Antiphospholipid Syndrome in A Young Stroke Patient - A Case Report

Antiphospholipid syndrome is an autoimmune clotting disorder characterized by the presence of antiphospholipid antibodies in the setting of thrombosis or systemic lupus erythematosus, pregnancy loss or other complications. The syndrome is diagnosed with clinical and laboratory criteria.^[1] Many, but not all, lupus anticoagulant patients also have elevated levels of IgG or IgM antiphospholipid antibodies. The prevalence of the syndrome in the general population is low and only a small percentage of patients diagnosed with thrombosis have a phospholipid circulating in their blood.^[3] The typical age of onset for primary APS is between 35 and 40 years, and the condition is more prevalent in women than men. The clinical manifestations of the syndrome include venous and arterial thrombosis and embolism, disseminated large and small vessel thrombosis with accompanying multi organ ischemia and infarction, stroke, premature coronary artery disease, and spontaneous pregnancy losses. In the early 1950s, the development of the partial thromboplastin time coagulation test, which used a phospholipid extract of animal brain (cephalin) as a platelet equivalent to accelerate coagulation, led to the recognition of an anticoagulant activity in patients with systemic lupus erythematosus frequently accompanied by BFP(Biologic false positive) - syphilis tests.^[2] This phenomenon, named the “lupus anticoagulant”. The diagnosis of antiphospholipid syndrome requires any of the following clinical and laboratory criteria (Table 1)^[8]

Figure 1: Other Manifestations That Are Associated With APS^[1]

The initial approach to APS treatment involves use of unfractionated heparin or low-molecular-weight heparin (LMWH) in combination with warfarin for 3 to 5 days until the warfarin reaches a therapeutic effect, with an increased international normalized ratio (INR) of 2 to 3. For most patients with definite APS and thrombosis, warfarin will need to be taken indefinitely. For pregnant women with definite APS, subcutaneous LMWH is used; warfarin is resumed in the postpartum period.^[6] For pregnant women with definite APS and a history of prior pregnancy morbidity, low-dose aspirin and LMWH are used in combination.^[7] Patients with either antiphospholipid antibodies or lupus anticoagulant should not receive oestrogen-containing oral contraceptive pills, since these patients may be at higher risk for thrombosis. For patients who have recurrent thrombosis despite warfarin treatment and an INR of 2 to 3, additional intensive therapy is suggested, such as an increase in target INR ranges from 3 to 4 and the addition of LMWH and low-dose aspirin therapy.^[9]Monitoring the INR in patients with antiphospholipid syndrome (APS) can be challenging due to the potential interference of anti-prothrombin antibodies, which can falsely extend the prothrombin time even in the absence of warfarin therapy or lupus anticoagulant, thereby raising the INR. A possible alternative is to monitor factor Xa levels.^[10]

Case Report:

A 27-year-old female of weight 75 kgs, height 5 feet 6 inches, and BMI of 29 kg/m² with no known drug allergies presented to our hospital with a history of daily headaches for the past year and one episode of tonic seizures- 12 days back. She had a recent history of weakness of the left upper and lower limb with loss of speech which recovered after a few hours and also a recent history of a right MCA- territory stroke due to right M1 occlusion for which she was evaluated at a local hospital where the patient was treated with DIVALGRESS ER 250 (Divalproex) for CNS vasculitis and referred to this hospital for further workup and management. Upon admission, the patient was conscious, coherent and revealed a left upper limb pronator drift, mild left hemisensory loss and no motor deficits. The routine laboratory test complete blood count [CBC] was performed which revealed Hb-14.0g/dl, Eosinophils-0.1%, MCV 83.0 fl, platelets- 3.84×10³/mm³, Absolute monocyte count-1.41×10³/mm³, Absolute lymphocyte count-3.47×10³/mm³, Absolute Neutrophil count-19.02×10³/mm³, Erythrocyte sedimentation rate (ESR) was 5.54 mm/hr and serum creatinine- 0.61 mg/dL. Other laboratory investigations mentioned in the tables 2,3,4,5.

Management:

In this report, we described a rare case of an adult female who presented with a history of daily headaches for the past year and episodes of tonic seizures 12 days back. She had a recent history of weakness of the left upper and lower limb with loss of speech which recovered after a few hours and also a recent history of a right MCA- territory stroke due to right M1 occlusion. The physician advised investigations of ANA, ANCA, and APLA which revealed APLA with anti-cardiolipin IgM was positive. Based on the objective evidence & clinical condition of the patient, she was diagnosed with Anti Phospholipid Antibody Syndrome(APS) and treated accordingly. The patient was discharged with these medications (Table 7). The patient and her caretaker were advised on how to proceed with the treatment, and the patient was counseled about drug adherence.

Discharge Medications:

Table 7: ROA (Route of Administration); PO- Per Oral; OD- Omni die (once daily); BD- Bis die (twice daily); APS (Anti phospholipid antibody syndrome); CVA (Cerebrovascular Accident).