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Uncommon presentation of a rare disease

“ A Grey case scenario”

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• Mrs. GKD, a 30 year a old housewife in her second pregnancy admitted to THM at 35 weeks of gestation with a twin pregnancy, as she was detected to have high blood pressure at her routine ante natal clinic.

• She has never recorded high blood pressure in her previous clinic visits.

• She has had progressive bilateral ankle swelling of recent onset.

• She didn’t have any other symptoms of pre-eclampsia, such as headache, blurred vision or abdominal pain.

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On systemic inquiry,

She never has had undue exertional dyspnoea.

Her urinary and bowel habits were normal. No frothy urine of note.

Haven't got fever at any point during her pregnancy.

No history of multiple small joint pain, stiffness, oral ulcers or photosensitive rashes.

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Past medical history

She does not give history of DVT in the past.No history of other medical cormordities in her.

Obstetric history

First pregnancy was a miscarriage at POA 7 weeks.

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Story after admission to THM

• She was found to have protein in her urine and underwent emergency LSCS on following day of admission.

• Immediate post-op, she developed Generalized tonic-clonic seizure within couple of hours of section which made her to be admitted to the ICU for close monitoring.

• She became almost anuric after 18 hours of admission to ICU.• She was transferred to THK for further management .

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Diagnosis card

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Summary of investigations at THMTEST Date DI – post partum D2 – post partumFBC Hb 9.0 g/dl 7.5 g/dl

PCV 27% 21%WBC 19900 17100N 84 90L 13 07Plt 100000 cumm3 30000 cumm3

Renal Function

BU 42.4 mg/dl

Scr 130 microm/l 145 microm/lLiver Enzymes

SGOT 133.7 u/l 105 u/l

SGPT 32 u/l 35 u/lAlbumin

38 mg/dl 37 mg/dl

S Billi 10.3mmol/ldl 20.4 mmol/lPT/INR 1.7 1.09APTT - 30 sec.

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Blood picture

Red cells are normocytic and normochromicFragmented RBC seen.Marked neutrophil leucocytosis with mild left shift.Changes may be due to convulsion.Platelets are moderately low

Comment:Low grade microangiopathic haemolysis.HELLP syndrome is likely.

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• Transfer form

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Summary of care given at THM - ICU

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On admission to THK

• She was conscious and rational.• Not dyspnoeic at rest. • Afebrile.• B/L ankle oedema, JVP was not elevated• No pallor, Anicteric, No evidence of mucosal bleeding.• HR – 122 bpm, RR – 20 cycles/min, SpO2 – 99% on oxygen 2 l/min• BP – 160/90 mmHg, • Lungs – Air entry equal• Abdomen –no shifting flank dullness, non tender hepatomegaly• Visual field defect in right eye with marginally reduced acuity.• Other cranial nerves were normal.• Reflexes were exaggerated and plantars upgoing.

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Problem list

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Problem list Acute medical problems1. Anuria in immediate post partum.2. High blood pressure, proteinurea, two GTC episodes in

peripartum3. Scotoma with reduced visual acuity in right eye immediate

post partum4. Thrombocytopaenia, MAHA with elevated liver enzymes

without liver failure.5. Secondary depression

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Other issues,1. Two babies born were deprived of breast

milk and mothers care.

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Differential diagnosis

1. Severe pre eclampsia leading to eclampsia and HELLP syndrome.

2. Thrombotic thrombocytopaenic purpura3. Catastrophic APLS4. SLE, First presentation5. Cerebral venous thrombosis6. ?? Sepsis as a obstetric complication

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Is it HELLP syndrome or TTP ?

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She was taken to ETU – ICU on admission

Dialyzed after admission to THK on D2 post partum

During dialysis two units of blood along with five units of Platelets was transfused.

