HELLP Syndrome
Original Editors - Carolyn S. Furdek from Bellarmine University's Pathophysiology of Complex Patient Problems project.
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Definition/Description[edit | edit source]
HELLP syndrome is an acronym for several life-threatening symptoms that occur together in a woman’s pregnancy.
These symptoms are:
- H – hemolysis
- EL – elevated liver enzymes
- LP – low platelet count. [1]
[Image courtesy of The Internet Journal of Anesthesiology]
Prevalence[edit | edit source]
As of Nov 2010, for every 1,000 pregnancies, 1 to 2 (0.5%-0.9%) women will be diagnosed with HELLP syndrome. Furthermore, 10-20% of women diagnosed with severe preeclampsia will be diagnsosed with HELLP.[1]
Patients have a 19-27% chance of reoccurance on subsequent pregnancies.[2]
Characteristics/Clinical Presentation[edit | edit source]
Approximately 7 out of 10 patients with HELLP syndrome will experience the symptoms prior to delivery between the 27th and 37th week of gestation. The remaining patients will develop the symptoms within 48 hours postpartum.[3]
There are two classifications of HELLP Syndrome:[2]
- Classification 1: Basis of 3 classic lab values
- Partial: one/two of the classic values present
- Full: all three abnormalities present
(Full HELLP syndrome classifications have a higher mortality rate and should be delivered within 48 hours)
- Classification 2: Basis of platlet count
- Class 1: Platlet count < 50,000 mm3
- Class 2: Platlet count 50,000-100,000 mm3
- Class 3: Platlet count 100,000-150,000 mm3
(Class 1 pts have a higher maternal morbidity and mortality rate)
Clinical symptoms of HELLP include discomfort in the upper right quadrant of the abdomen, pain in the epigastric area, vomiting, and nausea.[3] The abdominal discomfort can increase and decrease throughout the day.[4] Patients can report extreme fatigue prior to presentation or ‘feeling unwell’.[4][1] Other symptoms include headache, fluid retention, excess weight gain, blurry vision, nosebleeds (or bleeding that does not stop easily), seizures/convulsions.[1]
Associated Co-morbidities[edit | edit source]
HELLP syndrome can lead patients to be at a higher risk for the following conditions:[5][6]
- Renal Failure - loss of the kidney’s ability to function properly. The body will no longer be capable of filtering excess fluid, waste, and salts from the blood. This leads to dangerous levels in the system.[7]
- Consumptive coagulopathy - (also known as disseminated intravascular coagulation (DIC)) - clotting factors reduced[8]
- Abruptio placentae - the placenta nourishing the fetus abruptly separates from the uterine wall prior to delivering the baby[8]
- Pulmonary edema - fluid build up in the lungs - can lead to shortness of breath.[8]
- Cerebral edema - build up of fluid around the brain[9]
- Subcapsular liver hematoma - pooling of blood just outside of the liver[8]
- Hypovolemic shock - excessive fluid and blood loss that can lead to organ failure[8]
Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
Lab Tests and Values:[2]
Hemolysis:
- Low haptoglobin concentration (< 1 g/L – < 0.4 g/L) - more specific indicator [3]
- High LDH[3]
- presence of unconjugated bilirubin[3]
(if Hematocrit normal: decreased serum haptoglobin levels may be present indicating HELLP)[2]
Liver Enzymes: As high as 4,000 U per L
Platelets: As low as 6,000 per mm3 (anything less than 150,000 per mm3 should be of concern)
Plasma fibrogen: levels less than 300 mg per dL (DIC suspected)
Etiology/Causes[edit | edit source]
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Systemic Involvement[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
In patients diagnosed with HELLP syndrome prior to delivery, the immediate treatment is delivery of the fetus.[10] If the fetus is earlier than 34 weeks gestation, steroid injections and close monitoring for 24-48 hours may be provided to allow the fetus’ lungs to mature.[3]
[Chart courtesy of Journal of The American Family Physician: HELLP Syndrome]
Physical Therapy Management (current best evidence)[edit | edit source]
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Alternative/Holistic Management (current best evidence)[edit | edit source]
Due to the severity and risk of maternal mortality, conservative management is not recommended in the treatment of HELLP syndrome.[3]
Differential Diagnosis[edit | edit source]
HELLP Syndrome my be misdiagnosed as any of the below conditions:[3]
- Viral Hepatitis
- Cholangitis
- Acute fatty liver of pregnancy
- Haemolytic uremic syndrome
- Thrombotic thrombocytopenic purpura
- Systemic lupus erythematosus
Case Reports/ Case Studies[edit | edit source]
add links to case studies here (case studies should be added on new pages using the case study template)
Resources
[edit | edit source]
- American Pregnancy Association - www.americanpregnancy.org
- Preeclampsia Foundation - www.preeclampsia.org
- HELLP Syndrome Society - www.preeclampsia.org (merged with preeclampsia foundation)
Recent Related Research (from Pubmed)[edit | edit source]
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References[edit | edit source]
- ↑ 1.0 1.1 1.2 1.3 PubMed Health website. HELLP syndrome. Available at http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001892. Accessed February 18, 2011.
- ↑ 2.0 2.1 2.2 2.3 http://www.aafp.org/afp/990901ap/829.html Cite error: Invalid
<ref>tag; name "Padden" defined multiple times with different content Cite error: Invalid<ref>tag; name "Padden" defined multiple times with different content - ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Haram, K. Svendsen, E. Abildgaard, U. The HELLP syndrome: Clinical issues and management. A Review. BMC Pregnancy Childbirth [serial online]. 2009; 9:8.
- ↑ 4.0 4.1 Sibai BM. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstetrics and Gynecology [serial online]. 2004;103:981–991.
- ↑ Svenningsen R, Morken NH, Kahn JA. Corticosteroids in the treatment of HELLP-syndrome? Tidsskr Nor Laegeforen. 2006;126(17):2253–2256.
- ↑ Vigil-De Gracia PE, Tenorio-Marañón RF, Cejudo-Carranza E, Helguera-Martinez A, García-Cáceres E. Difference between pre-eclampsia, HELLP syndrome and eclampsia, maternal evaluation. Ginecol Obstet Mex. 1996;64:337–382.
- ↑ Mayo Clinic web site. Acute Kidney Failure. Available at: http://www.mayoclinic.com/health/kidney-failure/DS00280. Accessed February 22, 2011.
- ↑ 8.0 8.1 8.2 8.3 8.4 Definitions, Online - Medline Plus. Available online at http://www.nlm.nih.gov/medlineplus/. Accessed 1 March 2011.
- ↑ Definitions, Online – Medical Dictionary. Available online at http://www.medterms.com. Accessed 7 March 2011.
- ↑ Bacq Y. Liver diseases unique to pregnancy: A 2010 update. Clinics and Research in Hepatology and Gastroenterology. 2011; 20: (Article in Press) Available at http://www.ncbi.nlm.nih.gov/pubmed/21310683.
