HELLP syndrome

Summary

HELLP syndrome (MONDO:0008585) is a disease and 27 clinical trials. Top therapeutic interventions include dexamethasone, betamethasone, and calcium. A subtype of severe pre-eclampsia — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: Unknown (Worldwide) [Orphanet-validated]
• Phenotypes (HPO): 32
• Clinical trials: 27

Clinical features

Signs & symptoms

Clinical features (HPO)

32 HPO clinical features (Orphanet curated; top 32 by frequency):

HPO ID	Term	Frequency
HP:0001873	Thrombocytopenia	Obligate (100%)
HP:0002910	Elevated circulating hepatic transaminase concentration	Obligate (100%)
HP:0001878	Hemolytic anemia	Very frequent (80-99%)
HP:0000093	Proteinuria	Frequent (30-79%)
HP:0002315	Headache	Frequent (30-79%)
HP:0004324	Increased body weight	Frequent (30-79%)
HP:0007430	Generalized edema	Frequent (30-79%)
HP:0008071	Maternal hypertension	Frequent (30-79%)
HP:0008151	Prolonged prothrombin time	Frequent (30-79%)
HP:0011900	Hypofibrinogenemia	Frequent (30-79%)
HP:0012378	Fatigue	Frequent (30-79%)
HP:0100602	Preeclampsia	Frequent (30-79%)
HP:0001058	Poor wound healing	Occasional (5-29%)
HP:0001937	Microangiopathic hemolytic anemia	Occasional (5-29%)
HP:0002013	Vomiting	Occasional (5-29%)
HP:0002018	Nausea	Occasional (5-29%)
HP:0002027	Abdominal pain	Occasional (5-29%)
HP:0002202	Pleural effusion	Occasional (5-29%)
HP:0002615	Hypotension	Occasional (5-29%)
HP:0003418	Back pain	Occasional (5-29%)
HP:0003641	Hemoglobinuria	Occasional (5-29%)
HP:0005521	Disseminated intravascular coagulation	Occasional (5-29%)
HP:0011419	Placental abruption	Occasional (5-29%)
HP:0025435	Increased circulating lactate dehydrogenase concentration	Occasional (5-29%)
HP:0025547	Decreased mean corpuscular hemoglobin concentration	Occasional (5-29%)
HP:0030834	Shoulder pain	Occasional (5-29%)
HP:0100598	Pulmonary edema	Occasional (5-29%)
HP:0100601	Eclampsia	Occasional (5-29%)
HP:0410019	Epigastric pain	Occasional (5-29%)
HP:0001342	Cerebral hemorrhage	Very rare (<1-4%)
HP:0001919	Acute kidney injury	Very rare (<1-4%)
HP:0011029	Internal hemorrhage	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	HELLP syndrome
Mondo ID	MONDO:0008585
EFO	EFO:0007297
MeSH	D017359
Orphanet	244242
DOID	DOID:13133
ICD-10-CM	O14.2
ICD-11	1748922908
NCIT	C84750
UMLS	C0162739
MedGen	58162
GARD	0008528
MedDRA	10049058
Is cancer (heuristic)	no

Also known as: hemolysis, elevated liver enzymes, low platelets in pregnancy · hemolysis, elevated liver enzymes, lowered platelets · hemolysis-elevated liver enzymes-low platelet count syndrome · hemolysis-elevated liver enzymes-low platelets syndrome · PEE1 · PREECLAMPSIA/eclampsia 1 · toxaemia of pregnancy

Disease family

This is a subtype of severe pre-eclampsia. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by etiologic mechanism › disease of genetic or genomic mechanism › hereditary disease › preeclampsia › severe pre-eclampsia › HELLP syndrome

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated for this disease

0 approved, 1 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

Drug	Development status
Dexamethasone	Phase 3 (in late-stage trials)

