Cerebral Venous Thrombosis in Sickle Cell Trait — a Rare Entity

1Unity Health System, Rochester, NY
2Unity Health System, Rochester, NY
3Unity Health System, Rochester, NY

Meeting: Hospital Medicine 2013, May 16-19, National Harbor, Md.

Abstract number: 498

Case Presentation:

Cerebral venous thrombosis is a rare type of cerebrovascular disease affecting about 5 people per million and accounts for 0.5% of all strokes. It is a challenging condition because of the variability of clinical symptoms and is often unrecognized at the initial presentation. Headache is the most common presenting complaint occurring in about 90% of cases. Our patient is a relatively healthy 49‐year‐old African American woman who presented with severe, right‐sided, 8/10 headache for 5 days duration. Associated symptoms included nausea and vomiting. Patient denied visual symptoms, fever, chills, weakness, numbness, chest pain, palpitation, involuntary movements or loss of consciousness. She does not consume alcohol, smoke or use recreational drugs. She has never been on oral contraceptive medications. Her family history is not significant for any cardiovascular disorders, stroke or malignancy. On admission, her vitals were stable, she was not in acute distress. She appeared mildly dehydrated. Funduscopy was done with no papilledema. Physical examination including cardiovascular, pulmonary, abdominal and central nervous system examination were within normal limits. Blood work including complete blood count, comprehensive metabolic profile, prothrombin time and activated partial prothrombin time were within normal limits. Lumbar puncture showed normal opening pressure, normal cell count, normal protein and normal glucose levels. CT scan of the head revealed abnormal appearing transverse sinus suspicious for possible sinus thrombosis. Hence, an MRV was done which showed transverse and sigmoid sinus thrombosis. Patient was started on IV heparin and Coumadin, heparin was discontinued after 2 days of therapeutic INR. Patient was discharged home after 4 days in the hospital. Coagulation panel including antithrombin III, protein C, protein S, and factor V Leiden were all normal. Hemoglobin electrophoresis was also done which showed that the patient had HgA 56.4%, HgA2 3.8, HgS 39.8. Hence, our patient was diagnosed to have cerebral venous thrombosis due to sickle cell trait.

Discussion:

Sickle cell disease (homozygous) is a well‐known cause of cerebrovascular thrombosis. Very few cases of cerebrovascular thrombosis associated with sickle cell trait(heterozygous) have been reported in literature. The key point here is that we should always look for a cause of the cerebral venous thrombosis even after the patient is stabilized and treated appropriately with intravenous fluids, heparin and anticoagulants.

Conclusions:

Many studies have suggested that the risk of sickling in sickle cell trait with high hemoglobin S approaches that in sickle cell disease. Hemoglobin S values were >36% in the few cases of cerebral venous thrombosis associated with sickle cell trait that were reported in literature. Hemoglobin electrophoresis should be carried out in young African American patients, especially women with unexplained thrombotic phenomenon.

To cite this abstract:

Ramesh N, Suliman S, Alkhoury Z. Cerebral Venous Thrombosis in Sickle Cell Trait — a Rare Entity. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 498. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/cerebral-venous-thrombosis-in-sickle-cell-trait-a-rare-entity/. Accessed September 18, 2019.

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