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)

Forty primary splenic angiosarcomas occurring in 21 men and 19 women, 19-84 years old (median 59 years) are reported. Patients presented with splenomegaly (35 of 38, 92%), abdominal pain (33 of 40, 83%), and systemic symptoms such as fatigue (2 of 40, 5%), fever (4 of 40, 10%), and/or weight loss (16 of 40, 40%). Five (13%) experienced splenic rupture associated with hemoperitoneum. Abnormal laboratory findings included cytopenia (31 of 34, 91%), leukocytosis (8 of 21, 38%), and thrombocytosis (1/39, 3%). Most spleens weighed 500-1,000 g (mean, 1,180 g). The cut splenic surfaces showed multiple hemorrhagic nodules that were frequently associated with infarction, although some had a diffuse pattern of involvement. Microscopically, there were a variety of histologic patterns displayed by the vasoformative component. A honeycomb or sponge-like pattern was common in some, whereas others simulated a cavernous hemangioma or normal splenic sinuses (pseudosinusoidal pattern). Papillary endothelial tufts and solid proliferations of spindled to round to epithelioid cells were also seen. Factor VIII-related antigen was detected in 19 of 23 cases, BMA-120 in 18 of 23, UEA-1 receptor in 18 of 23, and vimentin in 23 of 23 as well as CD68 antigen in 1 of 23 cases. S-100 protein and cytokeratin were not found in any of the 23 cases studied. Metastases in 22 of 32 patients (69%) were to the liver (13 patients), bone or bone marrow (7 patients), lymph nodes (1 patient), and brain (1 patient). Three patients had concomitant malignancies and one had a prior history of a mixed B-cell lymphoma 5 years previously that had been treated with chemotherapy. Follow-up in 38 patients revealed that 30 (79%) are dead at a median interval of 6 months (range 0-48 months) and 8 are alive 5-21 months after diagnosis. These findings indicate that splenic angiosarcoma is an aggressive neoplasm with a high metastatic rate and an abysmal prognosis. Recognition of the wide range of histologic patterns is of diagnostic value but no apparent prognostic significance.
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PMID:Primary angiosarcoma of the spleen. A clinicopathologic study of 40 cases. 780 32

We report a case of intravascular large B-cell lymphoma. A 52-year-old man gradually developed dementia and abnormal behaviors, which were later accompanied by spastic paraplegia and sensory disturbance in his lower limbs. MR imaging of his brain showed high signal intensity lesions on T2 imaging. IMP-SPECT images of the brain showed diffuse reduction of radioisotope uptake. Many skin rashes that looked like senile hemangioma were observed on his body. Several of those were biopsied, and the diagnosis of intravascular large B-cell lymphoma was made because of malignant B lymphocytes filling the vessel lumens in one of the seven biopsy specimens. CHOP therapy was performed and found to be effective for the neurological disorders such as dementia, paraplegia, and sensory disturbance. Our case suggests that skin biopsy for more than one sample of the skin rashes is very important for the diagnosis of intravascular large B-cell lymphoma. CHOP therapy might be effective in this case because of early diagnosis by skin biopsy.
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PMID:[Beneficial effects of CHOP therapy in a case of intravascular large B-cell lymphoma diagnosed by skin biopsy]. 1100 31

An 82-year-old man without notable medical history was admitted to our hospital following subacute deterioration of apettite, disorientation and strange behavior. There was spasticity of the right extremities without weakness. LDH and serum soluble IL2 receptor antibody levels were elevated, and as well as the protein level and IgG levels in the cerebrospinal fluid. CT scanning of the brain revealed a lesion at the left corona radiata. The patient's level of consciousness was worsening, and follow-up study of the brain showed a new lesion in the left occipital lobe. Intravascular lymphomatosis was therefore suspected. We performed a skin biopsy from two typical senile angiomas. In one of these biopsy specimens, a capillary hemangioma was present in the mid-reticular dermis and it was filled with abnormal B cells. The diagnosis of intravascular B cell lymphoma (IVL) was thus established. IVL is a rare subtype of extranodal diffuse large B cell lymphoma with a poor outcome. However, it is recently thought that if the diagnosis is established early, aggressive chemotherapy increases survival. Senile angioma is a skin eruption that is considered prevalent for the most part in elderly people. If a patient is suspected to have IVL, and there is no appropriate site of biopsy, it might be beneficial to try a skin biopsy aiming at senile angiomas for early diagnosis.
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PMID:Intravascular large B cell lymphoma diagnosed by senile angioma biopsy. 1258 32

