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ANTIPHOSPHOLIPID ANTIBODY SYNDROME

Poornima R Ranka*, Alka S Gupta**, Shashank V Parulekar***
*Third Year Resident; **Associate Prof.; ***Prof.; Dept. of Obstetrics and Gynaecology, Seth GS Medical College, KEM Hospital, Parel, Mumbai - 12.

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A case of antiphospholipid antibody syndrome is being reported which was diagnosed because of its classical history, clinical presentation and its complications in a patient with bad obstetric history, intra-uterine foetal death and thromboembolism in current pregnancy.

Although antiphospholipid antibodies which are nonspecific, may be found in normal persons in 3-5% population, [1] however, these antibodies also have been associated with arterial and venous thromboses and adverse pregnancy outcomes. [2,3]

INTRODUCTION

Antiphospholipid antibody syndrome or APLA syndrome, now called as "PAPS", primary antiphospholipid syndrome [3] is a syndrome consisting of lupus anticoagulant and anticardiolipin antibodies in patient’s serum. Other antibodies also associated with it, but rare are antibodies to phosphotidylserine, phosphotidylethanolamine. [4]

These are antibodies directed against negatively changed phospholipids on the cell membrane. They may be of IgG, IgM or IgA classes, alone or in combination. Lupus anticoagulant is characterized by a prolonged partial thromboplastin time and paradoxically the so-called ‘anticoagulant’ is a powerful thrombotic agent in vivo. [5]

The prevalence of lupus anticoagulant in [4]

1. Low risk population is less than 1%
2. Bad obstetric history - 9.1%
3. Early pre eclampsia - 16%
4. Abruption - 33%
5. Systemic lupus erythematosus - 34%

LAC interferes with platelet function, causing platelet aggregation and thrombosis [6] and also it interferes with endothelial function, causing procoagulant activation and thrombosis. [7,8]

CASE REPORT

Mrs. AG, 24 year, married since 4 years, Gravida3, Para3 Living0 with 28 weeks gestation with sonography showing 20 weeks intrauterine foetal death with preeclampsia came to us on 6/3/98 with left sided upper motor neuron facial palsy and left sided hemiparesis.

She had a bad obstetric history with previous two macerated still births in early third trimester and now again a intrauterine foetal death. She presented with preeclampsia; a BP of 150/100 mm Hg and presence of urine albumin and oedema feet.

On clinical examination

CVS	Loud S1 and mid diastolic murmur
CNS	Left upper motor neuron facial palsy left sided weakness - grade 2/5. Suggestive of right sided middle cerebral artery infarct probably thrombotic in origin
PA	20 weeks uterus
PV	Cervix tubular / closed

Her investigations were

 

Hb / CBC	within normal range
PTTK	Prolonged	Control - 13 secs. Patient - 25 secs
PT	Prolonged	Control - 14 secs. Patient - 36 secs
CT scan	Right sided middle cerebral artery infarct - thrombotic in origin.

(As shown in Fig. 1 and Fig. 2)

Fig 1:	Shows CT scan with Right sided middle cerebral artery infarct. Marker showing hypodense area suggestive of infarct, also seen are the ventricles.

 

Fig 2:	Same infarct, but at a higher level of scan.

- 2D echo - Moderate mitral stenosis 1.3 cm2 area.

Patient was started on inj. heparin 5000 IU intramuscular followed by 1000 IU/hour as an i.v. infusion. Warfarin also was started and PT and APTT were monitored.

Diagnosis of APLA syndrome was established and patient was later given low dose aspirin and dexamethasone. Patient spontaneously aborted a 350 gm macerated foetus on date 6/3/98. She aborted completely.

Patient recovered uneventfully and went home after physiotherapy with instructions to continue low dose aspirin and dexamethasone and to get evaluated before planning the next pregnancy.

DISCUSSION

Primary antiphospholipid antibody syndrome can present in a classical manner as seen in our case or it can be present and be totally asymptomatic. [9]

The various clinical presentations associated with lupus anticoagulant antibody are: [10]

• Recurrent pregnancy loss
• Unexplained second or third trimester loss
• Early onset severe preeclampsia
• Arterial or venous thrombosis
• Unexplained foetal growth restriction
• Prolonged coagulation studies
• Autoimmune diseases
• Cardiac valvar diseases
• Neurological disorders
• Thrombocytopenia.

Our patient a case of LAC-APLA syndrome was given heparin and warfarin for prevention of stroke and later low dose aspirin and dexamethasone for chronic treatment of APLA syndrome. [11,12] Such women who have had thromboembolism requires therapeutic anticoagulation during pregnancy and pueperium in next pregnancy. [13]

Unfortunately patient had not taken adequate treatment prior to current pregnancy and have had poor antenatal care.

Early diagnosis could have prevented middle cerebral artery infarct, pregnancy loss, preeclampsia and also her cardiac valvar lesion as there was no history of rheumatic heart disease on direct questioning.

Various treatment regimes for APLA syndrome have been described like:

Low dose steroids, aspirin, heparin, immunoglobulin, azathioprine and plasma pheresis. [14-20]

This case has been reported to remind the readers that such clinical presentations alone or in combinations should always evoke the suspicion of APLA or PAP syndrome as they are associated with poor maternal and foetal outcome.

ACKNOWLEDGEMENT

We would like to thank our Dean of Seth GS Medical College, KEM Hospital for permitting us to publish this interesting case.

REFERENCES

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