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HELLP/Imitators of Severe Preeclampsia/Eclampsia

Baha M. Sibai
Professor
University of Cincinnati College of Medicine

Imitators of Severe Preeclampsia/Eclampsia
Learning Objectives:

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Describe the signs/symptoms in various syndromes confused with preeclampsiaeclampsia Compare diagnostic tests among these syndromes Discuss management/complications in these syndromes

B. Sibai

Imitators of Severe Preeclampsia

 Acute fatty liver pregnancy (AFLP)  Thrombotic thrombocytopenic purpura
(TTP)

 Hemolytic uremic syndrome (HUS)  Exacerbation of Lupus (SLE)  Catastrophic APA syndrome  Sepsis: viral  Stroke
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HELLP Syndrome ? LP EL ELLP HEL ? ? ? ? B. Sibai .

Recommended Criteria for HELLP Syndrome  Hemolysis (at least two of these) —Peripheral smear (schistocytes. Sibai .000/mm3) * Also elevated in severe hemolysis B.2 mg/dl) —Low serum haptoglobin —Severe anemia. unrelated to blood loss  Elevated liver enzymes —AST or ALT ≥ twice upper level or normal —LDH ≥ twice upper level or normal*  Low platelets (<100. burr cells) —Serum bilirubin (≥ 1.

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Maternal Complications (%) ELLP Severe HELLP (n=67) (n=71) (n=178) Transfusion DIC Wound disruption Renal failure 25 15 4 0 3 0 14 3 8 1.5 11 0 4 0 2 0 3 0 Pulmonary edema ICH B. Sibai .

Onset of HELLP in Relation to Delivery (n=506) B. Sibai .

Sibai Unpublished.169:1000-6) . see Sibai et al (Am J Obstet Gynecol 1993.Presenting Complaints (n=506) Complaint % RUQ or epigastric pain Nausea or vomiting Headache Visual changes Bleeding Jaundice Shoulder or neck pain Diarrhea 64 36 30 11 9 5 5 4 B.

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Management of HELLP Syndrome  Refer to tertiary care facility (< 35 wks)  Admit to labor & delivery area  IV magnesium sulfate  Antihypertensives if SBP ≥ 160. Sibai . or DBP ≥ 105mmHg  < 24 wks or ≥ 34 wks  Fetal distress  Maternal distress •Eclampsia •Abruptio placentae / DIC •Renal failure •Respiratory distress •Suspect liver hematoma Delivery 24-34 wks  Complete steroid course • 24-48 hours latency B.

if present  Consult anesthesia  Delivery B.HELLP Syndrome Management  IV MgSO4  Control severe hypertension  Assess fetus (GA. Sibai . FHR)  Consider steroids  Correct DIC.

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1) 1.3-28) 1.6) 3 (4.6) 0.R. crude (95% CI) 0.3-28) 0.8 (0.6) *Only included patients without the event before randomization B.8 (0.5 (0.0 (0.4-4. Sibai .3-2.3 (0.1 (0.6-2.9) 0.8 (0. edema* Eclampsia Infections Death Platelets Tx Plasma Tx 8 (13) 4 (6) 1 (2) 10 (15) 10 (15) 1 (2) 10 (15) 6 (9) Dexamethasone n (%) 6 (10) 5 (7.2 (0.2-1.4) 3.Complications in HELLP Syndrome & Steroids Fonseca et al (Am J Obstet Gynecol 2005) Placebo n (%) ARF* Oliguria Pulm.5) 3.3-1.3-2.6) 8 (14) 5 (8) 3 (5) 12 (18) 5 (8) R.

6 35.2 28.Dexamethasone for Postpartum HELLP Syndrome Katz et al (Am J Obstet Gynecol 2008) Complicationa Pulmonary edema Hemorrhagic manifestation ARF Oliguria Blood Tx Any Complication Death Dexamethasone (n=56) n % 2 20 9 27 16 37 2 3.2 32.7 16.7 24.1 3.6 Placebo (n=49) n % 5 16 12 22 19 25 2 10.1 48. Sibai .9 38.6 66.5 44.6 51.0 4.1 aEach patient may have more than 1 complication B.

Cesarean Delivery  Vesicouterine peritoneum left open  Subfascial drain  Skin closure • Subcutaneous drain OR • Closure 3 days later  Transfusions as needed B. Sibai .

