Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 34-month-old black boy who had contracted acquired immunodeficiency syndrome from his mother presented with fever, vomiting, and cough. He was cachectic, hypertonic, and developmentally delayed. A brain computed tomography scan revealed masses in the left frontal horn, subependymal, and periventricular regions; secondary edema; and hydrocephalus. The differential diagnosis was cerebral lymphoma versus toxoplasmosis. The patient had disseminated Mycobacterium avium-intracellulare infection, lymphoid interstitial pneumonitis, as well as Pseudomonas and Klebsiella pneumonia. He died of respiratory insufficiency 53 days after admission. The autopsy confirmed a primary cerebral B-cell lymphoma, large cell type, which was positive for Epstein-Barr virus, latent phase, by in situ hybridization. Primary central nervous system lymphomas are rare in children, in contrast to adults. To our knowledge, only five well-documented cases of primary cerebral lymphomas in infants and children with acquired immunodeficiency syndrome have been reported previously. The current study shows that these childhood lymphomas are associated with and presumably caused by Epstein-Barr virus and thus have a pathogenesis similar to that of primary central nervous system lymphomas in adults.
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PMID:Epstein-Barr virus-associated primary central nervous system lymphoma in a child with the acquired immunodeficiency syndrome. A case report and review of the literature. 943 21

Unusual cytomegalovirus (CMV)-related complications were seen after autologous stem cell transplantation (SCT) in a 50-year-old patient with diffuse large B cell lymphoma. One month after SCT, the patient developed life-threatening upper gastrointestinal tract (GIT) bleeding with several episodes of hemorrhagic shock. Endoscopy and subsequent explorative laparotomy revealed deep-seated bleeding ulcers containing intracellular CMV inclusion bodies distributed extensively in the GIT, from the lower esophagus to the small bowel. Later, she developed gradual loss of consciousness with communicating hydrocephalus which was possibly secondary to CMV-induced ventriculitis. She recovered completely after insertion of a ventriculostomy and subsequent V-P shunt.
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PMID:Unusual cytomegalovirus complications after autologous stem cell transplantation for large B cell lymphoma: massive gastrointestinal hemorrhage followed by a communicating hydrocephalus. 1218 Jan 20

We describe the clinicopathological features of a patient with an autopsy-proven primary CNS lymphoma, who had a relatively long remission period after onset. A 61-year-old man experienced disorientation and gait disturbance. A ventriculoperitoneal shunt operation was performed, based on the diagnosis of hydrocephalus due to aqueductal stenosis, after which his symptoms subsided. Three months later, T2-weighted magnetic resonance imaging (MRI) revealed high-intensity lesions in the corpus callosum and tectum. However, he remained asymptomatic for the next three and a half years, during which periodic MRI studies constantly detected the lesions. At the age of 65 years, he suffered respiratory and consciousness disturbances, and his general condition gradually deteriorated. MRI studies disclosed that the callosal lesion had spontaneously disappeared, whereas the tectal lesion had developed to become an enhanced linear lesion with a tendency to spread on the dorsal side of the brain stem. Examination of the cerebrospinal fluid disclosed a markedly elevated beta2-microglobulin content, and a tentative diagnosis of malignant lymphoma was made. Steroid pulse therapy had little effect, and the patient died four years after presentation. Examination at autopsy disclosed a malignant, large B-cell lymphoma that had diffusely infiltrated the cerebrum and brain stem. In the corpus callosum, a small number of residual lymphoma cells were seen around the vessels. Therefore, the initially detected lesions in the corpus callosum and tectum might have been attributable to lymphoma, and the unusual clinical and radiological features of this case provide further information that might aid in diagnosis and help to ensure prompt treatment.
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PMID:A primary CNS lymphoma in spontaneous remission for 3.5 years after initial detection of the lesions by MRI. 1460 29

Though rare, primary intracranial tumors can present with Parkinsonian symptoms, and diagnosis can be delayed unless there is a high index of suspicion. We herein present an 81-year-old man who was seen in our neurology clinic due to acute onset of unsteady gait and altered consciousness. Parkinsonism was initially diagnosed because of the typical manifestations. Levodopa was prescribed; however, there was a limited effect on his symptoms. Upon detail history and neurological examination, left sided hemiparesis was disclosed. Cerebral imaging studies revealed a solid mass over the right infratentorial para-midbrain area leading to reactive obstructive hydrocephalus. Work-up including chest and abdominal CT scanning, upper and lower GI endoscopy, and tumor marker studies failed to uncover any abnormalities. A neurosurgeon was consulted and a shunt procedure and biopsy of the infratentorial mass were performed. Histopathological examination of the biopsy tissue revealed tumor diffusely intermixed with large cells consistent with large B-cell lymphoma. The patient and his family declined further treatment. Though rare, cerebral tumors can present with Parkinsonian features and represent a diagnostic challenge. Clinicians should be aware of the possibility of cerebral neoplasms causing Parkinsonism, and include them in the differential diagnosis, especially for patients presenting with atypical Parkinsonian features, or those not responsive to initial therapy.
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PMID:Cerebral infratentorial large B-cell lymphoma presenting as Parkinsonism. 2020 13

