To describe a case of cerebral venous thrombosis (CVT) as the first manifestation of Ph+ ALL with concomitant secondary antiphospholipid syndrome (APS).

Cerebral venous thrombosis (CVT) represents a rare but catastrophic neurological emergency, accounting for 0.5-1% of all strokes. Autoimmune and hematologic diseases have been reported to be associated with CVT, but lymphoblastic leukemia (ALL) as a first manifestation is extremely uncommon. Thrombotic complications in ALL are predominantly associated with L-asparaginase therapy. The coexistence between antiphospholipid syndrome (APS) with this hematologic malignancy represents another layer of complexity, as both conditions independently increase thrombotic risk through distinct pathophysiologic mechanisms.

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A 38-year-old Ecuadorian female, with a background of preeclampsia in all three pregnancies, presented to the emergency department due to a one-week history of worsening headache, tinnitus, and vertigo. First laboratory findings revealed leukocytosis with predominant lymphocytosis and circulating blasts. Brain CT demonstrated a hyperdense lesion in the left occipital area, later confirmed as thrombosis of the left transverse venous sinus. Hematologic assessment identified Philadelphia chromosome–positive acute lymphoblastic leukemia with a BCR-ABL1 t(9;22) translocation. Immunological tests showed a positive lupus anticoagulant and elevated IgM anticardiolipin antibodies, consistent with secondary antiphospholipid syndrome. The patient was started on therapeutic enoxaparin, corticosteroids, and hydroxychloroquine, followed by specific chemotherapy for ALL. During hospitalization, she remained neurologically stable, and a significant reduction in leukocyte count was achieved within ten days of corticosteroid initiation.

This case demonstrates a rare presentation of Ph+ ALL manifesting initially as CVT, with secondary APS. While thrombotic complications are recognized in ALL, CVT as the presenting feature before chemotherapy initiation is unusual. It’s crucial to have a correct hematologic evaluation in patients presenting with unexplained CVT and severe leukocytosis. The coexistence of Ph+ ALL with secondary APS creates a unique therapeutic challenge requiring coordinated management of malignancy, thrombophilia, and autoimmunity.