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)

Lymphomas of the marginal spleen zone are an entity recently considered as separate by the International Lymphoma Study Group. There are B-cell non Hodgkin's lymphomas (NHL) of low grade malignancy with a characteristic phenotype that allows to differentiate from mantle lymphomas and other B-cell lymphoproliferative syndromes. The case of a 69-year-old female patient admitted for abdominal pain due to large splenomegaly is reported. Pancytopenia and the presence of atypical large-sized lymphocytes with extensive cytoplasm and a rounded nucleus with indentations, reticulated appearing chromatin and one or several nucleoli were of note in the hemogram. Microscopic examination of the bone marrow demonstrated moderate-degree lymphocytary infiltration with grade I reticulin fibrosis. Laparotomy with splenectomy was performed. White pulp invasion with multifocal infiltration of the red pulp by lymphocytes of the same characteristics as those observed in the peripheral blood and bone marrow were observed on microscopic bone marrow examination. Immunophenotypic study of these lymphocytes was positive for CD19, CD20 and CD22 while being negative for CD5, CD10, CD23, CD25, CD11c and FMC7, the phenotype belonging to the lymphocytes of marginal spleen zone. Following splenectomy the patient recovered hemoperipheral counts and did not undergo additional treatment. The patient died due to septic shock of respiratory origin 4 months later. The clinical, morphologic and immunophenotypic features of marginal spleen zone lymphomas are reported with emphasis on the differences with other B-cell non Hodgkin's lymphomas of low malignancy.
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PMID:[Lymphoma of the marginal zone of the spleen. A case study]. 923 20

In B-chronic lymphoproliferative disorders (B-CLD) adhesion molecules (AM) have been investigated in order to explain the variable biologic behavior and dissemination patterns and to assess their contribution to the differential diagnosis and prognosis of these diseases. The main AM studied either by immunohistochemistry on lymph node sections or by flow cytometry in blood and bone marrow specimens are L-selectin, CD11a/CD18 (LFA-1), CD54 (ICAM-1), CD44 (HCAM), CD11c/CD18 (gp150/95), and CD49d/CD29 (VLA-4). Among B-CLD, hairy-cell leukemia (HCL) and follicular lymphoma (FL) show a uniform AM expression pattern. Thus, HCL is characterized by high CD54, CD44, VLA-4, CD11c, and CD18 and by low or absent CD11a and L-selectin, whereas FL confined to the lymph nodes is characterized by high CD11a, CD18, and CD54 expression. Diffuse growth and dissemination of FL is associated with alteration in the AM profile. Mantle-cell lymphoma (MCL) seems to be characterized by low or absent L-selectin and CD11c and high CD54 expression, especially compared with B-chronic lymphocytic leukemia (B-CLL). B-CLL is the most heterogeneous among all B-CLD with respect to AM expression. In general, low LFA-1 and CD54, high L-selectin and CD44, and variable CD11c characterize B-CLL. Cases with splenomegaly as their prominent feature bear high CD11a, CD18, CD29, and CD11c on the surface of the leukemic cells. Small lymphocytic lymphoma (SLL) shares the same AM phenotype with B-CLL, with the possible exception of LFA-1, which is strongly expressed on SLL cells. LFA-1 and CD54 are more frequently positive in lymphoplasmacytic lymphoma (LPL) as compared with B-CLL. Splenic lymphoma with villous lymphocytes differs from B-CLL by its high LFA-1, VLA-4, and CD54 and low L-selectin expression, whereas its high LFA-1 positivity can differentiate it from HCL. Surface and soluble AM have been investigated as possible prognostic markers in these diseases. Conflicting data exist concerning the prognostic significance of surface AM. However, high soluble (s)CD44 and CD54 levels in B-CLL and non-Hodgkin's lymphomas (NHL) are considered as adverse prognostic factors.
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PMID:Adhesion molecules in B-chronic lymphoproliferative disorders. 1031 87

An 88-year-old Japanese woman with splenomegaly, but without lymphadenopathy, was admitted because of epigastric distress. Laboratory data disclosed an RBC of 310 x 10(4)/microliter, Hb of 10.1 g/dl, Ht of 30.6%, Plt count of 9.8 x 10(4)/microliter, and WBC of 4,470/microliter with 38% abnormal lymphocytes. Peripheral blood films revealed lymphocytes with thin, short cytoplasmic villi, condensed nuclear chromatin, and small nucleoli. The lymphocytes stained negative for tartrate-resistant acid phosphatase. Also, immunophenotyping was positive for expression of the cell surface markers CD19, CD20, IgG, kappa and HLA-DR, but not for CD5, CD10, CD11c, CD23, CD25, CD38, or CD103 antigens. Chromosomal analysis of peripheral blood cells disclosed the 46, XX, del(7), (q32) aberration. A splenectomy was performed simultaneously with partial colon resection because of a mucinous carcinoma found in the transverse colon. Histologic examination of resected spleen tissues revealed a distinctive pattern of white pulp infiltration by lymphoma cells. The histologic findings and clinical data were consistent with the features of splenic lymphoma with circulating villous lymphocytes. Our patient exhibited a relatively benign clinical course, and was being followed on an outpatient basis with no additional therapy.
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PMID:[Splenic lymphoma with villous lymphocytes expressing chromosomal abnormalities]. 1035 43

