Sunday, September 19, 2021

HELLP Syndrome Case File

Posted By: Medical Group - 9/19/2021 Post Author : Medical Group Post Date : Sunday, September 19, 2021 Post Time : 9/19/2021
HELLP Syndrome Case File
Eugene C. Toy, MD, Edward Yeomans, MD, Linda Fonseca, MD, Joseph M. Ernest, MD

Case 16
A 36-year-old G3P2002 Hispanic female at 36 weeks’ gestation is sent from her physician’s office to the obstetrical triage unit for mildly elevated blood pressures. The patient denies headache, visual abnormalities, or right upper quadrant pain. She has no uterine contractions or vaginal bleeding. Her blood pressure in the first trimester was in the 100/60 mm Hg range. On examination today, her BP is 156/98 mm Hg, HR 98 beats per minute, temperature 98.4°F, and RR 12. Her heart and lung examinations were normal. The fetal heart tones are in the 135 beats per minute range with accelerations. There are no uterine contractions. The vaginal examination reveals that the cervix is closed, long, and presenting part is cephalic. The CBC shows a hemoglobin level of 11 g/dL, platelet (plt) 80,000/mm3, and glucose 98 mg/dL, serum glutamic oxaloacetic transaminase (SGOT) 400 IU/L and serum glutamic pyruvic transaminase (SGPT) 440 IU/L, and lactate dehydrogenase (LDH) 1000 mg/dL. Serum creatinine level is 0.8 mg/dL.

➤ What is the most likely diagnosis?
➤ What are your next steps?
➤ What additional lab tests are indicated at this stage?


ANSWERS TO CASE 16:
HELLP Syndrome

Summary: A 36-year-old G3P2002 Hispanic female at 36 weeks’ gestation has a BP of 156/98 mm Hg. Her cervix is unfavorable and presenting part is cephalic. The patient has thrombocytopenia (plt 80,000), glucose 98 mg/dL, SGOT 400 IU/L and SGPT 440 IU/L, and LDH 1000 mg/dL. Serum creatinine level is 0.8 mg/dL.

Most likely diagnosis: Hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome.
Next steps: Admission to labor and delivery unit, begin magnesium sulfate as prophylaxis against convulsions, assess and stabilize maternal condition, particularly blood pressure and coagulation abnormalities (DIC screen), and consider the best option for delivery.
Lab tests: A DIC screen should be obtained. The blood bank should be contacted for possible need for blood products. Currently, there is no overt sign of bleeding or coagulopathy, and no transfusion is needed. The hemoglobin level and platelet count should be monitored, and the platelet count should be above 20,000/mm3 to guard against spontaneous hemorrhage, and above 50,000/mm3 for surgical hemostasis.


ANALYSIS
Objectives
  1. Be able to describe the clinical presentation of HELLP syndrome.
  2. Be able to describe the differential diagnosis of HELLP syndrome including causes of abnormal liver function tests.
  3. Be able to describe the complications of HELLP syndrome.
  4. Describe the treatment of HELLP syndrome including management of the blood products.

Considerations
The clinical presentation of patients with HELLP syndrome is highly variable and is considered a variant of preeclampsia. Patients may present with nonspecific symptoms or subtle signs of preeclampsia. Not all women (12%-18% cases) with HELLP syndrome have hypertension.1 Generally, patients who present with HELLP syndrome are older, multiparous, white females who present at less than 35 weeks gestation.2

Patients usually present remote from term, complaining of epigastric or right upper quadrant pain, some have nausea or vomiting, and others have nonspecific viral syndrome-like symptoms. Most patients (90%) give a history of malaise for the past few days before presentation and some will have nonspecific viral syndrome-like symptoms.3 A subset of patients with severe thrombocytopenia may present with bleeding from mucosal surfaces, hematuria, petechial hemorrhages, or ecchymosis.

There is no strict definition of HELLP syndrome. Criteria for HELLP syndrome is hemolysis (abnormal peripheral blood smear, LDH > 00 U/L, total bilirubin > 1.2 mg/dL); elevated liver enzymes (AST > 70 U/L); and low platelets (< 100,000/mm3), low serum haptoglobin levels.4 Peripheral blood smear may show schistocytes, burr cell, and echinocytes. There is no consensus in the literature regarding the liver function test to be used or the degree of elevation in these tests to diagnose elevated liver enzymes.3