Clinical trials & evidence

Clinical trials

Clinical trials: 27.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	18
PHASE4	4
PHASE3	4
PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT07510568	PHASE4	NOT_YET_RECRUITING	Vitamin D Supplementation Among Pregnant Women in Uganda for the Prevention of Hypertensive Disorders in Pregnancy, and Other Adverse Maternal and Foetal Outcomes
NCT00711841	PHASE4	TERMINATED	Effectivity of Postpartum Dexamethasone for Women With Class I HELLP Syndrome
NCT02911701	PHASE4	COMPLETED	Effect of Acetaminophen on Postpartum Blood Pressure Control in Preeclampsia With Severe Features
NCT02920593	PHASE4	UNKNOWN	A Randomized Control Trial of Vitamin D Prophylaxis in the Prevention of Hypertensive Disorders of Pregnancy
NCT06452498	PHASE3	NOT_YET_RECRUITING	Preeclampsia Intervention Netherlands
NCT00000534	PHASE3	COMPLETED	Calcium for Pre-Eclampsia Prevention (CPEP)
NCT00097110	PHASE3	COMPLETED	RCT of Antioxidant Therapy to Prevent Preeclampsia in Brazil
NCT01138839	PHASE3	UNKNOWN	Dexamethasone Efficacy in HELLP I Syndrome
NCT04725812	PHASE2	TERMINATED	Complement Regulation to Undo Systemic Harm in Preeclampsia
NCT05500989	Not specified	RECRUITING	PlacEntal Acute Atherosis RefLecting Subclinical Atherosclerosis
NCT06377878	Not specified	RECRUITING	The Preeclampsia Registry
NCT07151339	Not specified	RECRUITING	Pilot Project Renal and Cardiovascular Tertiary Prevention in Preeclampsia
NCT07377786	Not specified	NOT_YET_RECRUITING	Study of Prognostic Values of Platelet Indices and Inflammatory Markers in Patients With HELLP Syndrome.
NCT01736826	Not specified	COMPLETED	Free DNA and Nucleosome Concentrations in Pathological Pregnancies
NCT02247297	Not specified	UNKNOWN	Pancreatic Stone Protein (PSP) in Pregnant Women
NCT02754778	Not specified	COMPLETED	Influence of Conditional Workout Postpartum on Arterial Stiffness Among Women With Status After Preeclampsia, Superimposed Preeclampsia or HELLP-syndrome
NCT02807324	Not specified	UNKNOWN	Women Specific Cardiac Recovery After Preeclampsia
NCT03111446	Not specified	COMPLETED	General Anesthesia Versus Spinal Anesthesia in Patients of HELLP Syndrome
NCT03232359	Not specified	COMPLETED	Immature Platelet Fraction as a Promising Biomarker in Prediction Outcome of HELLP Syndrome
NCT03246542	Not specified	COMPLETED	Promising Bimarker Prediction of Outcome of HELLP Syndrome.
NCT03455387	Not specified	COMPLETED	Evaluation of the Serum Markers sFLt1 and PlGF for the Prediction of the Complications of the Placental Vascular Pathologies in the 3rd Quarter of the Pregnancy.
NCT03605511	Not specified	UNKNOWN	TTP and aHUS in Complicated Pregnancies
NCT03715647	Not specified	COMPLETED	Correlation of Survival in Puerperae by Electrical Impedance Tomography
NCT03834285	Not specified	UNKNOWN	Liver Disease in Pregnancy
NCT04216706	Not specified	COMPLETED	Early Vascular Adjustments to Prevent Preeclampsia
NCT04823949	Not specified	COMPLETED	Pregnancy-Related Hypertension: Adherence to a New Type of Monitoring
NCT06758960	Not specified	COMPLETED	Analysis of Biological Progression and Regression of HELLP Syndrome in Time

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
DEXAMETHASONE	4	2
BETAMETHASONE	4	1
CALCIUM	4	1
ECULIZUMAB	4	1
IBUPROFEN	4	1
METFORMIN	4	1
WATER	4	1
CHEMBL5072826	0	1