Blood, Heart, and Mind: A Case of Lymphoma-Triggered Antiphospholipid Syndrome Leading to Recurrent Embolic Strokes
Mohammed Qussay Ali Al-Sabbagh1, Prasanna Venkatesan Eswaradass4, Aliya Rashid2, Arpan Patel3
1Department of neurology, 2Department of Internal Medicine, University of Kansas Medical Center, 3University of Kansas Medical Center, 4University of Kansas Health System
Objective:
To present a case of multifocal cortical embolic infarcts with pancytopenia, being eventually diagnosed with Nonbacterial thrombotic endocarditis (NBTE) secondary to antiphospholipid syndrome (APLA), emphasizing the importance of being cognizant to different hematological causes of embolic stroke of unknown etiology (ESUS).
Background:
APLA is a potential hypercoagulable pathology causing venous and arterial thromboembolism cerebral infarctions. Arterial stroke due to APLA might be due to in situ thrombosis or NBTE. In stroke patients with underlying malignancies or suspected hypercoagulable conditions, APLA can complicate the diagnostic process
Results:
A 60-year-old female with a history of pancytopenia, type 2 diabetes, and recurrent strokes (2 years ago and 2 months ago, both labeled as ESUS) presented with generalized weakness and recurrent falls. During her recent admission for stroke, she was diagnosed to have splenic marginal zone lymphoma by bone marrow biopsy. Her neurological exam revealed confusion, left-sided weakness, and encephalopathy. Initial stroke work up, including CT angiography, A1C, LDL, rhythm monitoring, and TTE were all negative. Patient continued to worsen clinically, and repeat MRI confirmed new multifocal embolic cortical strokes. LP was performed to rule out possible intravascular lymphoma. CSF analysis was normal and negative flow cytometry. TEE revealed a mitral leaflet mass. Comprehensive infectious work up came back negative, but coagulopathy workup was notable for triple-positive APLA (anticardiolipin, beta2 glycoprotein, hex lupus anticoagulant), which was deemed to cause the stroke through NBTE, and projected to be secondary to marginal zone lymphoma.
Conclusions:
The prevalence of APLA in the normal population range from 1% to 5.6%, in newly diagnosed lymphoma, APLA prevalence was 37.5%. Lymphoma triggers APLA by either direct synthesis or immune mediated. This case underscores the importance of performing thorough, yet targeted work up for ESUS patients, especially if recurrent.
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