The differential diagnoses of HELLP syndrome is extensive and may be found in Table 16–1. Because some patients with HELLP syndrome may present with gastrointestinal, respiratory, or hematologic symptoms in association with elevated liver enzymes or low platelets in the absence of hypertension or proteinuria, many cases of HELLP syndrome will initially be misdiagnosed.HELLP is most commonly confused with two other medical conditions, acute fatty liver of pregnancy (AFLP), and thrombotic thrombocytopenic purpura/ hemolytic uremic syndrome (TTP/HUS).5 The differentiation among HELLP, AFLP, and TTP/HUS is based on specific laboratory findings and slight differences in clinical presentation. An effort should be made to attempt to identify an accurate diagnosis given that management strategies may differ among these conditions. Patients with acute fatty liver of pregnancy typically present with nausea, vomiting, abdominal pain, and jaundice. Most patients will have

Table 16–1 DIFFERENTIAL DIAGNOSIS OF HELLP SYNDROME

Acute fatty liver of pregnancy
Hemolytic uremic syndrome
Thrombotic thrombocytopenic purpura
Systemic lupus erythematosis
Appendicitis
Hyperemesis gravidarum
Idiopathic thrombocytopenia
Gastroenteritis
Viral hepatitis
Pancreatitis
Gallbladder disease
Glomerulonephritis
Hemorrhagic or septic shock


prolonged PT and PTT with low fibrinogen and low serum glucose.5 TTP is an extremely rare condition during pregnancy characterized by neurologic dysfunction, fever, severe thrombocytopenia, and severe hemolysis with a very low hematocrit. The liver enzymes may be normal or slightly elevated. Patients with HUS usually present with renal failure and most cases develop in the postpartum period.3

The presence of HELLP syndrome is associated with an increased risk of maternal death (1%).6 Maternal morbidities of HELLP syndrome include eclampsia, placental abruption, acute renal failure, pulmonary edema, adult respiratory distress syndrome, subcapsular liver hematoma, disseminated intravascular coagulopathy, intracerebral hemorrhage, and sepsis. There is also an increased rate of wound hematomas and the need for transfusion of blood and blood products. The development of HELLP syndrome in the postpartum period also increases the risk of renal failure and pulmonary edema.Perinatal mortality and morbidities also are increased in HELLP syndrome with the reported perinatal death rate ranging from 7.4% to 20.4%.4


APPROACH TO
HELLP Syndrome

Management and Treatment of HELLP Syndrome
Laboratory evaluation should include a complete blood count with platelet count, a peripheral smear, coagulation studies, serum AST, creatinine, glucose, bilirubin, and LDH levels. HELLP syndrome requires the presence of all the following: platelet count less than 100,000/mm3, AST more than 70 IU/L (> 2 times upper limit for normal values), abnormal peripheral smear, LDH more than 600 IU/L, and/or bilirubin more than 1.2 mg/dL.

The clinical course of women with HELLP syndrome is characterized by usually progressive and sometimes sudden deterioration in maternal and fetal conditions. The first priority is to assess and stabilize maternal condition, particularly coagulation abnormalities.3 Transfer to a tertiary care center with appropriate neonatal care facilities is recommended. If present, severe hypertension should be controlled as well as correction of coagulopathy (Table 16–2). A magnesium sulfate infusion should be started for the prevention of an eclamptic seizure. Computed tomography or ultrasound of the abdomen should be considered if subcapsular hematoma of the liver is suspected.

Evaluation of the fetal well-being includes nonstress testing, biophysical profile, and ultrasound to evaluate the fetal growth and rule out intrauterine growth restriction.

Table 16–2 ANTIHYPERTENSIVE MEDICATION (TO KEEP BLOOD PRESSURE < 160/100 MM HG)


Hydralazine
5 mg IV bolus, repeat as needed every 15 to 20 min for a maximum dose of 20 mg/h
 
Labetalol
20-40 mg IV every 10-15 min for a maximum of 220 mg over 1 h
 
Nifedipine
10-20 mg orally every 30 min for a maximum dose of 40 mg/h


Immediate delivery is indicated in patients more than 34 weeks’ gestation age. For patients less than 34 weeks, antenatal corticosteroids should be given and delivery planned in 48 hours, provided the maternal or fetal status does not decompensate (Table 16–3).3 Because of the significant morbidity and mortality associated with HELLP syndrome, expectant management past 48 hours is generally not advised.

Other authors recommend a more conservative approach to prolong pregnancy in cases of fetal immaturity. The aim of expectant management is to improve neonatal morbidity and mortality. There is no high-quality evidence demonstrating that overall perinatal outcome in patients with HELLP syndrome is improved with expectant management compared with pregnancies delivered after a course of glucocorticoids.8 Expectant management of HELLP syndrome remains an investigational approach and is contraindicated in women with DIC.