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jaundice  Weight loss. GI)  Low grade fever B. Sibai .vomiting  Diarrhea.Acute Fatty Liver of Pregnancy Presentation  Mean onset 34 wks (21-41)  Epigastric/RUQ pain  Malaise. dehydration  Reduced fetal movement  Bleeding (vaginal. nausea.

Acute Fatty Liver of Pregnancy Laboratory Findings  Hemoconcentration  Platelet count: normal or reduced  Fibrinogen (< 300 mg/dl)  Prolonged PT & PPT (90%)  Elevated WBC with left shift  Elevated creatinine (≥ 1.2 mg/dl)  Elevated uric acid (> 8 mg/dl)  Reduced antithrombin III (100%)  Increased lactate B. Sibai .

Acute Fatty Liver of Pregnancy Liver Function Tests  Increased AST. Sibai .7 g/dl)  Increased LDH  Increased bilirubin (direct)  Elevated ammonia (50%)   Hypoglycemia (postpartum) Low cholesterol B. ALT (100-500 IU)  Reduced albumin (< 2.

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Acute Fatty Liver of Pregnancy Management  Stabilize maternal condition  Immediate fetal testing  Delivery (vaginal if possible)  Supportive care • Management of complications  Liver transplant? B. Sibai .

coma) B.Acute Fatty Liver of Pregnancy Complications        Acute renal failure (ATN) Pulmonary edema .ARDS Metabolic acidosis ( lactate) Pancreatitis • Sepsis (UTI. uterine) Urinary tract infection Resistant DIC (severe hemorrhage) Encephalopathy (lethargy. Sibai . seizures.

Sibai .HELLP with ascites AFLP with ascites B.

TTP/HUS AFLP B.HELLP. Sibai .

HELLP HELLP AFLP B. Sibai .

Sibai .AFLP HELLP B.

Sibai .AFLP with subcapsular hematoma HELLP with subcapsular hematoma B.

3%) 8 16 18 NR 13 10 11 43 129/205 (63%) 13 28 29 9 11 7 11 40 160 (78%) .Maternal Outcome in AFLP Authors Usta # of Pregs 14 Death 0 Hypoglycemia 10 DIC 12 Reyes Castro Pereira Yang Muldenhauer De-Garcia Law Knight TOTAL 16 28 32 27 22 10 18 57 224 0 0 4 5 2 2 2 1 14 (6.

Maternal Complications in Pregnancies with AFLP Complications Death DIC % 0-20 50-100 Hypoglycemia ATN Encephalopathy Ascites Sepsis Pulmonary edema/ARDS 50-100 50-100 20-67 30-50 30-50 15-30 Pancreatitis Diabetes insipidus 0-40 ? B. Sibai .

Perinatal Outcome in AFLP (n=214)  GA at delivery 22-42 wk • Mean 34 wk • < 37 wk (75%)  Twins 8-20%  Perinatal mortality (10-18% B. Sibai .

Thrombotic Thrombocytic Purpura (TTP)   CNS manifestations (typically fluctuate in severity) Renal impairment   Hemolytic anemia Thrombocytopenia  Fever B. Sibai .

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Sibai .Thrombotic Thrombocytic Purpura (TTP) Clinical Presentation  Anemia (pale looking)  Hematuria-proteinuria  Ecchymosis-purpura  Neurologic complaints •Transient & recurring  Retinal detachment B.

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Sibai .Thrombotic Thrombocytopenic Purpura Treatment        Plasmapheresis Plasma infusions Low-dose aspirin Steroids/rituximab Platelet transfusions? Splenectomy Supportive care B.

Ischemic changes in TTP PRES in HELLP/Eclampsia B. Sibai. MD .

et al. of Women 9 5 No.† Dulcoy-Bouthars 11 11 9 5 5 11 13 9 7 6 2 3 1 0 0 1 0 0 0 0 4 5 0 1 0 Shamseddine Stella. Sibai . Total 4 12 71 4 14 81 1 3 12/71(17%) 0 0 3 (3%) 0 1 13 (18%) †Only definite cases developing during pregnancy are included B. et al. Vesley. et al. et al. et al.† No. Dashe. Castella. et al.Maternal Outcome TPP/HUS Authors Hayward. Ezra. et al. of Pregs 9 8 Death 1 1 CNS Injury 2 0 Renal Injury 2 0 Egerman.

et al. of Women 9 5 11 11 9 5 5 4 12 71 No. Castella. et al. et al. et al. Sibai . of Pregs. 9 8 11 13 9 7 6 4 14 81 Death 1 4 2 1 2 3 1 3/5 4/16 4/16 Preterm 4/9 4/8 5/11 3/13 4/9 4/7 2/5 4/5 -30/67 (45%) B. Dashe. et al. Ezra.Perinatal Outcome in TTP/HUS Authors Hayward. et al. Vesely. Egerman. Dulcoy-Bouthars Shamseddine Stella Total No.

