Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
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We present the first case of successful non-surgical treatment of an internal carotid aneurysm, embedded within a macroprolactinoma. A 53 year old male, with a previous history of Non-Hodgkin's Lymphoma (NHL), presented with severe right sided frontal headache, decreased visual acuity, and ophthalmolplegia due to a third nerve palsy. A CT scan showed a 4.6 by 4.8 cm mass in the pituitary fossa with bony erosion. Initially, it was thought to be a cerebral recurrence of the Non-Hodgkin's disease. Direct questioning revealed a long history of erectile dysfunction with loss of libido. Prolactin at presentation was 537, 200 mU/l and a diagnosis of macroprolactinoma, with apoplexy was made. A subsequent MRI brain confirmed a large macroadenoma with an intra cavernous aneurysm encased by the tumour. A therapeutic dilemma ensued due to the need for urgent decompression of the visual pathways, preferably by surgery. However, in the presence of an intrasellar aneurysm, surgery would have been extremely hazardous. The patient was therefore commenced on cabergoline and rapidly titrated up to 4 mg per week. The aneurysm was treated by endovascular occlusion of the right carotid artery under radiological control. The combination of these therapies, without conventional surgical intervention, resulted in resolution of the third nerve palsy and recovery of visual acuity in the left eye. The diagnosis and management of this condition was challenging and the final outcome, with non-surgical treatment and carotid artery occlusion was satisfactory.
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PMID:A case of macroprolactinoma encasing an internal carotid artery aneurysm, presenting as pituitary apoplexy. 1789 87

AMD3100 given with G-CSF has been shown to mobilize CD34+ cells in non-Hodgkin's lymphoma (NHL), multiple myeloma (MM), and Hodgkin's disease (HD) patients who could not collect sufficient cells for autologous transplant following other mobilization regimens. These poor mobilizers are usually excluded from company-sponsored trials, but have been included in an AMD3100 Single Patient Use protocol, referred to as a Compassionate Use Protocol (CUP). A cohort of 115 data-audited poor mobilizers in CUP was assessed, with the objective being to collect > or =2 x 10(6) CD34+ cells per kg following AMD3100 plus G-CSF mobilization. The rates of successful CD34+ cell collection were similar for patients who previously failed chemotherapy mobilization or cytokine-only mobilization: NHL -- 60.3%, MM -- 71.4% and HD -- 76.5%. Following transplant, median times to neutrophil and PLT engraftment were 11 days and 18 days, respectively. Engraftment was durable. There were no drug-related serious adverse events. Of the adverse events considered related to AMD3100, two (1.6%) were severe (one patient -- headache, one patient -- nightmares). Other AMD3100-related adverse events were mild (84.8%) or moderate (13.6%). The most common AMD3100-related adverse events were gastrointestinal reactions, injection site reactions and paresthesias. AMD3100 plus G-CSF offers a new treatment to collect CD34+ cells for autologous transplant from poor mobilizers, with a high success rate.
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PMID:AMD3100 plus G-CSF can successfully mobilize CD34+ cells from non-Hodgkin's lymphoma, Hodgkin's disease and multiple myeloma patients previously failing mobilization with chemotherapy and/or cytokine treatment: compassionate use data. 1799 19

