - Pregnant trauma > 16 weeks

 - ED MD discretion

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Exam:

Vitals: T 37.3 C  P 120  BP 140/90  R 18  SpO2 97%RA
Gen: Adult female, laying in bed, opens eyes and looks around, moans

HEENT: Small hematoma with overlying abrasion on left occiput, Normocephalic, PERRL, EOMI, Oropharynx clear, no signs of facial trauma

Neck: Supple, C-collar in place, trachea midline, no JVD, no signs of trauma

Cardiac: Tachycardiac with regular rhythm, no m/r/g

Pulm: Clear and equal, no tachypnea

Abdm: Soft, nondistended, no reaction to palpation

Extrem: Warm, well perfused, atraumatic

Neuro: Alert, minimal verbal response with few words spoken, no overt dysarthria, no facial droop, moves all extremities spontaneously, follows commands (opens eyes/squeezes hands)

Skin: Outside of scalp abrasion/hematoma, no other signs of trauma nor skin changes

Bedside eFAST negative

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2-- What is their GCS?  Do they need airway control?

GCS 13 = motor 6 + verbal 3 + ocular 4

Although altered, appear to be protecting their airway

3-- Differential diagnosis?

Arrhythmia, CVA, Seizure, Hypoglycemia, Hypovolemia, Vasovagal, Valvular disease, Heat stroke, ACS, SAH, ICH, TBI, Overdose and others

4-- What imaging/labs do you order?

Initial orders included:
 - Portable CXR, CT/CTA head/neck, CT cspine

 - iSTAT creatinine

 - CBC, Comp, Troponin, PT/PTT, Ethanol, ASA, Tylenol, UA/Upreg

 - EKG

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Portable CXR negative

Patient taken to head CT for planned CT/CTA head/neck, CT C-spine. While in CT reviewing images, the tech informs you iSTAT creatinine is 1.9. CTA canceled.

CT head/C-spine negative

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Patient taken back to room 1, EKG with sinus tach & you ask the RN to cath to obtain urine.

Hmm… that doesn’t look right.  You decide to repeat the FAST exam, still negative.  Labs pending.

You walk away to check on another patient when shortly later lab calls with critical values on CBC:

  - HGB           8 

  - Platelets      10

You go back to reassess the patient...

She opens her eyes, states she has a headache. When you ask more questions, she looks around and does not respond, then closes her eyes. Patient squeezes your hands, moves her toes, exam otherwise unchanged, protecting her airway.

Family has just arrived and state she was doing well until 2 days ago, went to Urgent Care for abdominal pain with N/V/D, they thought she was better and no other complaints they were aware of.  No prior med hx, on no meds, NKDA. Smokes cigarettes, drinks socially, no drug use.

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5-- Any change in your differential diagnosis? 

Acutely altered patient with fever, tachycardia, presumed new anemia, thrombocytopenia and renal insufficiency:

TTP, HUS, Sepsis +/- DIC, Meningitis, Preeclampsia/HELP, Malignant hypertension, Connective tissue disorders/Systemic Vasculitis, Autoimmune hemolytic anemia, Systemic Malignancy, Overdose

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Remainder of labs return:
 - WBC 11, 75% neutrophils, no bands

 - Hgb 8, microcytic

 - Platelets 10

 - Na 134, Potassium 5, Cl 111, CO2 18, BUN 14, Creat 1.9, Glucose 124

 - AST/ALT/Alk Phos/Alb wnl, TBili 2.7 

 - PT/PTT wnl

 - Troponin 0.050

 - ASA/Tylenol - negative, Ethanol - negative

 - UA +protein, +blood, +LE, >100 RBCs, 5-10 WBCs, no bacteria

 - UPreg - negative   

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6-- Further narrowing of the differential?  What additional labs do you consider?  Medications?  Consults?

 - Patient presenting with classic pentad of TTP

 - Add on type & screen, peripheral smear/LDH/indirect Bili to help confirm MAHA, blood cultures, lactate, procalcitonin, retic count

 - Heme/Onc and place catheter placement for plasma exchange

 - Also need to include sepsis in differential, would start broad-spectrum antibiotics and supportive therapy while completing diagnostic workup

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Thrombotic Thrombocytopenic Purpura, aka TTP:

Rare blood disorder caused by clotting in the microvasculature.  Cause unknown, however thought to be due to abnormally large forms of von Willebrand factor in patient’s plasma, either due to congenital defects, acquired antibodies, release from endothelial injury and others.  The enlarged vWF forms lead to platelet aggregation/microthrombi, which in turns causes consumptive thrombocytopenia.  Microangiopathic hemolytic anemia results due to fragmentation of RBCs as they cross arterioles/capillaries occlude by thrombi.

Microvascular ischemia leads to symptoms/signs involving multiple organ systems.

The “Classic” Pentad of TTP includes: microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever, and renal disease. However it is rare to see all five present.

High index of suspicion for clinical diagnosis is required as treatment is time sensitive.  The mortality rate is >90% if untreated, 10-20% with treatment. Treatment of choice is plasma exchange.

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Questions:

1. True/False: At least 3/5 findings in the pentad of TTP are required to establish the diagnosis.

2. Which one of the following explains the etiology of most cases of TTP?

a. Drugs

b. Idiopathic

c. Familial

d. Iatrogenic

e. Paraneoplastic

3. Which one of the following laboratory variables would not be expected in a patient with TTP?

a. Low platelet count

b. Elevated indirect bilirubin level

c. Normal APTT and PT values

d. Normal reticulocytes

e. High serum LDH value

4. If plasma exchange therapy is not available immediately, which one of the following therapeutic options would be best until plasma exchange can be initiated?

a. Platelet transfusion

b. FFP infusions

c. Red blood cell transfusions

d. Hemodialysis

e. Intravenous immunoglobulin

5. True/False: Platelet transfusions are indicated in TTP with a platelet count <10,000.

6. Which one of the following therapeutic or supportive measures is not indicated in the management of TTP?

a. Heparin

b. Red blood cell transfusions

c. Glucocorticoids

d. Plasma exchange

e. Antihypertensive agents

7. Which of the following drugs is not classically associated with TTP?

a. Cyclosporine

b. Clopidogrel

c. Bactrim

d. Tacrolimus
e. Quinine

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Answers:

1. False. TTP is a clinical diagnosis with significant time sensitivity to initiate treatment as well as a high mortality rate if untreated.  Only thrombocytopenia and microangiopathic hemolytic anemia without other clinically apparent etiology is required to establish the diagnosis.

2. b. Idiopathic

3. d. Normal reticulocytes -- Would expect elevated reticulocytes given normal bone marrow response to hemolysis/anemia.  A, B, E all classic due to hemolysis and consumptive thrombocytopenia.  Abnormal PT/PTT levels is suggestive of DIC.  Would expect to see schistocytes on peripheral smear to confirm MAHA.

4. b. FFP infusions -- Risk of FFP infusions alone without plasma exchange is volume overload, particularly if oliguric/anuric or CHF.  If unable to obtain timely plasma exchange, patient should be transferred to definitive care.

5. False.  Platelet transfusions are generally contraindicated in TTP and can lead to significant morbidity due to increased microcirculatory aggregation.  Only indicated in life-threating hemorrhage, which is rare in TTP.

6. a. Heparin

7. c. Bactrim