Title: The HELLP Syndrome
1The HELLP Syndrome A Therapeutic Challenge
- JOHN ESSIEN M.D.
- JESSICA BARDALES MITAC M.D.
- J.M RODRÍGUEZ FERNÁNDEZ M.D.
- EMILIO ORTEGA CALLAVA M.D.
- HOSPITAL GINECOBSTÉTRICO PROVINCIAL
- CAMAGÜEY.
2- Pre-eclampsia - Is a multisystemic, idiopathic
disorder specific to the pregnancy and puerperium
of the human species. It is characterized by the
clinical triad of - Hypertension
- Proteinuria
- Edema
3- Literature dating from the XIXth century report
- Very unusual varieties of severe pre-eclampsia
with complicated progress. - These unusual descriptions of pre-eclampsia are
recognised today as the HELLP Syndrome. - Today
- HELLP Syndrome is considered to be an
association of characteristic hepatic and
hematologic disorders.
4HELLP
WEINSTEIN(1982)
H
HEMOLYSIS
EL ELEVATED LIVER
ENZYMES
LP LOW
PLATELETS
5- The reported incidence 2 a 12 .
- Elevated perinatal morbidity and mortality.
- Maternal Mortality 35.
6HELLP SYNDROME POSIBLE PATHOPHYSIOLOGY CAUSAL
AGENTES Increase in volume., Fetal presence /
decidual cell?, Vasospasm?, Deficiente vascular
repair?, Idiopathic? Vasculo-endothelial
Disorder Platelet Agregation/Consumption Fibri
n Activation/Consumption Selective organic
Isquemia/nsuficiency Variable Manifestations
7Other Factors to consider
- ERITHROCYTIC MORPHOLOGY
- PLATELET DISORDERS
- RENAL COMPROMISE
- HEPATIC DISORDERS
- IMMUNOLOGIC DISORDERS
- GENETIC DISORDERS
-
8The Causal Factors induce
- Thrombocytopenia
- Microangiopathic Hemolytic Anemia
- Periportal necrosis and distension of the livers
Glissons capsule. -
9DIAGNOSIS
- MID-II TRIMESTRE
- FIRST DAYS POSTPARTUM
- Antepartum diagnosis is made in 70 between 27
and 37 weeks of gestation.
10Criteria for establishing the diagnosis of the
HELLP Syndrome
- HemolysisAbnormal peripherical blood smear
Elevated Bilirubin gt1.2 mg/dl - Elevated liver enzymesSGOT gt72 UI / LLDH gt600
UI / L - Low PlateletsPlatelet Count lt 100 103 /mm3
-
11We can also observe
- Excessive body weight increase .
- Pulse pressure amplification.
- Systole pressure gt 140 mmHg, but diastole
pressure lt 90 mmHg. - Ophthalmic disorders
- -Minor alterations
- -Cortical blindness (amaurosis)
- -Retinal detachment
- -Vitreous hemorrhage.
-
12We can also observe
- Elevation of Biomarkers
- -HCG
- -Maternal alfa-fetal protein
- -LDH
- -Serum Haptoglobin
13- The presence of these disorders in an
hypertensive woman with epigastric and/or right
hypochondrial pain, nausea, vomiting as well as
hemolysis, will help in making the right
diagnosis. -
14Clasification of the HELLP Syndrome based on the
platelet count (MISSISSIPPI)1.
- Class 1 Platelet count lt50 000/mm3.
- Class 2 - Platelet count between 50 000 y 100
000/mm3. - Class 3 - Platelet count ltbetween 100 000 y 150
000/mm3. -
15- Hemolysis Liver disfunction
- LDH 600 UI/l
- ASAT (SGOT) and/or ALAT (SGPT) 40 UI/l
- ALL HAVE TO PRESENT
- 1Magann E.F., Martín J.N. Twelve Steps to
Optimal Management of HELLP Syndrome. Clinical
Obstetrics and Gynecology. Lippincott Williams
Wilkins, Philadelphia, 1999. Vol. 42 No. 3
532-50.
16Another classification based on the partial or
complete expression of the HELLP
Syndrome(MEMPHIS)1.