The presence of HELLP syndrome is not an indication for immediate delivery by cesarean section. Patients with a favorable cervix should undergo a trial of labor. Labor may be initiated with oxytocin infusion as for routine induction in all patients with gestational age over 30 weeks’ gestation. The option of prostaglandin induction or elective cesarean section should be considered in patients at less than 30 weeks’ gestational age with an unfavorable cervix.3

Table 16–3 INDICATIONS FOR DELIVERY IN HELLP SYNDROME

• < 23 wk or limits of viability
> 34 wk
• After corticosteroid administration if < 34 wk
• Fetal distress
• Maternal distress
    • Eclampsia
    • DIC
    • Respiratory distress
    • Suspect liver hematoma


Consultation with the anesthesiology team is warranted. Epidural anesthesia should be used with caution as many anesthesiologists are reluctant to place an epidural catheter with a platelet count less than 75,000/mm3. General anesthesia is the method of choice for cesarean sections in severe thrombocytopenia. The use of pudendal block is contraindicated in the setting of HELLP syndrome.

In the presence of significant bleeding (ecchymosis, bleeding from the gums, oozing from puncture sites, wound, intraperitoneal) with HELLP syndrome and in all patients with a platelet count less than 20,000/mm3, platelet transfusion is indicated either before or after delivery. If cesarean delivery is required, platelet transfusion of approximately 5 to 10 units should be initiated on call to the operating room in patients with severe thrombocytopenia (< 50,000) before intubation. There is rapid consumption of platelets in these patients (see Table 16-4 for blood product transfusion).

The surgeon should consider placement of a drain (subfacial, subcutaneous, or both) due to generalized oozing. The risk of hematoma formation without preventive therapy is approximately 20%.3

Postpartum management of HELLP patients should include close hemodynamic monitoring and serial laboratory evaluations approximately every 6 hours for 48 hours. Generally, most patients show resolving disease process within 48 hours postdelivery.

Table 16–4 BLOOD COMPONENT THERAPY FOR DIC IN PREGNANCY
 
Fresh frozen plasma (FFP) volume = 250 mL
Corrects PT, PTT, and fibrinogen.Use 4 units initially and then as needed.
Each unit increases fibrinogen 5-10 mg/dL.
 
Cryoprecipitate (volume 35-40 mL)
Rich in fibrinogen.
Utilizes less volume than FFP.
Give when fibrinogen < 100 mg/dL or if clinical hemorrhage and fibrinogen < 150 mg/dL.
Each unit increases fibrinogen 5-10 mg/dL.
 
Platelets
Transfuse if maternal platelets < 20,000/mm3 whether or not clinically bleeding.
Transfuse if maternal platelets < 50,000/mm3 if maternal hemorrhage or cesarean delivery.
Each pack of platelets increases platelet count by 7,000-10,000/mm3.
Rapid consumption of platelets in the setting of DIC.
 
Packed RBCs
Increases oxygen carrying capacity.
Transfuse to keep hematocrit > 25%.
Follow electrolytes as hemolysis and RBC transfusion may lead to hyperkalemia.
 

 
Management of DIC
DIC may complicate HELLP syndrome and is a perilous situation in which there is the simultaneous processes of accelerated formation and lysis of clots. As a result, the body consumes clotting factors more rapidly than can be produced and replaced. Definitive therapy for DIC is removal of the inciting factor. In HELLP syndrome, the inciting factor is pregnancy related, and the definitive therapy is delivery.


Comprehension Questions

16.1 When counseling patients with a history of HELLP syndrome, what do you tell them is the approximate risk of recurrence of HELLP syndrome in subsequent pregnancies?
A. < 5%
B. 15%
C. 20%
D. 50%

16.2 A patient with HELLP syndrome is noted to have repetitive late decelerations and significant vaginal bleeding. Placental abruption is suspected. During an emergency cesarean delivery, the patient starts bleeding from venipuncture and IV site, mucous membranes, and incision sites. Stat labs are sent from the operating room, resulting in decreased fibrinogen, prolonged prothrombin time, and aPTT. What is the best management?
A. Transfusion of whole blood
B. Recombinant factor VII
C. Embolization of the hypogastric arteries
D. Fresh frozen plasma

16.3 A 34-year-old G2P1 woman is at 30 weeks’ gestational age. She is noted to have severe preeclampsia and is delivered by cesarean due to nonreassuring fetal heart rate patterns. Two days postoperatively, the patient is noted to have severe right upper quadrant pain, and elevated liver function tests. On CT scan, she is noted to have a 4 cm hepatic hematoma without evidence of rupture. Her BP is 140/80 mm Hg, HR 90 beats per minute. Her hemoglobin level is 11.2 g/dL and plt count
of 140,000/mm3. Which of the following is the most appropriate management of this patient?
A. Blood transfusion of 3 units of packed erythrocytes
B. Percutaneous embolization of the hepatic arteries
C. Surgical repair
D. Serial ultrasound or CT


