Case

Patient Profile and Reason for Visit:

32 yo G1 female at 27w1d presents as transfer from outside hospital due to concern for severe preeclampsia.

History of Presenting Problems:

Three days prior to transfer, the patient developed new severe cramping epigastric pain that radiated to her mid back. She vomited once that day. She denied diarrhea, melena, hematochezia, fever, chills, recent trauma or falls. The pain got progressively more intense, so she presented to the ED at the outside hospital, where her blood pressure was as high as 226/138. She was treated with multiple IV hypertensive agents. She was given betamethasone, started on magnesium and then transferred to your hospital.

Upon arrival here, her abdominal pain has improved. She denies headaches, vision changes, chest pain, or shortness of breath.

Past history:

Cholecystectomy

Social History:

Non-smoker, social drinker

Family History:

Non-contributory

Medications:

Multiviatmins

Allergies:

None

Review of Systems:

Otherwise negative

The following describes this patient’s physical exam in the ED:

General appearance: In no distress

Vital signs: Heart rate 60/min, blood pressure 134/76 mmHg, respiratory rate 13/min, T 98^oF

Head and neck: No lymphadenopathy

Chest: Normal to inspection, palpation, percussion, and auscultation

CVS: S1, S2, no extra heart sounds, no murmurs

Abdomen: Gravid

CNS: No focal findings

LABS

Thrombocytopenia reported to occur in estimated 7%-10% of pregnancies; of these about 70%-80% reported to have gestational thrombocytopenia, and 3% reported to have ITP.

The PT and aPTT are not prolonged and the fibrinogen is elevated (the latter being consistent with pregnancy +/- inflammation).

Which of the following conditions is most likely to be ruled out based on these coagulation assays (choose one answer)?

Which of the following conditions is most likely to be ruled out based on these chemistry assays (choose one answer)?

Causes of liver dysfunction in pregnancy include:

• Pregnancy-related liver disorders, including:
  • HELLP
  • Acute fatty liver of pregnancy (AFLP)
  • Hyperemesis gravidarum
  • Intrahepatic cholestasis of pregnancy
• Non-pregnancy-related liver diseases – liver disorders that are coincidental with pregnancy, including:
  • Cirrhosis and portal hypertension
  • Chronic hepatitis B and C
  • Autoimmune liver disease

This patient has no history of antecedent liver disease. Her presentation is not consistent with hyperemesis gravidarum. The presence of liver function test abnormalities points away from a diagnosis of TTP or HUS. The concomitant presence of hypertension and thrombocytopenia raises a concern for HELLP vs. AFLP. Which of the following features most favors HELLP over AFLP (choose one answer):

So far, the data point more towards a diagnosis of HELLP.

The H in HELLP refers to hemolysis. Our patient presented with a normal Hb. Is it possible she has hemolysis?

Further investigation showed:

• Reticulocyte count 189 x 10⁹/L (appropriate)
• Haptoglobin 10 (low)
• 1-2 schistocytes per high power field on peripheral smear (abnormal)

There is no single lab value that confirms a diagnosis of HELLP. Diagnosis is one of exclusion and typically relies on one of three classification schemes:

• American College of Obstetricians and Gynecologists:
  • LDH ≥ 600 IU/L
  • AST and ALT elevated more than twice the upper limit of normal
  • Platelet count < 100 x 10⁹/L
• Tennessee Classification System for complete HELLP syndrome – widely used for diagnosis:
  • Platelets ≤ 100 x 10⁹/L
  • AST ≥ 70 units/L
  • LDH (or bilirubin) (with hemolysis as evidenced on abnormal peripheral smear) levels of 600 IU/L (≥0.2 mg/dL) or more.
• Mississippi Triple-Class System – underlines the severity of the disorder according to the nadir of the platelet count:
  • HELLP class 1 (severe)
    • Platelets ≤ 50 x 10⁹/L
    • AST or ALT ≥ 70 units/L
    • LDH ≥ 600 units/L
  • HELLP class 2 (moderate)
    • Platelets 50-100 x 10⁹/L
    • AST or ALT ≥ 70 units/L
    • LDH ≥ 600 units/L
  • HELLP class 3 (mild)
    • platelets 100-150 x 10⁹/L
    • AST or ALT ≥ 40 units/L
    • LDH ≥ 600 units/L

If we accept the LDH of 575 as meeting the threshold for diagnosis (the day after admission, it increased to 623), the patient meets criteria for diagnosis of moderate HELLP.

True of false: the patient should receive platelet transfusion.

Once the patient was stabilized with antihypertensive medication and magnesium for seizure prophylaxis, she underwent urgent delivery by C-section. Both mother and baby did well. The labs normalized within 3 days of delivery.