- Complete HELLP
- Microangiopathic hemolytic anemia in women with
severe pre-eclampsia - LDH 600 UI / L
- SGOT 70 UI/l
- Thrombocytopenia lt 100 000/mm3
- PARTIAL HELLP One or two of the above.
17THROMBOTIC MICROANGIOPATHIES -Thrombotic
thrombocytopenic purpura- Microangiopathic
hemolytic anemia induced by sepsis or drugs-
Hemolytic Uremic Syndrome FIBRINOGEN CONSUMPTION
DISORDERS CID-Acute fatty liver-Sepsis-
Severa Hypovolemia / Hemorrhage
(Abruptio/Amniotic fluid embolism)CONNECTIVO
TISSUE DISORDERS-Systemic Lupus Erithematosus
Differential Diagnosis of the HELLP Syndrome
18PRIMARY RENAL DISEASE Glomerulonefritis
OTHERSHepatic encephalopathiesViral
hepatitisHyperemesis Gravidarum Idiopathic
Thrombocytopenia Renal calculi Peptic
ulcerPielonephritisApendicitisDiabetes
Mellitus
Differential Diagnosis of the HELLP Syndrome
19(No Transcript)
20MANAGEMENT OF THE HELLP SYNDROME
211. ANTICIPATE THE DIAGNOSIS2. EVALUATE THE
MATERNAL CONDITION3. EVALUATE THE FETAL
CONDITION 4. CONTROL THE HYPERTENSION5. PROFILAX
IS OF CONVULSIONES WITH MgSO46. WATER AND
ELECTROLITIC BALANCE 7. HEMOTHERAPY8. MANAGEMENT
OF LABOR AND DELIVERY9. OPTIMIZE PERINATAL
CARE10.INTENSIVE POSTPARTUM TREATMENT OF THE
PATIENT 11.BE ALERT FOR MULTIPLE ORGAN
FAILURE12.ADVISE ON FUTURE PREGNANCY
22The Maternal Condition can be evaluated by
- Complete Hemogram. If plateletslt150.000/mm3
requieres more study. - Liver Enzymes. The elevation of the transaminases
and LDH is a sign of hepatic disfunction. - Renal function. Deficencies in renal function are
observed in late stages of the illness.
Creatinine and Uric acid levels are variable.
23- Bilirubin. Unconjugated bilirubin is increased
due to the hemolysis but rarely above 1-2 mg. - Serial evaluation laboratory parameters every 12
to 24 hours or more if necessary. - Differential diagnosis with othere pathologies.
24Evaluating the Fetal Condition
- Determine the gestational age.
- Evaluate fetal well-being Non-stress test,
Tolerance to contracction test and/or biophysical
profile. - Use corticosteroids between 24 and 34 weeks to
improve fetal pulmonary maturity/neonatal
pulmonary function as well as maternal and
perinatal results.
25Controlling the hypertension
- 80-85 of patients with HELLP need control of
their BP to avoid significant maternal and
perinatal morbidity and mortality. - Treat systolic BP whengt150mmHg and avoid
placental hypoperfusion maintaining the diastolic
BP not less than 80-90 mmHg.
26Choice of hypotensive medication
- Hydralazine Bolus of 5-10 mg IV every 20-40
min. If uneffective or unavailable, use
labetalol, nifedipine o sodium nitroprussiate. - Labetalol Initial bolus of 20 mg IV, with
increases in dosage until a satisfactory BP is
obtained or up to maximum dose of 300 mg. - Nifedipina oral(not sublingual) at usual dosage.
27- Sodium Nitroprussiate is a fast acting
hypotensive agent(venous and arterial) which can
be used in an hypertinsive crisis when all other
hypotensive drugs have failed Loading dose 0,25
µg/kg/min, increasing upto 10 µg/kg/min. Above
this dose there is a greater risk of cyanide
intoxication of the fetus. When using, remember
its photosensitivty and sever rebound effect.