Sibai .Hemolytic Uremic Syndrome (HUS)      Extremely rare antepartum Onset 1-4 weeks’ postpartum Thrombocytopenia Microangiopathic hemolytic anemia Severe renal failure • • Dialysis Residual renal deficit Sepsis Severe abruptio  May be secondary to • • B.

Sibai .Hemolytic Uremic Syndrome Treatment      Plasma infusions Plasmapheresis Dialysis Antithrombotic agents Supportive care B.

Sibai .Imitators (Laboratory Findings) HELLP Anemia Platelets LDH AST Fibrinogen PT/PTT Glucose Creatinine Uric Acid Ammonia Bilirubin ± ++ +++ ++ N N N ± + + TTP/HUS +++ +++ ++++ ± N N N ++ ++ ++ AFLP ± ++ ++ Reduced Prolonged Reduced ++ ++ + +++ B.

Sibai . kidneys. lungs. skin. & nervous system B.Systemic Lupus Erythematosis  Autoimmune-mediated chronic inflammatory disease  Deposits of antigen-antibody complexes in capillaries & visceral organs  Most commonly affect joints.

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Exacerbation of Lupus
Clinical Findings

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Hypertension + proteinuria Renal insufficiency Thrombocytopenia Pleuritis – pneumonitis Fever Cerebral vasculopathy
• Seizures

Lupus hepatitis
• Liver infarction*

*In association with APA’s

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Exacerbation of Lupus
Treatment


High-dose prednisone
Immune suppressive agents
• Hydroxychloroquine • Azathioprine

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IV gamma globulin Heparin Plasmapheresis

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Sibai .Imitators (Clinical Findings) HELLP Hypertension ++ TTP/HUS + AFLP ± Lupus + Proteinuria Seizures Purpura Jaundice ++ + ++ ± ++ ++ ++ ± ± ± ++ + + - Neurologic Renal failure Fever + ± - ++ ++ ++ ± ++ + ± + ++ B.

Sibai .Catastrophic Antiphospholipid Syndrome  Small vessel occlusion in at least 1 organ  Involvement of ≥ 3 organ systems • • • • • Renal (70%) Pulmonary (65%) Cerebral (60%) Cardiac (50%) Skin (50%)  Onset of manifestations together or < 1 wk  Presence of APA’s (high titer) B.

DIC Hemolysis Elevated creatinine + IgG & IgM. MD . encephalopathy Myocardial ischemia Purpura. multiple emboli. headache. livedo reticularis  Laboratory findings • • • • Thrombocytopenia. Sibai. pulmonary infiltrates Seizures.Signs & Symptoms of CAPs  Clinical findings • • • • • • Abdominal pain Severe HTN. ACAs B. proteinuria ARDS.

Sibai .Liver infarction in CAPS Liver infarction in HELLP syndrome B.

MD . Sibai.MRI revealing liver infarcts in APS Repeat MRI done 3 months later showing resolution of infarction Guiu B Arch Gynecol Obstet 2010 B.

80% without B. rituximab Hemodialysis Mechanical ventilation *Mortality is 30% with treatment. Sibai. MD .Treatment of CAPS*        Full dose heparin (7-10 days) High dose corticosteroids (3-5 days) Plasma exchange IVIg Cyclophosphamide.

Disseminated Herpes Simplex        Elevated transaminases (> 2000 IU/L) Elevated LDH/hemolysis Thrombocytopenia/DIC Fever. leukocytosis Elevated ammonia level Bilirubin normal or slightly elevated Liver biopsy shows intranuclear inclusions & cell necrosis & hemorrhage B. Sibai .

Disseminated Herpes Simplex      Encephalitis Hepatitis Hemolysis High maternal mortality Treatment: IV acyclovir B. Sibai .

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Sibai. MD .Fatty changes Ischemic changes Micro abscesses CT of liver in HELLP CT of liver in herpes hepatitis B.

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The End .