Primary Lymphoma of the Brain in a Young Man Whose Brother Died of Hemophagocytic Lymphohistiocytosis: Case Report: Journal of Intellectual and Developmental Disability: Crimson Publishers
Primary
Lymphoma of the Brain in a Young Man Whose Brother Died of Hemophagocytic
Lymphohistiocytosis: Case Report by Eleonora Džoljić*, Tatjana Stošić-Opinćal, Milica Skender-Gazibara and
Vladimir Kostić in Crimson Publishers
We report a case of a 25-year-old male patient with primary aggressive diffuse
large B-cell lymphoma affecting the brain and
PCNSL. Despite one year of medical treatment outcome was lethal.
However, our patient had a relatively longer survival compared to median survival time for
PCNSL. Additionally, he had two older brothers who died at the age of about 11
years. One died of fulminate
malignancy, shortly after pediatric admission, before
the diagnosis could be established. The other one died from genetically
confirmed (perforin mutation/PRFl) HLH. Our patient was heterozygous
carrier of perforin mutation representing the genetic marker for HLH.
Our patient’s father was the carrier of the same mutation but had no symptoms
of any disease.
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