Thalassemia and Moyamoya Syndrome: Epidemiology and long-term outcome

Das, Shambaditya; Ray, Biman Kanti; Pandit, Alak; Ghosh, Ritwik; Chakraborty, Arka Prava; Dubey, Souvik

doi:10.1016/j.ejpn.2022.10.002

Cited by 2 publications

(1 citation statement)

References 37 publications

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“…14 The pathophysiology of HbH-CS alpha thalassemia shares some aspects with that of SCD, suggesting that patients with HbH-CS alpha thalassemia may be at though successful medical treatments with chronic transfusion therapy, antiplatelet therapy, and hydroxyurea have been described in patients with beta thalassemia. 15 We have observed a high prevalence of silent cerebral infarcts in our patients with non-deletional HbH disease (data unpublished), but the observation of moyamoya vasculopathy was surprising. It is of particular interest that our patient has both epidemiologic risk factors for moyamoya disease as well as a condition whose pathophysiology could theoretically give rise to moyamoya syndrome.…”

Section: E T T E R T O T H E E D I T O R Moyamoya Vasculopathy In Hem...mentioning

confidence: 68%

Moyamoya vasculopathy in hemoglobin H Constant Spring: Epidemiological coincidence or pathological consequence?

Chok

Maamari

McCartney

et al. 2023

Pediatric Blood & Cancer

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Section: E T T E R T O T H E E D I T O R Moyamoya Vasculopathy In Hem...mentioning

confidence: 68%

Moyamoya vasculopathy in hemoglobin H Constant Spring: Epidemiological coincidence or pathological consequence?

Chok

Maamari

McCartney

et al. 2023

Pediatric Blood & Cancer

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Moyamoya Syndrome in a Child with Beta-Thalassemia Trait: A Case Report with Review of Literature

Kumar,

Singh,

Aggarwal

2024

Indian Pediatrics Case Reports

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Background: The association of moyamoya angiopathy with hematological conditions such as sickle cell anemia and beta-thalassemia major or intermedia is well-known, but moyamoya syndrome (MMS) associated with thalassemia trait is hardly reported. We report such a rare association. Clinical Description: A 7-year-old boy presented with right-sided monoparesis with right-sided focal seizures, along with right-sided upper motor neuron type facial nerve palsy. He had a history of recurrent transient episodes of limb weakness, which were unevaluated. Examination revealed pallor and mild splenomegaly. Management and Outcome: Routine investigations revealed microcytic hypochromic anemia with normal serum iron levels. High-performance liquid chromatography was suggestive of beta-thalassemia trait. Contrast-enhanced computerized tomography of the head showed infarction in the territory of the left anterior cerebral artery (ACA) and middle cerebral artery (MCA). Magnetic resonance angiography revealed significantly attenuated bilateral internal carotid arteries in the supraclinoid region with attenuated bilateral ACA and bilateral MCA along with collateral formation suggestive of moyamoya angiopathy. The final diagnosis of MMS secondary to beta-thalassemia trait was made. The child improved with aspirin and supportive management. Conclusion: MMS can be rarely associated with the beta-thalassemia trait. Such an underlying, unsuspected heterozygous beta-thalassemia (trait) may be diagnosed only after the identification of moyamoya disease following a stroke.

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Thalassemia and Moyamoya Syndrome: Epidemiology and long-term outcome

Cited by 2 publications

References 37 publications

Moyamoya vasculopathy in hemoglobin H Constant Spring: Epidemiological coincidence or pathological consequence?

Moyamoya vasculopathy in hemoglobin H Constant Spring: Epidemiological coincidence or pathological consequence?

Moyamoya Syndrome in a Child with Beta-Thalassemia Trait: A Case Report with Review of Literature

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