Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe two patients with low-grade and one patient with mixed low- and high-grade B-cell lymphoma of mucosa associated lymphoid tissue (MALT) type arising in the large intestine. In each patient the lesion occurred as a single polyp. Two patients presented with rectal bleeding and in one the lesion was discovered incidentally. The bone marrow was uninvolved in all three cases but in the patient with mixed low- and high-grade lymphoma involvement of mesenteric lymph nodes and liver was found. CT scan revealed no lymphadenopathy or splenomegaly in any of the patients. Two patients remain well 9 and 24 months respectively after polypectomy whereas the patient with mixed low- and high-grade lymphoma died 7 days after hemicolectomy due to cardiac failure. These previously undescribed solitary polypoid MALT lymphomas can closely resemble both benign lymphoid polyposis of the colon and lymphomatous polyposis (mantle cell lymphoma). Because of their different behaviour accurate diagnosis of polypoid MALT lymphoma is important.
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PMID:Primary B-cell mucosa-associated lymphoid tissue lymphoma presenting as a solitary colorectal polyp. 804 24

Monoclonal antibody FMC7 detects subgroups of B-cell leukemias that have arisen from cells in late stages of B-cell maturation. FMC7 was studied by flow cytometry on cell samples from 192 patients with a diagnosis of chronic lymphocytic leukemia (CLL) or lymphoma. The leukemic cells from 16 patients were reactive with this antibody. These 16 cases were evaluated for other surface markers, morphology of cells, and clinical characteristics. Of the 16 patients, 14 had cells that strongly expressed surface immunoglobulin (SIg). This is atypical of CLL cells, which characteristically show weak expression of SIg. Eleven cases had kappa and five had lambda light chain. All patients' cells had consistently brighter CD20 expression than that of CD19. Fourteen patients had expression of CD5 on their leukemic cells. One patient had more than 55% prolymphocytes, meeting the criteria of prolymphocytic leukemia (PLL), two patients had CLL in prolymphocytic transformation (CLL/PL), and two other patients were classified as having a paraimmunoblastic variant of small lymphocytic lymphoma based on a high number of paraimmunoblasts and on the histologic features. Another nine patients had immature lymphoid cells distinct from prolymphocytes or paraimmunoblasts on morphologic study. The immature cells were variable in size, and the nuclear chromatin was less clumped than that of prolymphocytes. The histologic diagnoses in four of these cases were consistent with mantle cell lymphoma. Splenomegaly was observed in 11 patients (69%), and 11 patients had advanced Rai 3 or 4 disease. Among 10 patients treated with fludarabine, five responded to therapy. Monoclonal antibody FMC7 is useful for identifying a group of atypical variants of CLL, PLL, and other B-cell lymphomas in leukemic phase that can be easily confused with CLL. Careful attention to the cell morphology and histologic features is important for the differential diagnosis of FMC7-positive, B-cell lymphoproliferative diseases.
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PMID:Detection of subgroups of chronic B-cell leukemias by FMC7 monoclonal antibody. 813 83

Only 1 to 2% of all non-Hodgkin's lymphomas (NHL) present with an enlarged spleen, most of them "small B-cell lymphomas." Recently, several reports have identified these lymphomas as marginal zone B-cell lymphomas. We reviewed 39 cases of NHL presenting with an enlarged spleen without lymphadenopathy, documented by fixed and frozen material. Two were peripheral T-cell lymphomas, four diffuse large B-cell lymphomas, and 14 hairy cell leukemias. The remaining 19 belonged to the "small B-cell" category and constitute the focus of our study. Subtyping was achieved by combining morphology, immunophenotype, and cytogenetic features according to the proposal of the International Lymphoma Study Group; in addition, analysis of the peripheral blood and bone marrow smears was performed adopting the French-American-British (FAB) criteria. From this study, we can conclude that most "small B-cell" NHL of the spleen were either mantle cell lymphomas or marginal zone cell lymphomas and, by peripheral blood analysis, that the mantle cell lymphomas corresponded to intermediate lymphocytic lymphoma and the marginal zone cell lymphomas to splenic lymphomas with villous lymphocytes. As a result, several diagnostic criteria can be proposed that may be helpful in differentiating mantle cell lymphoma from marginal zone cell lymphoma in the spleen.
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PMID:"Small" B-cell non-Hodgkin's lymphomas with splenomegaly at presentation are either mantle cell lymphoma or marginal zone cell lymphoma. A study based on histology, cytology, immunohistochemistry, and cytogenetic analysis. 855 11

