Morphological picture in paroxysmal nocturnal hemoglobinuria. Case report. 1994

J Rafałowska, and D Dziewulska, and B Szyluk, and J Wieczorek
Department of Neurology, School of Medicine, Warszawa.

25-year-old woman with paroxysmal nocturnal hemoglobinuria was admitted to the hospital because of headache, progressing right hemiparesis and speech disorders. Several days later patient lost consciousness. Cerebrospinal fluid was xanthochromic with increased pleocytosis and protein level. CT-scan revealed ischemic area with hemorrhagic focus within left cerebral hemisphere. Patient died 3 weeks after the admission. Brain section revealed hemorrhagic infarct in the cortex of the left parietal lobe, thrombosis of the superior sagittal sinus and "respiratory brain" changes. Microscopic examination revealed meningeal venous thrombosis, hemorrhagic infarct, vasculitis, abundant accumulation of bacteria within blood vessels, and other pathological changes such as petechiae, perivascular exudates and small, round areas composed of acellular fibrillary network. There were no macrophages and GFAP-positive astrocytes in any of these areas. Authors suggest that weak cell reactivity may be connected with alterations in cell membranes, mainly low phosphatidylinositol (GPI) content.

UI MeSH Term Description Entries
D007839 Functional Laterality Behavioral manifestations of cerebral dominance in which there is preferential use and superior functioning of either the left or the right side, as in the preferred use of the right hand or right foot. Ambidexterity,Behavioral Laterality,Handedness,Laterality of Motor Control,Mirror Writing,Laterality, Behavioral,Laterality, Functional,Mirror Writings,Motor Control Laterality,Writing, Mirror,Writings, Mirror
D008576 Meningeal Arteries Arteries which supply the dura mater. Arteries, Meningeal,Artery, Meningeal,Meningeal Artery
D002540 Cerebral Cortex The thin layer of GRAY MATTER on the surface of the CEREBRAL HEMISPHERES that develops from the TELENCEPHALON and folds into gyri and sulci. It reaches its highest development in humans and is responsible for intellectual faculties and higher mental functions. Allocortex,Archipallium,Cortex Cerebri,Cortical Plate,Paleocortex,Periallocortex,Allocortices,Archipalliums,Cerebral Cortices,Cortex Cerebrus,Cortex, Cerebral,Cortical Plates,Paleocortices,Periallocortices,Plate, Cortical
D002545 Brain Ischemia Localized reduction of blood flow to brain tissue due to arterial obstruction or systemic hypoperfusion. This frequently occurs in conjunction with brain hypoxia (HYPOXIA, BRAIN). Prolonged ischemia is associated with BRAIN INFARCTION. Cerebral Ischemia,Ischemic Encephalopathy,Encephalopathy, Ischemic,Ischemia, Cerebral,Brain Ischemias,Cerebral Ischemias,Ischemia, Brain,Ischemias, Cerebral,Ischemic Encephalopathies
D005260 Female Females
D006457 Hemoglobinuria, Paroxysmal A condition characterized by the recurrence of HEMOGLOBINURIA caused by intravascular HEMOLYSIS. In cases occurring upon cold exposure (paroxysmal cold hemoglobinuria), usually after infections, there is a circulating antibody which is also a cold hemolysin. In cases occurring during or after sleep (paroxysmal nocturnal hemoglobinuria), the clonal hematopoietic stem cells exhibit a global deficiency of cell membrane proteins. Paroxysmal Cold Hemoglobinuria,Paroxysmal Nocturnal Hemoglobinuria,Marchiafava-Micheli Syndrome,Paroxysmal Hemoglobinuria,Paroxysmal Hemoglobinuria, Cold,Paroxysmal Hemoglobinuria, Nocturnal,Cold Paroxysmal Hemoglobinuria,Hemoglobinuria, Cold Paroxysmal,Hemoglobinuria, Nocturnal Paroxysmal,Hemoglobinuria, Paroxysmal Cold,Hemoglobinuria, Paroxysmal Nocturnal,Marchiafava Micheli Syndrome,Nocturnal Paroxysmal Hemoglobinuria,Syndrome, Marchiafava-Micheli
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013345 Subarachnoid Hemorrhage Bleeding into the intracranial or spinal SUBARACHNOID SPACE, most resulting from INTRACRANIAL ANEURYSM rupture. It can occur after traumatic injuries (SUBARACHNOID HEMORRHAGE, TRAUMATIC). Clinical features include HEADACHE; NAUSEA; VOMITING, nuchal rigidity, variable neurological deficits and reduced mental status. Hemorrhage, Subarachnoid,Perinatal Subarachnoid Hemorrhage,Subarachnoid Hemorrhage, Aneurysmal,Subarachnoid Hemorrhage, Spontaneous,SAH (Subarachnoid Hemorrhage),Subarachnoid Hemorrhage, Intracranial,Aneurysmal Subarachnoid Hemorrhage,Aneurysmal Subarachnoid Hemorrhages,Hemorrhage, Aneurysmal Subarachnoid,Hemorrhage, Intracranial Subarachnoid,Hemorrhage, Perinatal Subarachnoid,Hemorrhage, Spontaneous Subarachnoid,Hemorrhages, Aneurysmal Subarachnoid,Hemorrhages, Intracranial Subarachnoid,Hemorrhages, Perinatal Subarachnoid,Hemorrhages, Spontaneous Subarachnoid,Hemorrhages, Subarachnoid,Intracranial Subarachnoid Hemorrhage,Intracranial Subarachnoid Hemorrhages,Perinatal Subarachnoid Hemorrhages,SAHs (Subarachnoid Hemorrhage),Spontaneous Subarachnoid Hemorrhage,Spontaneous Subarachnoid Hemorrhages,Subarachnoid Hemorrhage, Perinatal,Subarachnoid Hemorrhages,Subarachnoid Hemorrhages, Aneurysmal,Subarachnoid Hemorrhages, Intracranial,Subarachnoid Hemorrhages, Perinatal,Subarachnoid Hemorrhages, Spontaneous
D013924 Thrombophlebitis Inflammation of a vein associated with a blood clot (THROMBUS). Phlegmasia Alba Dolens,Dolens, Phlegmasia Alba,Thrombophlebitides

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