The presence of IgM paraproteinemia in low-grade lymphomas is usually considered a clinical syndrome known as Waldenstrom's macroglobulinemia (WM). In the WHO classification, WM is associated to lymphoplasmacytic lymphoma (LPL); it is a clinicopathologic entity characterized by a monoclonal expansion of predominantly small B-lymphocytes with variable plasmacytoid differentiation. LPL constitutes less than 5% of all NHL and it is associated with hepatitis C virus infection in 26% of cases. Cells of LPL/WM are B cells positive for monocytic Ig light chains, IgM, pan-B-cell markers, and negative for CD3 and CD103. The t(9;14)(p13;q32) is present in 50% of LPL, and determines PAX-5 over-expression. 6q21 deletion is observed in 42% of cases. LPL occurs in older adults. Clinical presentation usually consists of disseminated disease, but extranodal involvement and leukemic phase are rare. Most WM patients have symptoms attributable to tumour infiltration and/or monoclonal protein. In fact, a monoclonal serum paraprotein of IgM type and hyperviscosity symptoms may occur in more than 20% of cases (WM). Hyperviscosity syndrome is usually manifested by bleeding, blurring or loss of vision, dizziness, headache, and neurologic symptoms. Malignant infiltration of the CNS (Bing-Neel syndrome) is uncommon. LPL/WM is an indolent malignancy that is not usually curable with conventional treatments. The median survival of patients with LPL or WM is 50-60 months, transformation to large cell lymphoma may occur. Stage definition is irrelevant in WM considering that initiation of therapy is decided on the bases of prognostic factors and the development of disease-related symptoms and signs. The main adverse prognostic factors are older age, B symptoms, anemia, low albumin serum levels, raised SGOT, and high beta 2-microglobulin values. Several therapeutic alternatives for newly diagnosed or relapsed LPL/WM are available; however, the best location for every strategy is a matter of investigation. Several new drugs are being assessed in prospective trials. As a significant progress in this field, response criteria and therapeutic recommendations were updated during the Third International Workshop on WM (7-10 October 2004, Paris, France).
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PMID:Lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia. 1849 69

A prospective study of combined modality therapy of non-AIDS related lymphomatous meningitis was carried out. Lymphomatous meningitis is diagnosed increasingly as anti-lymphoma therapies become more effective and result in prolonged patient survival. Twenty-two patients (range 38-69 years; median 60) with lymphomatous meningitis due to metastatic non-AIDS related non-Hodgkins lymphoma were treated. Neurologic presentation included: headache (n=13); cranial neuropathies (n=9); ataxia (n=5); cauda equina syndrome (n=3); myelopathy (n=1); and meningismus (n=1). All patients underwent radiographic evaluation of the extent of central nervous system disease (CNS) followed by radiotherapy (n=8) and sequential intraventricular chemotherapy (methotrexate in 22 patients; cytarabine in 12; thio-TEPA in 5). CNS imaging demonstrated: interrupted CSF now (n=8); intra-cranial subarachnoid nodules (n=2); hydrocephalus (n=2); spinal subarachnoid nodules (2); nerve root enhancement (n=2); and epidural spinal cord compression (n=1). Cytologic responses were seen in 16 patients (73%) to first-, 7 (58%) to second- and 2 (40%) to third-line chemotherapy. Treatment-related toxicity included 14 patients (64%) with aseptic meningitis and 12 patients (55%) with thrombocytopenia or neutropenia (all unrelated to intraventricular chemotherapy). Median survival was 10 months (range: 3-24 months). Fourteen patients (64%) died of their systemic disease, 3 patients (14%) died of progressive lymphomatous meningitis, 4 patients (19%) died of progressive combined systemic disease in lymphomatous meningitis and 1 patient (5%) is disease-free. Fourteen patients (64%) received concurrent systemic chemotherapy and no differences were seen in outcome within this group of patients including 6 patients treated with dose intensive chemotherapy and autologous bone marrow transplantation. Lymphomatous meningitis in patients with non-AIDS related non-Hodgkin's lymphoma may be palliated with combined modality therapy, however, despite the application of standard or dose intensive systemic chemotherapy, therapy remains non-curative.
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PMID:Lymphomatous meningitis in immunocompetent patients. 2159 Feb 44

A 31 years female presented with painful swelling, proptosis of right eyeball and headache of 9 months duration. There were absense of perception of light, pupillary reaction and extraocular movements. Exposure keratopathy with proptosis was observed. She was provisionally diagnosed a case of orbital cellulitis. The computerized tomography revealed lymphoma. Her right orbit was exenterated. The histopathologic examination revealed non-Hodgkin's lymphoma of diffuse small type. Immunohistochemistry demonstrated malignant cells of B cell origin and it was classified as mantle cell lymphoma. Orbital lymphoid tumors typically affect older persons, but occasionally, it can involve middle aged adults also. Mucosa associated lymphoid tumors are the most common affecting eye and orbit, but mantle cell lymphoma is extremely rare.
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PMID:Mantle cell lymphoma of orbit in a middle aged female--a case report. 2174 74

