1. Hellping diagnose the misdiagnosed
Jayna Guild and Jessica Archer
Core Hematology

2. case

3. Case
On December 4, 2014, a 36-year-old woman G1P0 at 34 weeks gestation with triplet gestation was admitted to the labor and delivery ward of the hospital from the clinic during a routine non-stress test to rule out preeclampsia secondary to elevated blood pressure
The patient was recorded to have an expected delivery date of January 11, 2015 and had her last menstrual period on April 4, 2014
She was found to have elevated blood pressure of 144/90 (normal BP is at or below 120/80)
Patient denied any headaches, blurry vision, right upper quadrant pain, cervix pain, or abdominal pain

4. Case
On December 4, 2014, a 36-year-old woman G1P0 at 34 weeks gestation with triplet gestation was admitted to the labor and delivery ward of the hospital from the clinic during a routine non-stress test to rule out preeclampsia secondary to elevated blood pressure
The patient was recorded to have an expected delivery date of January 11, 2015 and had her last menstrual period on April 4, 2014
She was found to have elevated blood pressure of 144/90 (normal BP is at or below 120/80)
Patient denied any headaches, blurry vision, right upper quadrant pain, cervix pain, or abdominal pain

5. History No previous history of abnormal bleeding episodes Medications
Benadryl
Zofran
No known allergies
Sickle Cell Trait

6. Sickle Cell Trait Genotype AS Heterozygous
Two alleles are codominant
Produce both normal (Hgb A) and abnormal (Hgb S) hemoglobin

7. Physical Examination Vaginal/Cervical Exam Abdominal assessment
Biophysical Profile
8/8 (normal BP = 8-10)
Triplet A (breech), Triplet B (oblique), Triplet C (transverse)
The doctor orders the following laboratory tests:
Complete Blood Count with Differential
Routine Coagulation
Liver Function
Macroscopic and Microscopic Urinalysis

8. Laboratory findings

9. Complete Blood Count Test Result Reference Range WBC 9.1 x 1000/uL
RBC
4.4 M/uL
M/uL
RDW
13.8 %
/5 %
Hemoglobin
14.3 g/dL
g/dL
Hematocrit
41.1 % %
MCHC
24.7 g/dL
g/dL
MCH
32.2 pg pg
MCV
93 fL
78-94 fL
Platelet Count
60 x 1000/uL
x 1000/uL
MPV
9.7 fL fL
NRBC
11 NRBC/100 WBC
0-1 NRBC/100 WBC

10. Differential Test Result Reference Range Neutrophils 69 % 38-71 %
Lymphocytes
21 %
14-46 %
Monocytes
8 %
2-15 %
Eosinophils
1 %
0-5 %
Basophils
0-2 %
ANC
6.3 x 1000/uL
x 1000/uL
ALC
1.9 x 1000/uL
x 1000/uL

11. Schistocytes Fragments of RBCs
Irregularly shaped with two pointed ends
Microcytic with absent central pallor
Indication of mechanical damage

12. Routine Coagulation Test Result Reference Range PT 11.8 seconds
INR
1.07 seconds
seconds
Fibrinogen
337 mg/dL
mg/dL

13. General Chemistry Test Result Reference Range Glucose 59 mg/dL
Urea Nitrogen (BUN)
26 mg/dL
7-20 mg/dL
Creatinine
2.2 mg/dL
mg/dL
GFR (NAA)
25 mL/min/1.73m2
>60 mL/min/1.73m2
GFR (AA)
31 mL/min/1.73m2
BUN/Creatinine Ratio
11.8
Sodium
140 mmol/L
mmol/L
Chloride
105 mmol/L
mmol/L
Potassium
4.8 mmol/L
mmol/L
Total CO2
14.6 mmol/L
mmol/L
Anion Gap 20
7-16
Calcium
9.5 mg/dL
mg/dL

14. General Chemistry Test Result Reference Range Uric Acid 14.2 mg/dL
Ammonia
42 umol/L
11-35 umol/L
Total Protein
6.4 g/dL
g/dL
Albumin
3.1 g/dL
g/dL
Globulin
3.3 g/dL
g/dL
Albumin/Globulin Ratio
0.9
Total Bilirubin
3.36 mg/dL
<1.20 mg/dL
LDH
624 U/L
U/L
AST
1,140 U/L
0-34 U/L
ALT
768 U/L
ALP
536 U/L
U/L

15. Urinalysis Macroscopic
Test
Result
Reference Range
Opacity
Cloudy
Clear
Color
Yellow
Specific Gravity
1.013 pH
6.0
Protein 3+
Negative
Glucose
Ketones
Blood
Moderate
Bilirubin
Leukocyte Esterase
Positive
Nitrites
Urobilinogen
0.2 EU/DL
≤2.0 EU/DL

16. Urinalysis Microscopic
Test
Result
Reference Range
Hyaline Casts
3/LPF
0-3/LPF
Epithelial Casts
Few
None-Few
WBC/HPF
23/HPF
0-5/HPF
RBC/HPF
8/HPF
Bacteria
Many

17. diagnosis

18. HELLP SYNDROME

19. HELLP Syndrome
Hemolysis
Elevated Liver Enzymes
Low Platelet Count

20. Hemolysis Hemolysis due to microangiopathic hemolytic anemia
Intravascular hemolysis
Schistocytes
The diagnosis of hemolysis is additionally supported by:
Unconjugated bilirubin
Lactate dehydrogenase
Haptoglobin