Two cases of mantle cell lymphoma are presented. Both patients were women, aged 68 and 56 years, who presented with lymph node enlargement. Each case was initially interpreted as a follicular small cleaved cell lymphoma. Both patients later presented with splenomegaly and recurrent lymphadenopathy. Morphologic and immunologic studies on a parotid swelling in one case and a splenectomy specimen in the other indicated a diagnosis of mantle cell lymphoma. Both women subsequently presented in leukemic phase associated with a poor prognosis, and both patients succumbed soon after. The characteristics, differential diagnosis, and leukemic phase of mantle cell lymphoma are discussed. The importance of appropriate immunohistochemical and flow cytometry analysis in distinguishing mantle cell lymphoma from morphologically similar follicular center cell lymphomas is demonstrated.
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PMID:Leukemic phase of mantle cell lymphoma two case reports and review of the literature. 1204 1

To characterize CD5+ B-cell neoplasms in Japan, where chronic lymphocytic leukemia (CLL) is rare and of different subtypes in comparison with Western countries, we collected 58 cases of CD5+ B-cell lymphomas/leukemias and analyzed their clinicopathologic features. According to the French-American-British (FAB) and standard histologic classification, the cases corresponded to small lymphocytic lymphoma (SLL, group I; n = 22, consisting of CLL, n = 10, CLL/PL, n = 3, and CLLmixed, n = 7); intermediate differentiated lymphoma/mantle cell lymphoma (IDL/MCL, group II, n = 18); and others with CD5-positive lymphomas (group III, n = 18). The CD5+ B-cell lymphomas showed morphologic and prognostic variability among the three groups. The clinical and immunophenotypic features were remarkably consistent in leukemic disease being seen in 73% of all cases, splenomegaly in 63%, and intense CD19, CD20, surface membrane immunogobulin M (SmIgM) or SmIgM and SmIgD, light-chain expression, and no CD10 expression. The median survival time of groups I, II, and III was 7.8, 3.3, and 0.8 years, respectively. These findings suggest that CD5 antigens may serve as valid markers for the prognosis and clinical features of B-cell lymphomas and that CD5+ B-cell lymphomas with an overall poor prognosis occurs at a relatively high frequency in Japan. This also suggests that a combination of immunophenotypic and morphologic features is of value for characterizing CD5+ B-cell neoplasms.
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PMID:CD5-expressing B-cell lymphomas/leukemias: relatively high frequency of CD5+ B-cell lymphomas with an overall poor prognosis in Nagasaki Japan. 872 41

A 69-year-old woman was admitted with generalized lymph node swelling and huge splenomegaly. CD5(+), Sm-IgM (+) and SmIgD (+) lymphocytes were increased in lymph nodes, spleen and bone marrow, and she was diagnosed as having mantle cell lymphoma. A diagnosis of hyper-IgE syndrome was also made, because IgE was markedly increased (174,780 u/ml) and chronic dermatitis, which was often complicated with infection, occurred repeatedly on her extremities. In this case, interleukin-4 was considered to be one of the factors involved in the hyper-IgE syndrome, because increased IgG1 and reduced IgG2 were observed. Immunological abnormality associated with the hyper-IgE syndrome seemed to contribute to the development malignant lymphoma in this case.
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PMID:[Mantle cell lymphoma associated with hyper-IgE syndrome]. 899 29

Spontaneous rupture of the spleen is a rare complication of hematological malignancies, occurring most commonly in patients with acute leukemia, but it has been documented in chronic leukemias and also in lymphomas. We report two patients with histologically and immunohistochemically confirmed mantle cell lymphoma (MCL) who experienced a spontaneous splenic rupture. An 80-year-old woman and a 51-year-old man had a blastoid variant of MCL and responded poorly to conventional treatment. Both patients recovered after splenectomy. The woman died of progressive lymphoma 2 months later. An allogeneic bone marrow transplantation was performed in the man with a good initial result, but an aggressive relapse was seen only 6 months later and he died of progressive lymphoma. In view of our data, we suggest special caution when MCL is complicated by rapid progression and severe splenomegaly. Although it is a rare phenomenon, the risk of splenic rupture should be kept in mind.
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PMID:Spontaneous splenic rupture in two patients with a blastoid variant of mantle cell lymphoma. 903 13