ANSWERS

16.1 A. Women with a history of HELLP syndrome are at increased risk of all forms of preeclampsia in subsequent pregnancies. The rate of recurrence of HELLP syndrome is less than 5%. However, the rate of preeclampsia is approximately 20% with significantly higher rates if the onset of HELLP syndrome occurred in the second trimester. Patients who have underlying chronic hypertension have higher risks of recurrence.9 Therefore, these patients require close monitoring during subsequent pregnancies and should be counseled on their increased risk for adverse pregnancy outcome. At this time, there is no preventive therapy for recurrent HELLP syndrome.4

16.2 D. The diagnosis of DIC is usually made clinically with confirmation made through laboratory studies. A falling fibrinogen is usually the hallmark of DIC. Prolonged PT usually occurs prior to prolongation of the aPTT. Two intravenous lines should be established and a Foley catheter should be placed. Aggressive fluid resuscitation can be accomplished while blood component therapy is given. See Table 16–4 for blood component therapy. The fluid status must be monitored closely, as hypovolemia can lead to the development of acute renal failure. Fluid overload, on the other hand, can lead to pulmonary edema.

16.3 C. Management of a contained hepatic hematoma should include close hemodynamic monitoring and checking coagulation status. The patient should be supported with volume replacement and blood transfusion as needed. If the size of the hematoma remains stable and the laboratory abnormalities are resolving, the patient may be discharged with outpatient follow-up. Avoidance of abdominal palpation, convulsions, or emesis should be undertaken to prevent increased intra-abdominal pressure.10

A ruptured subcapsular liver hematoma is a surgical emergency, and should involve liver trauma and vascular surgeons. It usually involves the right lobe. Correction of coagulopathy and massive transfusions of blood products is essential. The maternal and fetal mortality rate is over 50%.11 The current recommendation for treating rupture of subcapsular liver hematoma in pregnancy is packing and drainage.


Clinical Pearls

See US Preventive Services Task Force Study Quality levels of evidence in Case 1
➤ The diagnostic criteria used for HELLP syndrome are variable and inconsistent (Level III).
➤ HELLP syndrome may be confused with other medical, surgical conditions or obstetric complications (Level II-3).
➤ The potential benefits of expectant management in patients remote from term remain experimental (Level II-2).
➤ The use of pudendal block is contraindicated in patients with HELLP syndrome (Level III).
➤ Epidural anesthesia should be used with caution and is contraindicated with a platelet count less than 75,000/mm3 (Level III).
➤ The use of corticosteroids to improve maternal outcome remains experimental (Level I).

REFERENCES

1. Martin JN, Rinehart B, May WL, Magann EF, Terrone DA, Blake PG. The spectrum of severe preeclampsia: comparative analysis by HELLP syndrome classification. Am J Obstet Gynecol. 1999;180:1373-1384. 

2. Roberts JM, Funai EF. Pregnancy related Hypertension. In: Creasy RK, Resknik R, Iams JD, Lockwood CJ, Moore TR, eds. Creasy and Resnik’s maternal fetal medicine: principles and practice, 6th ed. Philadelphia: Saunders, 2009, 651-690. 

3. Sibai BM. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol. 2004;103:981-991. 

4. Queenan JT (ed). Chronic Hypertension in Pregnancy in High Risk Pregnancy. Washington DC: American College of Obstetricians and Gynecologists, 2007. 

5. Sibai BM. Imitators of severe preeclampsia. Obstet Gynecol. 2007;109:956-966. 

6. Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM, Friedman SA. Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). Am J Obstet Gynecol. 1993;169:1000-1006. 

7. Drakeley AJ, LeRoux PA, Anthony J, Penny J. Acute renal failure complicating severe preeclampsia requiring admission to an obstetric intensive care unit. Am J Obstet Gynecol. 2002;186:253-256. 

8. Abramovici D, Friedman SA, Mercer BM, Audibert F, Kao L, Sibai BM. Neonatal outcome in severe preeclampsia at 24 to 36 weeks’ gestation: does HELLP syndrome matter? Am J Obstet Gynecol. 1999;180:221-225. 

9. Van Pampus MG, Wolf H, Mayruhu G. Long-term follow up in patients with a history of HELLP syndrome. Hypertens Pregnancy. 2001;20:15-23. 

10. Fonseca JE, Mendez F, Catalono P, Arias F. Dexamethasone treatment does not improve the outcome of women with HELLP syndrome. A double-blind, placebocontrolled, randomized clinical trial. Am J Obstet Gynecol. 2005;193:1591-1598. 

11. Barton, JR, Sibai BM. Gastrointestinal complications of preeclampsia. Semin Perinatol. 2009;33:179-188.

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