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Henoch-Schönlein purpura with intracerebral hemorrhage
Tadashi Imai1, Hitoshi Okada, Masanori Nanba
1Department of Pediatrics, Kagawa Medical University, Ikenobe 1750-1, Miki, Kitagun 761-0793, Japan. taboimai@kms.ac.jp
Insights
Henoch-Schönlein purpura (HSP) can rarely cause severe brain bleeds (intracerebral hemorrhage). This case highlights a severe bleed in a child with HSP, linked to low factor XIII activity, which was successfully treated.
Area of Science:
- Pediatric Neurology
- Hematology
- Vascular Disorders
Background:
- Henoch-Schönlein purpura (HSP) is a common childhood vasculitis, typically presenting with rash, arthritis, abdominal pain, and glomerulonephritis.
- Intracerebral hemorrhage (ICH) is an extremely rare but devastating complication of HSP.
- The underlying mechanisms for ICH in HSP remain poorly understood.
Observation:
- A 7-year-old girl with Henoch-Schönlein purpura presented with massive intracerebral hemorrhage.
- Cranial CT scan confirmed extensive bleeding in the left parietal and right parieto-temporal-occipital regions.
- Initial assessment revealed no hypertension or general coagulation abnormalities, but markedly reduced factor XIII activity (9%).
Findings:
- The intracerebral hemorrhage in this case is strongly suspected to be a direct consequence of severe factor XIII deficiency.
- Prompt administration of factor XIII concentrate following the onset of ICH prevented further enlargement of the hemorrhagic region.
- This represents the first reported case of HSP with ICH where factor XIII levels were measured and correlated with the event.
Implications:
- This case underscores the critical role of factor XIII in hemostasis and suggests it as a potential factor in HSP-related ICH.
- Measurement of factor XIII activity should be considered in children with HSP presenting with neurological symptoms or suspected intracranial bleeding.
- Early factor XIII replacement therapy may be a crucial intervention for managing ICH in the context of HSP.
Abstract:
We describe a case of Henoch-Schönlein purpura (HSP) with massive intracerebral hemorrhage (ICH) in a 7-year-old-girl. A cranial CT scan revealed extensive ICH in the left parietal region and right parieto-temporal through occipital regions. At the time of ICH onset, hypertension and coagulation abnormality were not observed, but factor XIII activity was markedly reduced 9%. ICH was thought to have resulted from a marked decrease in factor XIII. Factor XIII preparation was administered immediately after the onset of ICH, and enlargement of the hemorrhagic region was not seen. The present case is the only reported case of HSP complicated by ICH in which factor XIII level was measured.