The incidence of non-Hodgkin's lymphoma (NHL) during pregnancy is about 0.8 cases per 100,000 women. We describe a case of a 33-year-old woman with primary brain T-cell NHL who was diagnosed at the 32nd week of gestation. She visited the emergency room complaining of a headache, vomiting and drowsiness. Her pregnancy had been uneventful prior to the admission. Brain magnetic resonance imaging (MRI) revealed a mass at the anterior cranial fossa in the mid liner in the front of the lateral ventricle of the brain. She was hospitalised and monitored regarding her complaints until the foetal lungs matured. During her stay, systemic evaluation revealed no other pathology. She delivered a healthy baby in the 34th week of gestation by Caesarean section and the tumour was evacuated while under the same anaesthesia. She underwent another surgery 12 hours after the first operation because of cerebral herniation. Despite aggressive treatment in the neurosurgical intensive care unit, her condition continued to deteriorate and she died on the tenth postpartum day. Examination of the patient at necropsy revealed no other pathology. The lymph nodes and bone marrow were not involved.
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PMID:Primary brain T-cell lymphoma during pregnancy. 2275 89

A 37-year old general practitioner thought to be in good health presented to the ophthalmology department with palinopsia, headaches and transient visual obscurations. A CT scan revealed a large destructive lesion centred on the occiput and stealth guided excisional biopsy of the occipital lesion showed diffuse large B cell, Non-Hodgkin's lymphoma (NHL) infiltration. To the best of our knowledge this is only the second report of a patient with NHL presenting with palinopsia.
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PMID:Palinopsia as the initial manifestation of non-hodgkin's lymphoma. 2318 89

Primary non-Hodgkin's lymphoma of the cranial vault is extremely rare. This case report presents a 42-year-old man with a painless subcutaneous scalp mass which extended intracranially associated with recent mild headache. Initial computed tomography and magnetic resonance imaging revealed two lesions emanating from the skull. Biopsy revealed a diagnosis of diffuse large B cell lymphoma (DLBCL). A thorough work-up revealed no other point of involvement. This case is concerned about considering lymphoma in the differential diagnosis of calvarial lesions with both intra- and extra cranial extensions but without obvious intervening bony destruction.
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PMID:Primary diffuse large B cell lymphoma of the cranial vault. 2332 70

Lymphoma is seen in up to 30% of patients with X-linked lymphoproliferative disease (XLP), but cerebral vasculitis related with XLP after cure of Burkitt lymphoma is rarely reported. We describe a case of a 5-year-old boy with XLP who developed cerebral vasculitis two years after cure of Burkitt lymphoma. He had Burkitt lymphoma at the age of 3 years and received chemotherapy (non-Hodgkin's lymphoma-Berlin-Frankfurt-Milan-90 protocol plus rituximab), which induced complete remission over the following two years. At the age of 5 years, the patient first developed headache, vomiting, and then intellectual and motorial retrogression. His condition was not improved after anti-infection, dehydration, or dexamethasone therapy. No tumor cells were found in his cerebrospinal fluid. Magnetic resonance imaging showed multiple non-homogeneous, hypodense masses along the bilateral cortex. Pathology after biopsy revealed hyperplasia of neurogliocytes and vessels, accompanied by lymphocyte infiltration but no tumor cell infiltration. Despite aggressive treatment, his cognition and motor functions deteriorated in response to progressive cerebral changes. The patient is presently in a vegetative state. We present this case to inform clinicians of association between lymphoma and immunodeficiency and explore an optimal treatment for lymphoma patients with compromised immune system.
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PMID:Lymphoma and cerebral vasculitis in association with X-linked lymphoproliferative disease. 2381 55

Bilateral trigeminal nerve involvement is a rare presentation of Non-Hodgkin lymphoma (NHL). The trigeminal nerve, also called the fifth cranial nerve, leaves the brainstem and exits the base of the skull to supply sensation to the face. In this case, we present a case of a 63-year-old male patient with a history of NHL and a more recent history of headache and trigeminal neuralgia. The patient underwent PET/CT demonstrating bilateral increased FDG uptake in trigeminal nerves.
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PMID:Bilateral trigeminal nerve recurrence of non-hodgkin lymphoma revealed with FDG PET/CT. 2459 86


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