Updated: 12/15/2018

Thrombotic Thrombocytopenic Purpura (TTP)

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Snapshot
  • A 21-year-old woman with past medical history of SLE presents with fever, fatigue, and altered mental status. On physical exam, a petechial rash is seen on her upper extremities.  Her temperature is 103°F (39.4°C). Her labs are notable for Hgb 7.3 g/dL, platelets 17,000/mm3, and creatinine of 3.4 mg/dL.
Introduction
  • Thrombotic microangiopathy characterized by a pentad
    • microangiopathic hemolytic anemia
    • acute renal failure
    • thrombocytopenia
    • fever
    • neurologic abnormalities
  • Similar to HUS but with fever and neurologic symptoms
  • Epidemiology
    • female > male
    • most commonly in young adults
  • Pathogenesis
    • deficiency or inhibition of metalloproteinase ADAMTS13 
      • most commonly disabled by an autoantibody
      • ADAMTS13 degrades vWF multimers
      • deficiency → large vWF multimers → increased platelet adhesion → platelet thrombosis 
      • important underlying risk factor, but requires a secondary trigger
    • triggers
      • drugs (ticlopidine, clopidogrel, and cyclosporine)
      • SLE
Presentation
  • Symptoms (recall pentad)
    • thrombocytopenia
      • easy bleeding
      • epistaxis
    • neurological symptoms
      • confusion
      • seizures
    • thrombosis
    • renal dysfunction (less so than HUS)
  • Physical exam
    • fever
    • pallor (from anemia)
    • purpura/petechiae
    • jaundice (from hemolysis)
    • splenomegaly
Evaluation
  • Complete blood count
    • anemia
    • thrombocytopenia
  • Peripheral blood smear
    • schistocytes (helmet cells)
  • Serum
    • ↑ LDH
    • ↑ creatinine
    • negative Coomb’s
  • ↑ bleeding time
  • normal PT/PTT
Differential Diagnosis
  • DIC
  • HUS
  • Sepsis
Treatment
  • Plasma exchange transfusion with fresh frozen plasma
  • Steroids
  • Splenectomy
  • Platelet transfusion contraindicated
    • platelet transfusion will worsen disease by feeding the platelet consumption
Prognosis, Prevention, and Complications
  • Prognosis
    • remission with plasma exchange in majority of patients
    • mortality rate 13-15%
  • Complications
    • permanent neurologic sequelae
 

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Questions (4)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.HE.203) A 27-year-old male presents to the emergency department with a one day history of progressive confusion and oliguria. The patient has had several episodes of epistaxis and bleeding gums since the onset of his illness. Past medical history is unremarkable and the patient does not take medications. Temperature is 39C, blood pressure is 100/62 mm Hg, pulse is 95/min, and respiratory rate is 18/min. Physical exam is notable for a purpuric rash in the lower extremities bilaterally. Peripheral blood smear is shown in Figure A. Which of the following is the most likely mechanism behind the patient's illness? Review Topic

QID: 106270
FIGURES:
1

Defect in platelet aggregation

2%

(3/142)

2

Deficiency in degradation of von Willenbrand Factor (vWF) multimers

61%

(86/142)

3

Peripheral platelet destruction

25%

(35/142)

4

Defect in platelet-to-collagen adhesion

5%

(7/142)

5

Decreased levels of von Willenbrand Factor (vWF)

7%

(10/142)

M1

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