We report a young 15-year-old boy with 6 months history of headache, vomiting, and seizure. He underwent septostomy followed by right ventriculoperitoneal shunt for obstructive hydrocephalus and was managed with empirical antituberculosis treatment. Magnetic resonance imaging (MRI) revealed solid, nodular, enhancing masses in bilateral lateral ventricles and 4(th) ventricle. Surgical biopsy from 4(th) ventricular lesion confirmed a B-cell lymphoma. Staging evaluation with MRI positron emission tomography and bone marrow biopsy were normal suggesting an intraventricular primary central nervous system lymphoma.
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PMID:Primary intraventricular central nervous system lymphoma in an immunocompetent patient. 2696 54

Sequence variants in CRB2 cause a syndrome with greatly elevated maternal serum alpha-fetoprotein and amniotic fluid alpha-fetoprotein levels, cerebral ventriculomegaly and renal findings similar to Finnish congenital nephrosis. All reported patients have been homozygotes or compound heterozygotes for sequence variants in the Crumbs, Drosophila, Homolog of, 2 (CRB2) genes. Variants affecting CRB2 function have also been identified in four families with steroid resistant nephrotic syndrome, but without any other known systemic findings. We ascertained five, previously unreported individuals with biallelic variants in CRB2 that were predicted to affect function. We compiled the clinical features of reported cases and reviewed available literature for cases with features suggestive of CRB2-related syndrome in order to better understand the phenotypic and genotypic manifestations. Phenotypic analyses showed that ventriculomegaly was a common clinical manifestation (9/11 confirmed cases), in contrast to the original reports, in which patients were ascertained due to renal disease. Two children had minor eye findings and one was diagnosed with a B-cell lymphoma. Further genetic analysis identified one family with two affected siblings who were both heterozygous for a variant in NPHS2 predicted to affect function and separate families with sequence variants in NPHS4 and BBS7 in addition to the CRB2 variants. Our report expands the clinical phenotype of CRB2-related syndrome and establishes ventriculomegaly and hydrocephalus as frequent manifestations. We found additional sequence variants in genes involved in kidney development and ciliopathies in patients with CRB2-related syndrome, suggesting that these variants may modify the phenotype.
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PMID:Expansion of phenotype and genotypic data in CRB2-related syndrome. 2700 16

As there are multiple factors causing hydrocephalus subsequent to intraventricular hemorrhage (IVH), it is difficult to achieve the best treatment effect using a single drug alone. In the present study, the protective effect of combination treatment with granulocyte-colony stimulating factor (G-CSF) and lithium chloride against hydrocephalus after IVH was investigated. A total of 130 adult male Sprague-Dawley rats were divided into five groups, including the IVH control, G-CSF treatment, lithium chloride treatment, combination treatment and sham surgery groups. An IVH rat model was established in order to examine the effect of combination treatment on hydrocephalus incidence. A TUNEL assay was performed to detect neuronal apoptosis in the five groups. In addition, the protein expression levels of B-cell lymphoma 2 (Bcl-2) and Bcl-2-associated X protein (Bax) were detected by western blot analysis. The differentiation of nerve cells in the brain tissue obtained from the five rat groups was also determined with double immunofluorescence staining. The results demonstrated that administration of G-CSF or lithium chloride alone was able to only partly relieve the incidence of hydrocephalus after IVH. By contrast, combination treatment with G-CSF and lithium chloride significantly attenuated the development of hydrocephalus following IVH. TUNEL assay showed that neuronal apoptosis was significantly reduced by the combination treatment with G-CSF and lithium chloride. Furthermore, the expression of Bcl-2 was upregulated, whereas Bax expression was downregulated in the combination treatment group. The results also detected the highest expression of BrdU/GFAP, BrdU/NeuN and BrdU/PSA-NCAM in the combination treatment group. In conclusion, the combination of endogenous neural stem cell mobilization (using G-CSF) and lithium chloride treatment resulted in highly reduced incidence of hydrocephalus after IVH by inhibiting neuronal apoptosis.
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PMID:Combination of endogenous neural stem cell mobilization and lithium chloride treatment for hydrocephalus following intraventricular hemorrhage. 2788 49