HELLP syndrome

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Classification according to ICD-10
O14.2 HELLP syndrome
ICD-10 online (WHO version 2019)

The HELLP syndrome is a serious, for the first time in 1982 by Louis Weinstein , an American gynecologist and obstetrician, variant described in detail preeclampsia , a pregnancy-related illness that lead to the hypertensive disorders belongs. The name HELLP one is acronym from the English names of the most important and typical laboratory findings H aemolysis ( hemolytic anemia ), E levated L iver enzymes (elevated liver enzymes) and L ow P latelet count (reduction in platelet count (platelets) = thrombocytopenia ).
The cause ( etiology ) of the HELLP syndrome has not been clearly established and the course cannot be calculated.

Epidemiology

The incidence is 0.17 to 0.8% of all live births. It occurs most frequently between the 32nd and 34th week of pregnancy, but can also only manifest itself in the puerperium (up to 30%). In 7 to 10% of patients, the disease occurs before the 27th week of pregnancy.
The median age of the pregnant women concerned is 25 years, the proportion of first-time women is 52 to 81%. about 4 to 35% of pregnant women with severe preeclampsia develop a HELLP syndrome.

Symptoms

The main clinical symptom of the HELLP syndrome are symptoms in the right upper abdomen, which are caused by tension in the liver capsule and occur in 80 to 90% of those affected. In 20 to 40% of cases, they can precede the laboratory changes by days.
Non-specific symptoms such as nausea, vomiting or diarrhea can also occur. Headaches and visual disturbances can also be an indication.
Due to the unspecific symptoms, there is a risk of confusion with impending eclampsia , cholecystitis , cholelithiasis , hepatitis or pyelonephritis . In addition, the symptoms of preeclampsia, high blood pressure and protein in the urine can be absent in up to 15% of patients.

In the fetuses there is a growth retardation in up to 50%. In CTG changes can occur that a hypoxia as a result of placental insufficiency point or placental abruption.

Diagnosis

The decisive factor for the diagnosis of a HELLP syndrome is the “laboratory chemical triad” consisting of hemolysis , increased transaminases ( ASAT , ALAT ) and thrombocytopenia .
Hemolysis not only leads to anemia with a drop in hemoglobin and hematocrit , but also to an increase in indirect bilirubin (in 47 to 62%). Fragmentocytes can be detected in the peripheral blood smear in 54 to 86% of the patient . In over 95% of the cases there is a decrease in haptoglobin , which is the most sensitive parameter.
In addition to the increase in transaminases, the LDH is significantly increased.
In the coagulation system, in addition to the progressive decrease in the platelet count, there is a decrease in antithrombin III and an increase in the D-dimer level. Classic coagulation parameters such as the Quick value , thrombin time and fibrinogen are pathologically changed in only 10 to 42%, depending on the severity of the disease.
Signs of renal insufficiency, such as increased creatinine and uric acid levels , as well as proteinuria can occur, but are also absent.
The laboratory parameter determinations should be repeated every 6 to 8 hours.

Classification

A classification system developed in Mississippi determines the severity of the disease according to the lowest observed platelet count in the patient. Class I is the most severe, with a relatively high risk of morbidity and mortality compared to the other two classes.

  • Class I: platelet count below 50,000 / µl
  • Class II: platelet counts from 50,000–100,000 / µl
  • Class III: platelet counts from 100,000–150,000 / µl

Another classification system introduced in Memphis categorizes HELLP syndrome based on its expression.

  • Incomplete HELLP syndrome is characterized by the manifestation of one or two of the most important diagnostic criteria.
  • a complete HELLP syndrome is characterized by the manifestation of all three main diagnostic criteria.

course

Large hematoma of the liver capsule in HELLP syndrome shown in sonography

The course of a HELLP syndrome is incalculable. With conservative therapy, the disease can regress for a few days, progress in relapses or even worsen very quickly.
There is a risk of rapid development of shock syndrome as a result of disseminated intravascular coagulopathy (DIC) (in 4 to 38%), acute kidney failure (up to 8%), pulmonary edema (up to 6%), cerebral hemorrhage, or rupture of a subcapsular liver hematoma with intra-abdominal bleeding and premature placental detachment (up to 16%).

therapy

A causal pharmacological therapy for HELLP syndrome is not known. Initial measures consist of a gentle lowering of blood pressure with antihypertensive drugs and the avoidance of eclampsia by administering magnesium or anticonvulsants .
Conservative therapy attempts , for example with acetylsalicylic acid (ASA), immunoglobulins , plasmapheresis , are considered special cases and are used in very early pregnancies and very slow disease progression. Such a procedure, combined with the induction of lung maturation through the administration of corticosteroids and in the expectation of a temporary regression of the HELLP syndrome, aims to achieve fetal organ maturity and thus avoid complications in children.