In 1992, after a history of more than two decades a subgroup within the diffuse low-grade B cell lymphomas designated centrocytic lymphoma, lymphocytic lymphoma of intermediate differentiation or mantle zone lymphoma gained general acceptance, now referred to as mantle cell lymphoma. Similarities between these entities were emphasized by identification of rearrangement and overexpression of CCND1 (bcl1/PRAD1) gene in the majority of cases. Unlike in all other non-Hodgkin's lymphomas sex distribution demonstrates a striking preponderance of males over females with a ratio of 3:1. Initial parameters in all published series are advanced disease with generalized lymphadenopathy in 90%, bone marrow infiltration in 60-75%, splenomegaly in 55%, hepatomegaly in 35%, gastrointestinal involvement in about 25% and peripheral blood lymphocytosis in 20-30% of patients. In generalized disease, clinical course is characterized by continuous progression with a median survival probability of 3-4 years within most series. Overall response rates of 56-88% with complete remissions in the range of 9-58% are attainable but relapse occurs predominantly within 20 months. At present there is no evidence that any conventional regimen is curative. Prospective multicenter studies are mandatory to overcome this therapeutic dilemma. Patients suitable for some form of maintenance or consolidation therapy should initially be treated intensively by anthracycline-containing regimens. Whether maintenance with interferon or intermittent chemotherapy including new agents, like purine analogues or (un)conjugated monoclonal antibodies are able to influence overall survival is a matter of (ongoing) investigations. Further experimental approaches arise from antisense oligonucleotides or ribozymes blocking the overexpression of bcl-1 especially in this lymphoma entity. At present high-dose myeloablative consolidation radiochemotherapy followed by stem cell rescue in first remission seems to be the most attractive option in younger patients.
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PMID:Mantle cell lymphoma: diagnostic criteria, clinical aspects and therapeutic problems. 917 43

A 79-year-old male was admitted to our hospital because of general fatigue and night sweat. Physical examination showed generalized superficial lymphadenopathy, marked splenomegaly, and tumors in the conjunctiva and the abdomen. Chest X-ray and computed tomography (CT) revealed pleural effusion and intrathoracic lymphadenopathy. Abdominal ultrasonography and CT showed hepatosplenomegaly and intraperitoneal tumors. Upper gastrointestinal fiberscopy revealed multiple polypoid lesions and ulcers in the duodenum and the stomach. Involvement of relatively small-sized lymphocytes with cleaved nuclei was identified in each biopsied specimen from a cervical lymph node, a tumor in the conjunctiva, gastrointestinal polypoid lesions, and the bone marrow. Surface marker analysis of abnormal lymphocytes in the bone marrow revealed that CD5, CD19, and CD20 were strongly positive, but CD23 was weakly positive. Although (11:14)(q13:q32) translocation was not identified by chromosome analysis of bone marrow cells, Northern blot analysis of bone marrow cells revealed overexpression of the PRAD1 oncogene. Diagnosis of mantle cell lymphoma (MCL) was made. Combination chemotherapy by cyclophosphamide and vincristine was not effective, but etoposide perorally given at a dose of 50 mg per day was effective. In MCL, extranodal involvement of a digestive tract and bone marrow is well known. This case suggests that involvement of multiple organs including lacrimal glands and pleura could be characteristic of MCL cells.
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PMID:[Mantle cell lymphoma with multiple extranodal involvement]. 924 28

We report the case of a 78-year-old man in whom routine physical examination revealed cervical adenopathy and splenomegaly. Peripheral blood showed a normal white blood cell count with an absolute lymphocytosis, which included a population with slightly indented nuclei. Lymph node biopsy showed morphology compatible with mantle cell lymphoma. Bone marrow biopsy showed replacement by a lymphoid proliferation composed of lymphocytes with features similar to those found in the peripheral blood. Immunophenotypic analysis of both peripheral blood and lymph node showed positivity for CD19, CD20 and CD22, with lambda light chain restriction. Tests for CD5 and CD10 were negative. Cytogenetic analysis and polymerase chain reaction studies confirmed the presence of t(11,14) supporting a diagnosis of mantle cell lymphoma. This unusual case of CD5-negative mantle cell lymphoma exemplifies the importance of combined molecular, cytogenetic, and morphologic evaluation when confronted with a lymphoma having an atypical phenotype.
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PMID:CD5 negative diffuse mantle cell lymphoma with splenomegaly and bone marrow involvement. 963 25


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