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

An 11-year-old Shetland Sheepdog was presented for exophthalmos caused by a locally extensive, poorly defined mass located behind the right eye. The primary orbital mass was identified by light microscopy and immunohistochemistry as a T-cell rich B-cell lymphoma (TCRBCL) composed predominantly of BLA.36-positive large neoplastic lymphoid cells admixed with fewer CD3- and CD79a-positive small lymphocytes. The dog was treated for lymphoma, but 6 months after presentation it was euthanatized for suspected hepatic and gastrointestinal metastasis. Gross findings revealed an enlarged liver with multiple well-demarcated, randomly distributed 0.1-1.5-cm white nodules, five firm white submucosal jejunal nodules, and ileocecal, mediastinal, and hilar lymphadenopathy. Metastatic liver lesions consisted of sheets of monomorphic large neoplastic lymphoid cells that effaced and expanded portal and centrilobular zones. These cells were morphologically similar to the large neoplastic cells of the original orbital tumor and were CD3-negative and variably BLA.36-positive, consistent with B-cell lineage. Similar cells comprised the jejunal nodules and effaced the lymph nodes. The progression of TCRBCL to a diffuse B-cell lymphoma in this case is consistent with reported human cases and has not been previously reported in the dog.
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PMID:Progression of an orbital T-cell rich B-cell lymphoma to a B-cell lymphoma in a dog. 1105 71

Relapsing polychondritis is characterized by recurrent inflammation of the cartilaginous tissues of the ears, nose, peripheral joints, and the tracheobronchial tree. The eye is also a frequent target organ in relapsing polychondritis, and proptosis is a well-recognized manifestation of eye involvement. Similar to other rheumatologic diseases, an association of relapsing polychondritis with malignancy has been reported. We describe a patient with relapsing polychondritis who presented with exophthalmos. When treatment directed toward control of her underlying disease was only partially effective, further investigation revealed that she had an orbital mucosa-associated lymphoid tissue (MALT)-type B cell lymphoma. We hypothesize that the lymphoma resulted from malignant transformation of the relapsing polychondritis-induced inflammatory pseudotumor and emphasize that neoplastic disease should be considered in the differential diagnosis in patients with relapsing polychondritis presenting with exophthalmos.
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PMID:Orbital mucosa-associated lymphoid tissue (MALT)-type lymphoma in a patient with relapsing polychondritis. 1146 24

Painless unilateral proptosis is a frequent manifestation of numerous orbital neoplastic and non-neoplastic processes. Various mesenchymal tumors of both fibrohistiocytic and vascular origin are well-described causes. Solitary fibrous tumors (SFTs) are rare spindle-cell neoplasms usually found associated with serosal surfaces, especially the pleura, but they have recently been described in a number of extrapleural sites including the orbit. The authors describe the case of an 18-year-old man who presented with a 6-month history of painless proptosis in the right eye. A visible nontender mass in the right supermedial orbit producing ptosis of the upper lid was present. Magnetic resonance imaging (MRI) showed a well-circumscribed soft tissue mass located above the right globe with no obvious invasion of adjacent orbital structures. Uneventful surgical excision through a right frontal-orbitotomy approach was performed. Histological evaluation showed a solid, highly vascular tumor mass composed of spindle cells arranged in short ill-defined fascicles. Intense immunohistochemistry staining for CD34 and B-cell lymphoma 2 (BCL-2) differentiated the lesion from the more common hemangiopericytoma. Though considered benign, local recurrence and extraorbital extension of orbital SFTs have been described. Malignant behavior, including distant metastases, has been documented in as many as 20% of pleural cases with mortality rates as high as 50%. The natural history of this tumor in the orbit is unclear. The authors report the 35th case of orbital solitary fibrous tumor and discuss the differential diagnosis, histopathology, radiological features, and clinical course.
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PMID:Solitary fibrous tumor of the orbit. 1221 91

We report 39 years old man with the history of chronic sinusitis and rhinitis. After tooth extraction he gradually developed unilateral proptosis with ophtalmoplegia and visual loss caused by retroorbital mass which was related to destruction of the adjacent orbital walls, sinuses and base of the skull. During the following month the progressing lung nodules with mediastinal and hilar lymphadenopathy, macular skin lesions, renal insufficiency with proteinuria and anaemia appeared. The diagnosis of Wegener's Granulomatosis (WG) was formed on the base of clinical features and result of pathologic examination of surgical specimen from the paranasal sinuses. The progressive course under the standard immunosuppressive therapy required reevaluation of histologic slides, which resulted with the diagnosis of diffuse large B-cell lymphoma confirmed by the immunohistochemical staining. Administration of CHOP regimen resulted in spectacular regression of all of lesions.
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PMID:[Diffuse large B-cell lymphoma mimicking Wegener's granulomatosis]. 1227 68

