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Morphological picture in paroxysmal nocturnal hemoglobinuria. Case report
J Rafałowska1, D Dziewulska, B Szyluk
1Department of Neurology, School of Medicine, Warszawa.
Folia Neuropathologica
|January 1, 1994
Summary
Paroxysmal nocturnal hemoglobinuria (PNH) can cause severe neurological complications, including stroke and cerebral venous thrombosis. This case highlights the potential for widespread vascular and inflammatory changes in PNH, leading to fatal outcomes.
Area of Science:
- Neurology
- Hematology
- Pathology
Background:
- Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired blood disorder characterized by complement-mediated hemolysis.
- Neurological manifestations in PNH, though less common, can be severe and include stroke and cerebral venous thrombosis.
Observation:
- A 25-year-old woman with PNH presented with headache, hemiparesis, and speech difficulties, rapidly progressing to unconsciousness.
- Cerebrospinal fluid analysis revealed xanthochromia, increased pleocytosis, and elevated protein levels.
- CT imaging showed an ischemic area with a hemorrhagic focus in the left cerebral hemisphere.
Findings:
- Autopsy revealed a hemorrhagic infarct in the left parietal lobe, superior sagittal sinus thrombosis, and meningeal venous thrombosis.
- Microscopic examination identified vasculitis, bacterial accumulation in blood vessels, and characteristic PNH-related pathological changes.
- Notably, there was a lack of macrophages and GFAP-positive astrocytes in affected areas, suggesting impaired cellular response.
Implications:
- The findings suggest that altered cell membrane composition, specifically low glycosylphosphatidylinositol (GPI) content, may contribute to the weak cellular reactivity observed in PNH.
- This case underscores the critical need for early recognition and management of neurological complications in PNH patients to improve outcomes.