Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023380 (lethargy)
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A rare, multisystemic intravascular proliferative disorder was identified postmortem in eight cats. The majority of these cats died or were euthanized following episodes of dyspnea, lethargy, and anorexia. Microscopic examination revealed occlusive, intraluminal proliferations of spindle cells within small vessels. The heart was consistently involved, and myocardial dysfunction was the probable cause of illness in all cats. Immunohistochemically, the majority of intravascular cells expressed von Willebrand factor, and a smaller number expressed smooth muscle actin, compatible with a dual population of endothelial cells and pericytes, suggesting a reactive rather than a neoplastic process. Four cases of a similar feline vascular disorder from the veterinary literature are reviewed. The histopathology resembles reactive angioendotheliomatosis in humans, a benign cutaneous intravascular endothelial and pericytic proliferative condition. However, in contrast, this feline disease is multisystemic and fatal. We propose the name "feline systemic reactive angioendotheliomatosis" for this unique, idiopathic disorder of domestic cats.
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PMID:Feline systemic reactive angioendotheliomatosis: eight cases and literature review. 1614 7

A 10-year-old, castrated male domestic short-haired cat was presented with an acute history of seizures, lethargy, anorexia, vomiting, and dyspnea. Magnetic resonance imaging of the brain showed multifocal areas of gray matter T2-weighted hyperintensity. The lesions did not enhance with intravenous contrast. The cat was diagnosed at necropsy with feline systemic reactive angioendotheliomatosis, a rare vascular proliferative disorder for which a treatment has not yet been identified. This report is the first to describe associated magnetic resonance imaging changes for this disease.
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PMID:IMAGING DIAGNOSIS-MAGNETIC RESONANCE IMAGING FINDINGS IN A CAT WITH SYSTEMIC REACTIVE ANGIOENDOTHELIOMATOSIS. 2714 46

A 3-year-old domestic shorthair cat presented with lethargy and anorexia. A blood test showed regenerative anaemia and thrombocytopenia. Thoracic radiographs showed a small amount of pleural effusion. The cat did not respond to treatment and died on the fifth day. Necropsy revealed moderate pericardial effusion, and multifocal coalescing haemorrhages were observed on both atria. Histological analysis revealed that the most severe lesions were located on the heart. Numerous arterioles supplying the heart were partially to completely filled with plump spindle cells that often formed glomerulus-like arrangements within the lumen. Similar vascular proliferative lesions were also found in the liver, pancreas and kidney. Immunohistochemical analysis showed that these intraluminal proliferative spindle cells were positive for anti-von Willebrand factor (vWF). Strongly positive antismooth muscle actin staining was observed at the periphery of these intraluminal proliferations (comprising arteriolar smooth muscle) and certain intraluminal cells (pericytes). The intraluminal thrombi were also positive for vWF. Those thrombi were confirmed as platelet thrombi by phosphotungstic acid haematoxylin and Masson's trichrome staining. These results were consistent with feline systemic reactive angioendotheliomatosis.
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PMID:A case of feline systemic reactive angioendotheliomatosis. 2849 48

Intravascular large B-cell lymphoma (IVLBCL) is a rare extra-nodal B-cell lymphoma that proliferates within small/intermediate blood vessels and capillaries while sparing large blood vessels and organ parenchyma. Clinical presentation is highly variable and may include B symptoms, neurological deficits, and/or cutaneous findings. The diagnosis of IVLBCL is difficult due to multiorgan involvement and nonspecific symptoms. We describe the case of a 68-year-old male who presented with progressive weakness, confusion, and falls. He had a past medical history of liver cirrhosis secondary to Wilson's disease. Physical exam and laboratory results revealed a lethargic man with jaundice, hepatic encephalopathy, and abnormal liver/kidney function tests. He expired after a short hospital course in the setting of hepatic and renal failure. Postmortem examination revealed large neoplastic lymphoid cells involving multiple organ blood vessels; however skin and neurologic involvement was absent. The neoplastic cells demonstrated B-cells positive for CD5, rendering a diagnosis of IVLBCL. Our case represents the occurrence of IVLBCL with CD5-positivity in a patient with Wilson's disease, diagnosed at autopsy demonstrating the challenging nature of diagnosing IVLBCL.
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PMID:CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson's Disease: Case Report and Review of the Literature. 3064 58