Ptosis on its own is an infrequent initial manifestation of orbital lymphoma. Orbital lymphoma usually presents as a palpable mass with proptosis, diplopia, and conjunctival ("salmon-pink") swelling. We report here a 62-year-old female patient who presented with right eye ptosis. The initial imaging study showed an indistinct enlargement of the superior rectus-levator muscle complex. After 3-4 months, ptosis and upward gazing movement were further restricted. The imaging study revealed a definite soft-tissue mass in the superior orbit surrounding the superior rectus-levator muscle complex. A tumor biopsy through anterior orbitotomy revealed a large diffuse B-cell lymphoma. With the experience of this case, we suggest that orbital lymphoma should be included in the differential diagnosis of ptosis accompanied by impairment of levator muscle function.
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PMID:Ptosis as an initial manifestation of orbital lymphoma: a case report. 1667 2

An 82-year-old man with hypothyroidism had vertical diplopia and swelling around his left eye. Visual acuity was 20/20 OD and 20/50 OS. There was moderate blepharoptosis and edema of the left eyelids and superior scleral show of the right eye. The left eye showed 4 mm of proptosis, motility restriction, afferent pupillary defect, and normal optic disc. Orbital MRI revealed enlargement of the left superior and medial rectus muscles without tendinous involvement. These findings were initially suggestive of thyroid orbitopathy. Thyroid function tests were normal. Coronal MRI showed additional superior oblique enlargement and involvement of the levator superioris palpebrae muscle, which are both suggestive of a non-thyroid pathology. Muscle biopsy revealed large B-cell lymphoma. The patient was treated with chemotherapy, immunotherapy, and radiotherapy, with complete tumor control. Orbital lymphoma can simulate thyroid orbitopathy, even in patients with classic "thyroid-like" symptoms and imaging.
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PMID:Orbital lymphoma simulating thyroid orbitopathy. 1685 8

Primary orbital lymphoma is an extremely rare entity; most of the reported cases being MALT lymphoma. Herein we report a case of extranodal primary diffuse large B cell lymphoma in a 17 years old boy who presented with proptosis. The patient responded well to chemotherapy; presently he is in remission with no residual disease.
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PMID:Primary large B-cell lymphoma of the orbit: a case report and review of literature. 1788 41

A 73-year-old woman was examined for palpable orbital masses behind the right upper eyelid and left lower eyelid leading to entropion. Hertel exophthalmometry readings were 6.0 mm in the right eye and 11.0 mm in the left eye with a base of 102 mm. MRI revealed bilateral hypointense orbital soft-tissue masses. Pathologic evaluation of incisional biopsy specimens revealed malignant tissue composed of diffuse, mitotically active, atypical large lymphoid cells positive for CD-20 with immunohistochemical staining, confirming the diagnosis of malignant diffuse large B-cell lymphoma. Systemic survey was negative for extraorbital involvement. After R-CHOP chemotherapy (Rituximab 375 mg/m2 intravenously, Cyclophosphamide 750 mg/m2 intravenously, Doxorubicin 50 mg/m2 intravenously, Vincristine 1.4 mg/m2 intravenously, Prednisolone 100 mg orally), Hertel measurements were 9.0 mm in the right eye and 11.0 mm in the left eye. The mass lesions were totally regressed in follow-up MRI. Although rare, non-Hodgkin lymphoma may present bilaterally as primary orbital lesions and can unexpectedly cause enophthalmos instead of proptosis.
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PMID:Bilateral simultaneous primary orbital lymphoma presenting with unilateral enophthalmos. 1835 23

T cell/histiocyte-rich large B-cell lymphoma (THRBCL) is an uncommon morphologic variant of diffuse large B-cell lymphoma characterized by a minor population of neoplastic B cells existing in a background of predominant reactive T lymphocytes. Diagnosis of this entity is occasionally difficult and would require careful immunohistochemical analysis of the tumor cells, as it may appear similar to other lymphoid diseases, such as nodular lymphocyte-predominant Hodgkin lymphoma, classical Hodgkin lymphoma, and peripheral T-cell lymphoma. The authors report a case of 37-year-old man who presented with a slowly growing, painless proptosis of the right eye with a well-defined mass in the posterosuperior aspect of globe. An incisional biopsy of the mass was performed. Histopathologic examination and immunohistochemical analysis revealed the diagnosis of THRBCL. To the best of the authors' knowledge, this is the first case of primary THRBCL occurring in the orbit.
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PMID:Primary T cell/histiocyte-rich large B-cell lymphoma of the orbit. 2056 65

Proptosis is commonly encountered in clinical practice. We report on a patient with acute proptosis, eyelid swelling, and chemosis, which was initially treated as cellulitis. After radiological and pathological assessments, a diagnosis of large B-cell lymphoma was made. The patient died within 2 months of presentation. Not all patients with proptosis have cellulitis. Proptosis, especially unilateral proptosis, should always lead to a radiological scan in case of malignancy.
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PMID:Diffuse large B-cell lymphoma masquerading as orbital cellulitis. 2113 27


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