However, since the dynamics of the HELLP syndrome are very difficult to assess, the obstetric procedure usually consists of an immediate termination of pregnancy, often by a caesarean section .
The HELLP patient requires intensive medical monitoring. A thrombosis prophylaxis with heparin should be initiated only after an improvement in the clotting disorder.

forecast

The maternal lethality is 3 to 5%. Serious maternal complications must be expected in 12.5 to 65% of cases. If the liver ruptures, the maternal lethality is up to 50%.
If the clinical picture takes a favorable course, the laboratory values ​​normalize within a few days after the end of the pregnancy. However, postpartum HELLP syndrome can also occur in up to 30% of cases.
The risk of recurrence in a subsequent pregnancy is between 3 and 24%.

The fetal perinatal lethality is between 5 and 30%. The risk to the child results from the risks of premature birth , the growth retardation due to chronic placental insufficiency and the high risk of premature placental detachment. The children also have thrombocytopenia in up to 28% and leukocytopenia in up to 42% .

causes

The cause ( etiology ) of the HELLP syndrome has not yet been clearly clarified. We are currently investigating whether damage to the inside ( endothelium ) of blood vessels in the placenta is causing blood to clot there . This massive coagulation causes the number of platelets to drop dangerously. The deposition of the excess fibrin leads to increasing damage to the liver.

Scientists at the University of Magdeburg at the Institute for Clinical Chemistry and Pathobiochemistry were able to demonstrate that platelet activation and the generation of extracellular vesicles (EVs) are of central importance. Platelets and EVs cause a thrombo-inflammatory reaction in the placenta. The platelets activated by EVs release the messenger substance ATP , which causes sterile inflammation in the cells of the placenta ( trophoblasts ). This inflammatory reaction leads not only to damage to the placenta and the embryo, but also to an increase in blood pressure and damage to the kidneys. In animal models , specific pharmacological interventions with acetylsalicylic acid or the anti-inflammatory substances anakinra , apyrase or purinergic receptor antagonists prevented the consequences associated with preeclampsia .

Literature and Sources

Web links

  • Familienplanung.de - HELLP Syndrome : The information portal of the Federal Center for Health Education (BZgA)

Individual evidence

  1. ^ Louis Weinstein: Syndrome of hemolysis, elevated liver enzymes, and low platelet count. A severe consequence of hypertension in pregnancy. In: American Journal of Obstet. Gynecology 142, 1982, pp. 159-167.
  2. ↑ a b c d e f g h i j k l m Werner Rath , Klaus Friese (Ed.): Diseases in pregnancy. Georg Thieme Verlag, 2005, ISBN 3-13-136271-5 , pp. 84-89
  3. ↑ Werner Rath, Wolfgang Loos, Walther Kuhn: The HELLP syndrome. Zentralbl Gynäkol 116 (1994), pp. 195-201, PMID 8023604 .
  4. ↑ a b c d e f Joachim W. Dudenhausen , HPG Schneider, Gunther Bastert (ed.): Gynecology and obstetrics. Verlag Walter de Gruyter, 2013, ISBN 3-11-090580-9 , pp. 196-200
  5. ↑ a b c d Jörg Baltzer (Ed.): Practice of gynecology and obstetrics: the complete practical knowledge in one volume. Georg Thieme Verlag, 2006, ISBN 3-13-144261-1 , pp. 244–246
  6. ↑ Werner Rath: The HELLP syndrome - an interdisciplinary challenge. In: Dtsch Arztebl. 1998; 95 (47), pp. A-2997 / B-2555 / C-2367.
  7. ↑ James Martin, Pamela Blake, Suzanne Lowry, Kenneth Perry, Joe Files, John Morrison: Pregnancy complicated by preeclampsia-eclampsia with the syndrome of hemolysis, elevated liver enzymes, and low platelet count: how rapid is postpartum recovery? . In: Obstet Gynecol . 76, No. 5 Pt 1, November 1990, pp. 737-41. PMID 2216215 .
  8. ^ François Audibert, Steven A. Friedman, Antoine Y. Frangieh, Baha M. Sibai: Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome . In: Am J Obstet Gynecol . 175, No. 2, August 1996, pp. 460-4. PMID 8765269 .
  9. ↑ Werner Rath: Aggressive versus conservative approach in the HELLP syndrome - a position assessment. Birth and Frauenheilk 56 (1996), pp. 265-271.
  10. ↑ Michael Bolz: Prematurity and HELLP Syndrome. In: Klaus Friese, C. Plath, Volker Briese (eds.): Premature birth and premature birth. Springer Verlag, ISBN Print ISBN 978-3-642-63046-0 , doi: 10.1007 / 978-3-642-57222-7_16 , pp. 215-224
  11. ↑ Ernst Beinder, Wolfgang Frobenius: Preeclampsia : An endothelial disease ? Dtsch Arztebl 97 (2000), pp. A-2703 / B-2298 / C-2044
  12. ↑ Shrey Kohli, Berend Isermann et al .: Maternal extracellular vesicles and platelets promote preeclampsia through inflammasome activation in embryonic trophoblast . In: Blood . 23 August 2016, doi : 10.1182 / blood-2016-03-705434 ( bloodjournal.org ).