Hairy cell leukemia-variant (HCL-V) is an extremely rare chronic B-cell lymphoproliferative disorder clinically and morphologically distinct from classic hairy cell leukemia (HCL). HCL-V is thought to represent a hybrid between prolymphocytic leukemia and HCL, the nucleus more closely resembling a prolymphocyte and the cytoplasm a hairy cell. The clinical course of HCL-V is aggressive with short survivals. Since single courses of cladribine have profound activity in HCL, inducing durable complete responses in 91% of patients, we administered cladribine to 4 patients with HCL-V over a 7-year period. During this time interval 357 patients with classic HCL received cladribine at Scripps Clinic. Each patient received cladribine at 0.1 mg/kg per day by continuous intravenous infusion for 7 days, repeated at 28-day intervals depending on response status. The 4 patients ranged in age from 28 to 70. Two presented with B-symptoms, 1 had peripheral adenopathy, and all 4 displayed massive splenomegaly. Peripheral blood counts were notable for lymphocytosis associated with mild anemia and thrombocytopenia. Only 1 of the 4 patients had received prior treatment. Peripheral blood immunophenotypic analysis revealed monoclonal B cells with expression of CD11c in 3 patients, lack of CD25 expression in 3 patients and expression of CD103 in all but 1 patient. The number of cladribine courses administered ranged from two to five. Of these 4 patients, 1 (25%) achieved a complete response and 2 (50%) partial responses, for an overall response rate of 75%. Three patients underwent splenectomy after cladribine. Cladribine is an active agent in HCL-V albeit with a lower response rate than in classic HCL. The role of other treatment modalities, such as splenectomy, interferon-alpha, and 2'-deoxycoformycin, alone or in combination with cladribine awaits further evaluation.
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PMID:Treatment of hairy cell leukemia-variant with cladribine. 1070 59

The presented case is a boy with T-cell acute lymphoblastic leukemia (ALL) with hairy cell (HC) features and monoclonal gammopathy. The disease process had an acute onset and followed a rapid, progressive course. The patient had minimal splenomegaly and bicytopenia, but the bone marrow displayed increased numbers of reticulin fibers. The blasts were positive for tartrate-resistant acid phosphatase (TRAP) and CD11c. Molecular analysis revealed rearrangement of immunoglobulin heavy chain genes and a rearranged T-cell receptor (TcRbeta) beta gene. The patient responded to conventional ALL therapy. Acute T-cell ALL with HC features in childhood has not been reported previously, either alone or in association with monoclonal gammopathy. We propose "T-ALL with hairy cell features" to describe this case.
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PMID:T-ALL with monoclonal gammopathy and hairy cell features. 1099 36

Clinical, hematologic, and immunophenotypic data were studied in 25 dogs with large granular lymphocyte (LGL) lymphocytosis. Primarily large-breed dogs were affected, with an average age at initial diagnosis of 10 years (range 5-14 years). All dogs had persistent (>4 months) LGL lymphocytosis except for three that were euthanized with aggressive disease. Splenomegaly was reported in 12 of 20 dogs in which splenic size was evaluated. The clinical course was heterogeneous and dogs were divided into four groups based on similar clinical and hematologic findings: acute leukemia (3/25), persistent lymphocytosis with anemia (12/25), persistent lymphocytosis without anemia (8/25), and reactive lymphocytosis (2/25). Immunophenotypes varied within groups but were homogeneous among cells from the same patient except in the two dogs classified as reactive LGL lymphocytosis. Analysis of T-cell receptor (TCR) usage identified three main LGL lineages. TCRalphabeta was expressed in 15/25 (60%) cases. TCRgammadelta was expressed in 8/25 (32%) cases, and 2/25 (8%) cases were CD3-, compatible with NK cells. beta2 integrin expression was distinctive. CD11a was consistently expressed, while CD11b was absent. CD11c was expressed only weakly in 16/25 (64%) cases. The leukointegrin alphadbeta2 was highly prevalent on all LGL lineages, being expressed in 23/25 (92%) cases. Prominent involvement of the spleen, relative sparing of bone marrow, an unexpectedly large proportion of gammadelta T-cell LGLs, and the distinctive beta2 integrin expression pattern on diverse lineages of LGLs suggest the disease arises from unique populations of lymphocytes that preferentially localize in the splenic red pulp.
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PMID:Clinical, hematologic, and immunophenotypic characterization of canine large granular lymphocytosis. 1110 53