21. Elevated Liver Enzymes
Aspartate aminotransferase
Alanine aminotransferase
Low Platelets
Decreased platelets due to increased consumption

22. Complete and Partial HELLP Syndrome
Complete HELLP Syndrome
Requires the presence of all three major componenets
Hemolysis
Elevated liver enzymes
Low platelet count
Partial HELLP Syndrome
Much less severe condition
Consists of a combination of two out of three elements of the triad

23. Confirmatory Abnormal Results
Test
Result
Reference Range
Blood Pressure
144/90
120/80
Protein (Urinalysis) 3+
Negative
Total Bilirubin
3.36 mg/dL
<1.20 mg/dL
Albumin
3.1 g/dL
g/dL
Lactate Dehydrogenase
624 U/L
U/L
Hypertension
Proteinuria
Elevated total bilirubin
Low albumin
Enhanced levels of lactate dehydrogenase

24. Pathogenesis Unidentified Periconceptional conditions
Fetal and placental genotype
Women at risk of developing HELLP Syndrome are of advanced maternal age and are anticipating delivering twins or higher-order multiples

25. Epidemiology
Occurs in about 1 in every 1,000 pregnancies and in about 10-20% of pregnant women with severe preeclampsia
Mortality rate is estimated 3-5% for the mother and 9-24% for the fetus(es)
Rate of recurrent HELLP Syndrome is 19-27%

26. Differential diagnoses

27. Acute Fatty Liver of Pregnancy
Characterized by:
Microangiopathic hemolytic anemia
Thrombocytopenia
Corrupt metabolism of fatty acids
Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency

28. Disseminated Intravascular Coagulation
Characterized by:
Widespread activation of the clotting cascade
Blood clots form in the small blood vessels yielding compromised tissue blood flow and eventually organ damage
Consumptive coagulopathy
Typically develops secondary to obstetrical issues of pregnancy such as preeclampsia

29. Thrombotic Thrombocytopenic Purpura
Characterized by:
Microangiopathic hemolytic anemia
Thrombocytopenia
Neurological abnormalities

30. Hemolytic Uremic Syndrome
Characterized by:
Renal failure
Thrombocytopenia
Thrombotic microangiopathic hemolytic anemia
Commonly associated with other disorders such as E. coli 0157:H7 due to shiga-like toxin
Development of HUS occurs during the post-partum period following childbirth

31. Differential Diagnoses
Finding
HELLP
AFLP
DIC
TTP
HUS
Hemolysis Y
Abnormal Liver Function Tests N
Abnormal Renal Function Tests
Thrombocytopenia
Abnormal PT/PTT

32. Treatment Immediate delivery of the fetus(es)
Delivery should be expedited by caesarean section without hesitation to avoid worsening symptoms of HELLP Syndrome
Supportive care is highly encouraged to control hypertension and seizures post-delivery

33. ROTEM Rotational thromboelastometry Impedance aggregometry
Clotting time
Clot formation
Clot stability
Lysis

34. ROTEM

35. Prognosis HELLP Syndrome is a clinical entity of difficult diagnosis
The onset of HELLP Syndrome is rapid
Signs and symptoms of preeclampsia usually subside within 6 months after delivery

36. Conclusion
HELLP Syndrome secondary to preeclampsia was resolved by immediate delivery of the fetuses
Post-delivery monitoring
Risk of recurrence
Pre-conceptional counseling

37. Multiple Choice Questions
1. HELLP Syndrome possesses all of the following laboratory findings except:
A. Thrombocytopenia
B. Prolonged PT/PTT
C. Elevated lactate dehydrogenase
D. Abnormal peripheral blood smear with schistocytes
2. Pregnant women found to have abnormal liver function tests, hemolysis, and a NORMAL platelet count are further tested to confirm the diagnosis of:
A. Partial HELLP Syndrome
B. Complete HELLP Syndrome
C. Hemolytic Uremic Syndrome
D. Disseminated Intravascular Hemolysis
3. Which of the following laboratory results are most common in HELLP Syndrome?
A. Low AST and ALT
B. Elevated platelet count
C. Increased serum bilirubin
D. Increased urobilinogen in macroscopic urinalysis

38. Citations
1. Aloizos S, Aravosita P, Gourgiotis S, Kanna E, Liakos N, Mystakelli C, Seretis C. HELLP syndrome: understanding and management of a pregnancy-specific disease. US National Library of Medicine May; 33(4):
2. Bakay K, Güven D, Kocak I, Ustün C. A review of HELLP syndrome. Open Journal of Obstetrics and Gynecology September; 2:
3. Baxter JK, Weinstein L. HELLP syndrome: the state of the art. Obstetrical and Gynecological Survey. 2004; 59(12):
4. Biswas SC, Dey R, Lahiri S, Rakshit A, Roy BR, Saha MM. A study to detect HELLP syndrome and partial HELLP syndrome among preeclamptic mothers and their impact on fetomaternal outcome. US National Library of Medicine. 2011;
5. Ciortea R, Costin N, Mihu D, Seicean A. HELLP syndrome: a mulisystemic disorder. Clinic of Obstetrics Gynecology. 2001;
6. Donald F, Satpathy HK. HELLP syndrome. The Journal of Obstetrics and Gynecology January; 59(1):
7. George JN, McMinn JR. Evaluation of women With clinically suspected thrombotic thrombocytopenia purpura hemolytic uremic syndrome during pregnancy. Journal of Clinical Apheresis. 2001; 16:
8. Sibai B. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. High-Risk Pregnancy Series: An Expert’s View May; 103(5):
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