Intravascular large B cell lymphoma (IVL) is a rare disease characterized by intravascular proliferation of neoplastic mononuclear cells within the vascular lumina. The diagnosis requires skin, liver, renal, meningeal, or brain biopsy, but is rarely made antemortem. We report a 67-year-old man in whom the histological examination of senile angioma-like eruption enabled us to establish the diagnosis of IVL. In a suspected case of IVL, a skin biopsy of the senile angioma-like eruption may be beneficial for an early diagnosis.
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PMID:Senile angioma-like eruption: a skin manifestation of intravascular large B cell lymphoma. 1531 57

Core biopsy has not traditionally been recommended in the study of spleen nodules due to the supposed fragility of this organ leading to a high risk of post-core biopsy complications. A total of 13 patients who presented solid spleen nodules, diffuse splenomegaly, or both on imaging studies (CT, MR, US) were biopsied under ultrasound control with 18G BioPince needles. Cytological (imprints and cytocentrifugates) and histological material were obtained for diagnosis in every case. Malignant lymphomas were the most commonly found pathology (four diffuse large B-cell lymphomas, two follicular lymphomas, one Hodgkin's disease, one B-cell lymphoma, NOS). In addition, there was one littoral-cell angioma, one well-differentiated neuroendocrine carcinoma, metastatic, and one haemangioma. The remaining two cases showed congestive features, and supposed spleen involvement by lymphoma in one of them was ruled out. On follow up, there were no complications related to the core biopsy. Splenectomy was performed in six cases, two diagnostic and four therapeutic. We conclude that core biopsy is a safe and efficient method in the diagnosis of spleen nodules that could be considered in the routine diagnostic algorithm of these lesions.
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PMID:Role of ultrasound-guided core biopsy in the evaluation of spleen pathology. 1690 54

Bilateral ovarian enlargement may reflect benign or malignant processes of the ovary. Benign causes of ovarian enlargement include luteomas, tumors such as mature cystic teratomas, fibrothecomas, cystadenomas and rare conditions including capillary hemangioma and massive edema of the ovaries. Ovarian malignancies include epithelial, stromal and germ-cell tumors. Primary malignancies that may exhibit metastases to the ovaries include gastrointestinal, breast and soft tissue tumors such as lymphoma. We present an unusual case in which a patient presenting with weakness and mild lower abdominal and pelvic pain was noted at sonography to have bilaterally enlarged ovaries with features similar to those of massive ovarian edema as described previously, which has been associated with venous and lymphatic obstruction. Subsequent computerized tomography (CT) imaging depicted a large retroperitoneal tumor, CT-guided biopsy of which revealed diffuse large B cell lymphoma. The patient responded well to chemotherapy with significant shrinkage of the tumor, and reappearance of normal findings on ovarian sonography. This case demonstrates that bilaterally enlarged ovaries may be the first clinical evidence of a large retroperitoneal tumor and that in such cases CT imaging may be warranted.
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PMID:Transient bilateral ovarian enlargement associated with large retroperitoneal lymphoma. 1725 29

Intravascular large B cell lymphoma (IVLBCL) presents various neurological symptoms, and the prognosis frequently deteriorates with a delay in diagnosis. In addition, for the diagnosis of IVLBCL, invasive biopsies are generally performed in main organs, such as the brain. We report a case of IVLBCL in which an early diagnosis was enabled by skin biopsy. The patient in this case had cauda equine syndrome and had developed multiple brain infarctions. She received six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone) treatment and is currently in complete remission. At the macroscopic level, her lesions resembled senile angioma, commonly observed in normal elderly persons. Eruptions of this type are not currently recognised as IVLBCL lesions and might easily be overlooked. In cases in which IVLBCL could be suspected, an active search and biopsy of skin lesions, including an eruption of this type, are useful for early diagnosis and treatment.
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PMID:Intravascular large B cell lymphoma with neurological symptoms diagnosed on the basis of a senile angioma-like eruption. 2168 74