The diagnosis of hairy cell leukemia (HCL) has traditionally been based on microscopic means. Immunophenotypic analysis of peripheral blood by flow cytometry is not widely recognized as a method for diagnosing HCL, perhaps due to the expectation of low yield of neoplastic cells in patients who are characteristically leukopenic. The abnormal coexpression of CD103, CD25, and intense CD11c and CD20 on monotypic, slightly large B-lymphocytes has previously been shown to be highly characteristic of HCL. We wished to determine if this pattern was valuable in the diagnosis of HCL in leukopenic patients with low levels of neoplastic cells in the peripheral blood. The abnormal immunophenotype above was observed in 25 peripheral blood specimens from patients with unexplained cytopenias or suspected lymphoproliferative processes. Ten of the 25 blood samples exhibited this abnormal phenotype in less than 5% of circulating leukocytes (ranging from <1% to 4%). All 10 patients had other manifestations of HCL, including cytopenias (mean white blood cell count, 1.8 x 10(3)/mm(3); hemoglobin, 11.0 gm/dl; platelets, 74 x 10(3)/mm(3)), splenomegaly, and typical bone marrow morphologic changes. Eight of the 10 patients achieved an excellent response to one course of 2-CDA, with significant improvement of cytopenias (mean white blood cell count: 5.3 x 10(3)/mm(3); hemoglobin: 14.4 g/dl; platelets: 181 x 10(3)/mm(3)) and regression of splenomegaly. One patient had a partial response to alpha interferon and a subsequent complete response to 2-CDA, and one died during treatment. In conclusion, flow cytometric immunophenotyping of peripheral blood is capable of detecting low levels of circulating malignant cells in HCL, even in leukopenic patients. As such, it can be a very useful, non-invasive tool in the diagnosis of this disorder.
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PMID:The diagnosis of hairy cell leukemia can be established by flow cytometric analysis of peripheral blood, even in patients with low levels of circulating malignant cells. 1144 33

A 45-year-old woman was admitted to our hospital in August, 1999. Laboratory data showed a white blood cell count of 5,050/microliter with 78% abnormal lymphocytes, hemoglobin 6.8 g/dl, platelets 4.8 x 10(4)/microliter, and soluble IL-2 receptor 97,600/ml. The abnormal cells were characterized by a hairy appearance under phase contrast microscopy, and showed strong tartrate-resistant acid phosphatase activity. Immunophenotype analysis revealed that these cells were positive for CD11c, CD19 and CD25, and negative for CD5. Bone marrow biopsy showed diffuse proliferation of hairy cells with moderate myelofibrosis. We diagnosed the patient as having European-American-type hairy cell leukemia. Pentostatin was administered at a dose of 5 mg/m2 weekly. After twelve doses, the peripheral blood data returned to the normal range with no hairy cells in the blood or bone marrow, although slight splenomegaly remained. The patient underwent splenectomy in December of the same year, and we were unable to find any hairy cells by histological and immunohistochemical examination. Although most patients with hairy cell leukemia in Japan have the Japanese variant, and the European-American type is rare, pentostatin is as effective as it is for European and American patients.
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PMID:[Successful treatment of hairy cell leukemia with pentostatin]. 1152 43

Hairy-cell leukaemia-variant (HCL-variant) is a rare B-cell disorder which accounts for 10% of HCL cases. It affects elderly or middle-aged males. The main features are splenomegaly, lymphocytosis and cytopenias without monocytopenia. The circulating cells have a morphology intermediate between prolymphocytes and hairy cells. The immunophenotype shows a mature B-cell phenotype with expression of the B-cell antigens CD11c and CD103-but unlike typical HCL the cells are CD25- and HC2-negative. The histology of bone marrow and spleen shows a pattern of infiltration similar to that in HCL. There is no recurrent chromosomal abnormality but complex karyotypes and monoallelic p53 deletion by fluorescence in situ hybridization are common. Patients are resistant to alkylating agents and interferon-alpha (IFN-alpha) and only half achieve partial responses to pentostatin and/or cladribine. Splenectomy results in long-lasting partial responses in over two-thirds of the patients and is a good palliative treatment. Despite the lack of response to most therapies, the clinical course of HCL-variant is chronic. The median survival is 9 years and 42% of patients die of unrelated causes. Transformation to large cell is seen in 6% of patients. The inferior survival in HCL-variant compared with typical HCL cases may reflect the chemotherapy resistance.
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PMID:The variant form of hairy-cell leukaemia. 1267 Apr 64

We report a case of hairy cell leukemia variant developing in a background of polycythemia vera in a 77-year-old man who presented with lymphocytosis and splenomegaly. Classic hairy cell leukemia in a patient with polycythemia vera has been reported previously, but hairy cell leukemia variant arising in a patient with polycythemia vera has never been described to the best of our knowledge. Initial testing of the peripheral blood showed circulating medium to large leukemic cells with large, centrally placed nuclei, each containing a prominent nucleolus, and some cells showed cytoplasmic projections. A bone marrow biopsy had marked myeloid and erythroid hyperplasia and interstitially distributed cells with a fried-egg appearance. We verified a monoclonal B-cell population by flow cytometric analysis, which revealed expression of bright CD11c, CD22, and CD103 expression, and a lack of CD25 expression. The patient received a 5-day course of cladribine and subsequently had a complete remission. Approximately 2 months later, he had a relapse and was treated with pentostatin; however, he had no clinical response and died.
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PMID:Hairy cell leukemia variant developing in a background of polycythemia vera. 1268 4


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