Hematology and Oncology Book

Anemia

Cardiovascular Medicine

Coagulopathy

Hemolytic Disorders

  • HELLP Syndrome

Pharmacology

http://www.fpnotebook.com/

HELLP SyndromeAka: Hemolysis Elevated Liver Enzymes Low Platelets

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  1. See Also
    1. Preeclampsia
    2. Severe Preeclampsia
  2. Definition
    1. Variant of Severe Preeclampsia
  3. Epidemiology
    1. More common in multiparous caucasians
    2. More common after age 25 years
    3. Onset after 20 weeks gestation
      1. Preterm <27 weeks: 7%
      2. Preterm <37 weeks: 46%
      3. Term (37-41 weeks): 14%
      4. Postpartum: 33%
  4. Pathophysiology
    1. Microangiopathic Hemolysis
      1. RBCs fragmented in vessels with damaged endothelium
    2. Fibrin deposition
      1. Vascular deposition contributes to Hemolysis
      2. Hepatic sinusoid deposition with periportal necrosis
        1. Results in liver enzyme abnormalities
    3. Platelet aggregation
  5. Risk factors
    1. Preeclampsia (HELLP occurs in 12% of PIH cases)
  6. Symptoms
    1. Viral-type prodrome
      1. Malaise
    2. Gastrointestinal symptoms (most common)
      1. Right Upper Quadrant Abdominal Pain
      2. Epigastric Pain
      3. Nausea or Vomiting
    3. Severe Preeclampsia symptoms may be present
    4. Bleeding may occur if coagulopathy is present
      1. Hematuria
      2. Gastrointestinal Bleeding
  7. Signs
    1. Hypertension (85% of cases)
    2. Weight gain
    3. Edema
  8. Differential Diagnosis
    1. See Right Upper Quadrant Abdominal Pain
    2. See Hemolysis Causes
    3. See Thrombocytopenia
    4. See Aminotransferase
  9. Labs
    1. Consider Preeclampsia labs
    2. Complete Blood Count
    3. Platelet Count <100,000/mm3
    4. Peripheral Smear with signs of Hemolysis
      1. Schistocytes
      2. Burr Cells
      3. Helmet Cells
    5. Liver Function Tests
      1. Aminotransferases (AST >70 IU/L)
      2. Serum Bilirubin >1.2 mg/dl
        1. Increased in Hemolysis
      3. Lactate Dehydrogenase (LDH) >600 IU/L)
        1. Increased in Hemolysis
    6. Coagulation Factors if platelets <50,000 or bleeding
      1. Fibrinogen
      2. Fibrin split products
      3. Prothrombin (PT)
      4. Partial Thromboplastin Time (PTT)
  10. Management
    1. See Severe Preeclampsia Management
    2. Consider Dexamethasone 10 mg IV q12 hours
      1. Consider when Platelet Count <100,000/mm3
      2. May improve lab abnormalities and delay delivery
      3. Magann (1994) Am J Obstet Gynecol 171:1148
    3. Consider Blood Products
      1. Platelet transfusion
        1. Platelet Count <20,000/mm3
        2. Platelet Count <50,000/mm3 prior to ceserean
      2. Other Blood Products to consider in active bleeding
        1. Packed Red Blood Cells for severe blood loss Anemia
        2. Fresh frozen plasma for coagulation abnormalities
  11. Complications
    1. Hepatic rupture
    2. Acute Renal Failure
    3. Pulmonary edema
    4. Ascites
    5. Pleural Effusion
    6. Abruptio Placenta
    7. Postpartum Hemorrhage
    8. Disseminated Intravascular Coagulation (DIC)
  12. Prognosis
    1. Maternal mortality: 1-3%
    2. Infant and fetal mortality: 6-36%
  13. References
    1. Fontaine (2000) in ALSO, B:1-36
    2. Padden (1999) Am Fam Physician 60:829

HELLP Syndrome (C0162739)

Definition (MSH)A syndrome of HEMOLYSIS, elevated liver ENZYMES, and low blood platelets count (THROMBOCYTOPENIA). HELLP syndrome is observed in pregnant women with PRE-ECLAMPSIA or ECLAMPSIA who also exhibit LIVER damage and abnormalities in BLOOD COAGULATION.
ConceptsDisease or Syndrome (T047)
MSHD017359
EnglishHaemolysis-elevated liver enzymes-low platelet count syndrome, HELLP, HELLP Syndrome, Hemolysis-elevated liver enzymes-low platelet count syndrome
Spanishsindrome de hemolisis-enzimas hepaticas elevadas-bajo recuento de plaquetas, sindrome HELLP
Parent ConceptsHypertension induced by pregnancy (C0340274), Pre-Eclampsia (C0032914), Thrombocytopenia (C0040034), Severe pre-eclampsia unspecified (C0341950), Duplicate concept (C1274013)
SourcesMSH, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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