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Monitoring chart, After admission to THK

Date D2 D3 D4 D5 D6

UOP 145 ml/24h 178ml/24h 235ml/24h 217ml/24h 185ml/24h

SBP 160 mmHg 150mmHg 160mmHg 150mmHg 140mmHg

DBP 110mmHg 100mmHg 100mmHg 90mmHg 90 mmHg

Spo2 97% 99% 98% 98% 99 %

HD HD

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Summation of Investigations

Date D2 after HD D3 D4 18 D5 D6

Hb 8.5 g/dl 6.8 g/dl 10.8 g/dl 8.6 g/dl 9.0 g/dl

PCV 24% 20.5% 28.6 % 24.9% 25.5%

WBC 16500 12000 12300 11400 10800

Plt 59000 cumm3

25000 cumm3

22000 cumm3

15000 cumm3

10000 cumm3

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Blood picture – D2Comment:Microangiopathic haemolytic anaemia ? HELLP syndrome ? DICWith the presence of elevated liver enzymes

HELLP is likely.

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Blood picture – D3

MAHA with thrombocytopeniaNeutrophil leucocytosis with toxic granule

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Blood picture – D4

MAHA with thrombocytopenia withincreasing number of fragmented RBCNeutrophil leucocytosis with toxic granule

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Blood piture – D5

Comment:

Microangiopathic haemolytic anaemia

With the thrombocytopaenia, neurological symptoms and renal shut down, TTP is more likely.

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Liver function test

Date D2 D3 D5

SGOT 89 U/l 62U/l 55U/l

SGPT 34U/l 27U/l 24U/l

Albumin 27 mg/dl 26mg/dl 24mg/dl

T.Billirubin 20.4micromol/l 13.8micromol/l 15.4micromol/l

ALP 223 205 204

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Renal function

Date D3 D4 D5 D6 D7

Scr 166micro/l 241micro/l 175micro/l 258 micro/l 321micro/l

BU 50mg/dl 100mg/dl 43mg/dl 72mg/dl 109 mg/dl

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Clotting profile

Date D3 D4 D7

PT/INR 1.2 1.0 1.0

APTT 30 Sec 28 Sec. 34 sec

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Is it HELLP or TTP ?

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American journal of Obstestric

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Diffrerentiation of HELLP vs TTP (postpartum)

1. ADAMTS 13 Level estimation

2. Renal Biopsy.

Both are not applicable in this clinical scenario.

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Multi disciplinary Approach

Agreed to Go for PEX

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She underwent four cycles of PEX on D7, D8, D 10 and D 12 of post op leading to complete recovery of urine output and partial recovery of vision.

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Blood pcture – D 8

RBC are normocytic normochromic with fragmented red cells and microspherocytes.

Percentage of microspherocytes are reducing in number comparing to previous blood films. White cells shows moderate leucocytosis.

Platelets are low, but increasing in number

? Recovery phase from TTP

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LDH monitoringDate D6 D 11 D 28

LDH 2765 ul/l 807 ul/l 356 ul/l

230-460

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1. Rh factor - < 8 Iu/ml2. ANA – Negative3. Anticardiolipin antibody4. Lupus Anticoagulant – not detected5. CT – Brain – Normal6. Repeated blood cultures were negative.

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• On discharge, her blood pressure was under control with Prazocin and Nifedipine which were tailed off her subsequent clinic visits

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Probable diagnosis

Thrombotic Thrombocytopaenic Purpura, probably

triggered by preeclampsia.

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Areas of uncertainty

1. Duration of PEX

2. Place for corticosteroids

3. Place for newer immunological therapy (Rituximab)

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Take home message

If laboratory and clinical parameters do not normalize promptly in spite of correct treatment for an assumed HELLP syndrome TTP may be the underlying diagnosis.

Do not delay PEX.

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Acknowledgement

1. Dr. W. Kodikara Arachchi.2. Dr. Arosha Dissanayaka.3. Dr. (Mrs.) Malani Mohotti.4. Dr. Theshanthi Gamage.5. Dr. Sanjaya Heiyanthuduwa.6. Dr. Pushpakantha De Silva.