Cavernous angiomas or hemangiomas and malignant lymphomas rarely involve the cavernous sinus. We report the case of a 72-year-old man with right circumorbital pain and right oculomotor nerve dysfunction because of a mass in the right cavernous sinus. It was removed via a transsphenoidal approach and histological examination revealed the mass was a cavernous hemangioma containing atypical large B cells in some sinusoidal vessels; no other evidence of lymphoma was detected on (18)F-2-fluoro-2-deoxy-D-glucose positron-emission tomography or bone marrow biopsy. The patient underwent gamma knife radiosurgery (GKS) of the right cavernous sinus, but no systemic chemotherapy was administered. Although good local control was achieved, the patient developed a systemic diffuse large B-cell lymphoma (DLBCL) 1.5 years after the GKS. The atypical lymphocytes of the cavernous hemangioma and biopsied lymph nodes expressed multiple myeloma oncogene-1 protein. This is a rare case of DLBCL occurring within a cavernous sinus that was diagnosed as hemangioma of the cavernous sinus by neuroimaging, surgical findings, and rapid-freezing histological diagnosis. The case indicates a importance of surgical sampling and detailed histopathological analysis.
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PMID:Diffuse large B-cell lymphoma within a cavernous hemangioma of the cavernous sinus. 2183 78

Intravascular large B-cell lymphoma (IVLBCL) is a rare variant of extranodal malignant large B-cell lymphoma characterized by the selective growth of lymphoma cells within the vessels. It is well known that the diagnosis of IVLBCL is sometimes difficult because of heterogeneity of clinical symptoms and the lack of lymphadenopathy and mass formation. Recently, the usefulness of performing random skin biopsy on healthy-appearing skin in patients with suspected of IVLBCL has been reported. Herein, we review the clinicopathological features of consecutive four cases of IVLBCL and discuss the possible diagnostic strategy, including the usefulness of analyzing biopsy specimen from senile hemangioma, in patients with suspected of IVLBCL. One of 4 cases of IVLBCL was diagnosed by random skin biopsies, which were performed in three cases; however, the random skin biopsy specimens showed no atypical lymphocytes in two cases. Biopsy from senile hemangioma clearly revealed accumulation of atypical lymphoid cells within the dilated vessels of senile hemangioma in the remaining three cases, which led to an ultimate diagnosis of IVLBCL. The results of this study demonstrated that performance and analysis of skin biopsy on senile hemangioma in combination with random skin biopsy is useful for the diagnosis of IVLBCL. The performance of combination skin biopsies on cutaneous hemangioma and normal-appearing skin could facilitate and verify early detection of lymphoma cells in patients suspected of IVLBCL, leading to appropriate therapy and good prognosis.
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PMID:[Proposal of possible diagnostic strategy for intravascular large B-cell lymphoma--usefulness of biopsy from senile hemangioma]. 2256 81

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of B-cell lymphoma characterized by selective growth of clonal B-cells in the lumen of the small vessels of various organs including the liver, spleen, lungs, skin, brain, and kidney. An 86-year-old male presented with weight loss, fever and night sweats (known as B symptoms). Blood examination revealed pancytopenia, high lactate dehydrogenase and high soluble interleukin-2 receptor, suggesting hematopoietic malignancy. However, there were no abnormal hematopoietic cells in the peripheral blood. No lymph node swelling was identified on examination by whole-body computed tomography scan. Therefore, IVLBCL was suspected, and random skin biopsies and a skin biopsy from a senile hemangioma were carried out. A small number of large atypical lymphoid cells resided in the small blood vessels in the deep dermis and subcutaneous tissue of the random skin biopsies, and numerous atypical lymphoid cells were identified in the small vessels of the senile hemangioma. These results suggest the usefulness of skin biopsy from senile hemangiomas in the diagnosis of IVLBCL.
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PMID:Benefits of skin biopsy of senile hemangioma in intravascular large B-cell lymphoma: A case report and review of the literature. 2493 79


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