28Preventing Convulsions
- MgSO4 Initial bolus of 4-6g IV, followed by a
continous infusion at 1,5-4g/h, individualized
according to the patient. Continue 48 horas o
more postpartum until clinical and laboratory
signs of improvement are obtained. -
- If contraindications of MgSO4 exist, use
Phenytoin. Loading dose 15 mg/kg at 40 mg/min
with continous monitorization of the cardiac
function and BP every 5 minutes. The therapeutic
range is 10-20 µg/ml.
29Water and Electrolytic Management
- Objectives
- -diuresis of 30-40ml/h.
- -Limit intake of liquids to 150ml/h.
- -Balance of electrolytes.
- REMEMBER
- NEGATIVE BALANCEvasoconstriction.
- EXCESIVE POSITIVE BALANCE pulmonary damage
- Monitorization of volume through pulmonary
capilar wedge pressure
30Hemotherapy
- The base of hemotherapy in patients with HELLP
is the transfusion of platelets. - The usual dose is one unit per every 10 kg of
corporal weight. - Spontaneous bleeding occurs in most cases with a
platelet count of lt50.000/mm3.
31Hemotherapy
- To avoid postpartum hemorrhage in a transvaginal
delivery the indication for platelet transfusion
is a count lt40.000/mm3. - In the immediate postpartum periodo Maintain
the count gt50.000/mm3 abdominal deliveries and
gt20.000/ mm3 in transvaginal deliveries.
32Hemotherapy
- The aggresive use of Dexamethasone in patients
with HELLP and severe thrombocytopenia has
eliminated virtually all need for platelet
transfusion. - Other therapeutic alternatives
- -Plasmaphersis
- -Immunoglobulins
33Management of labor and delivery
- When considering termination of gestation in a
patient with HELLP, determine - Gestational age.
- Maternal and fetal conditions.
- Fetal presentation.
- Cervical maturity
34Management of labor and delivery
- If transabdominal delivery is requiered,
perform - Vertical skin incision.
- Corporeal incision of the uterus (due to scarse
development of the inferior segment and abnormal
presentationes). - Spontaneous delivery of the placenta to avoid
hemorrhage
35Optimizing perinatal care.
- The main risk for the fetus in pregnancies with
HELLP is its prematurity. - The use of corticosteroids decreases the
morbidity associated with pulmonary immaturity in
preterm babies. - Delivery should be in a center with capability of
treating these children with a major risk of
cardiopulmonary instability.
36Postpartum Intensive Care.
- Admision in an obstetrical intensive care unit
until - Sustained increase in the platelet count and a
maintained decrease in LDH. - Diuresis gt100ml/h for 2 consecutive hours
without duiretics. - Well controled BP with systolic pressure ? 150
mmHg and diastolic pressure lt 100 mmHg. - Obvious clinical improvement and absence of
complications. - The absence of improvement of the
thrombocytopenia within 72-96 hours postpartum
indicates severe compromise of compensatory
mechanisms and possibel MULTIPLE ORGAN FAILURE.
37Postpartum Intensive Care - The use of
Dexamethasone
- ANTEPARTUM (0,15mg/kg)10mg IV bid
- - when Platelets lt100.000/mm3
- - if Platelets 100.000-50.000/mm3 AND
- Eclampsia, Severe Hypertension, Epigastric
Pain - POSTPARTUM 10mg IV bid for 2 dosis, then 5mg bid
for 2 additional doses - - when steroids were used in antepartum
- - suspend when there is clinical and laboratory
improvement (platelets gt100.000mm3, decreased
LDH, diuresis gt100 ml/h) -
38Be on the lookout for
- Signs of multiple organ failure.
- Complications
- Subcapsular Hematoma
- Subcapsular hepatica hemorrhage
- Hepatic Rupture.
- Therapeutic solutions
- Conservative Procedures
- Surgery.
-
39Advising on future pregnancies.
- The risk of recurrence of preeclampsia -eclampsia
is 42-43 and for the HELLP syndrome 19-27. - The risk of recurrence of preterm delivery is
high, about 61.1
40Conclusions
- HELLP Syndrome and its management still poses a
problem in modern obstetrics - Precise diagnosis and early treatment with
non-mineral corticosteroides such as
Dexamethasone may help achieve favorable maternal
and perinatal results.
41THANK YOU!