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)

The influence of early laparotomy with splenectomy on prognosis in patients with Hodgkin's disease who were regarded as having uncertain prognostic indices (all patients except those with lymphocytic predominance and nodular sclerosis stages IA and IIA with right-sided presentation, stage IV disease, splenomegaly or age greater than 65 years) was evaluated in a randomized trial initiated in Jan. 1973. The patients were treated with total nodal irradiation (excluding the splenic and hepatic areas) and 33 of 69 patients were randomized to laparotomy with splenectomy. No significant difference in relapse-free or overall survival was found between the two groups after a median observation time of 85 months. Progressive or recurrent disease was as common in splenectomized as in non-splenectomized patients. Splenectomized patients with splenic tumour involvement fared worse than those without. It is concluded that diagnostic laparotomy with splenectomy is of no benefit in this clinical setting.
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PMID:Prognostic influence of early diagnostic splenectomy in Hodgkin's disease. A long-term follow-up. 351 42

We describe 20 adult patients with malignant lymphoma with primary presentation in the spleen. The most common presenting symptoms were fever, malaise, and weight loss. Physical examination revealed prominent splenomegaly without palpable lymphadenopathy. Small lymphocytic lymphoma was the most frequent histologic type (11/20), followed by large cell lymphoma and mixed cell lymphoma (3/20 each). Bone marrow involvement was found in ten of 17 patients. At laparotomy, lymph node involvement, usually retroperitoneal, was found in six of 13 patients. There was liver involvement in seven of 15 patients. Follow-up has been relatively short, with an average of 24 months (range, one to 48 months). Four patients died as a result of progressive disease, one died of sepsis after splenectomy, and one died two years after diagnosis of a stroke. The prognosis in primary splenic lymphoma appears to be similar to that in nodal lymphoma.
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PMID:Malignant lymphoma with primary presentation in the spleen. A study of 20 patients. 384 Sep 80

Three patients with known or subsequently proven sarcoidosis had CT of the retroperitoneum because of clinical symptoms referable to this area. Gross splenomegaly was evident in all cases. The liver was massively enlarged in one patient and normal size in two patients. Lymphadenopathy was present in many nodal groups.
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PMID:Retroperitoneal manifestations of sarcoidosis on computed tomography. 396 78

Fifteen patients (11 males, four females; median age 57) manifested a disease characterized by (1) the histopathologic features of Castleman's disease, plasma cell type, in lymph node biopsies; (2) predominantly lymphadenopathic disease, involving multiple, preferentially peripheral nodal groups; (3) varied manifestations of multisystemic involvement (such as constitutional symptoms; splenomegaly and hypergammaglobulinemia; elevated ESR, anemia, and thrombocytopenia; hepatomegaly and altered liver function tests (LFTs); signs of renal disease); and (4) idiopathic nature. Two main patterns of evolution were recognized: persistent, with sustained clinical manifestations, and episodic, with recurrent exacerbations and remissions. Seventy-three percent of patients had infectious complications, and 27% developed malignancies. Complete remissions were obtained occasionally with antineoplastic agents and with splenectomy but not with glucocorticosteroids alone. The median survival time is 30 months; 60% of patients have died. Median follow-up in the six surviving patients is 97+ months. A review of 50 cases in the literature revealed similar clinical and laboratory features. Despite some similarities with autoimmune diseases, the main features of this process seem to best fit a hyperplastic-dysplastic lymphoid disorder in a setting of immunoregulatory deficit.
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PMID:A systemic lymphoproliferative disorder with morphologic features of Castleman's disease: clinical findings and clinicopathologic correlations in 15 patients. 403 67

Cyclophosphamide (Cy; 150 mg/kg) was administered (i.p.) to groups of Sprague-Dawley rats, followed two days later by immunization with ovalbumin (OVA). From that time, cyclosporin A (CsA; 25 mg/kg) or its vehicle was given (p.o.) for a further 13 days. Control animals tested 14 days after immunization, showed strong Arthus-like and modest delayed-type skin reactions to OVA, in contrast to almost total inhibition in animals tested with Cy, CsA or both. Similar effects were observed with respect to serum anti-OVA antibody levels. Despite itself producing lymphopenia, CsA had no additional effect on the lymphocyte depletion caused by Cy. Both drugs, either alone or in combination, caused neutrophilia and monocytosis. An additional eosinophilia due to Cy was prevented by CsA. Cy induced splenomegaly, nodal extramedullary haemopoiesis and increases in both tissue eosinophils and marrow neutrophils. There was also lymphoid depletion in both spleen and lymph nodes which was enhanced by CsA. Thymic lymphoid atrophy was found only when CsA was given. Despite the powerful immunosuppressive properties of both drugs, detailed biochemical and structural analyses showed no other synergistic toxicity, apart from modest hepatic abnormalities. In particular, there was no enhancement of the nephrotoxicity of CsA.
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PMID:Immunosuppressive activity and toxicity of cyclosporin A in rats pretreated with high dose cyclophosphamide. 408 81

The term lymphogranulomatosis X (LgX) designates a clinicopathological entity of unknown etiology ("X"), which was first described by Forster and Moeschlin in 1954. LgX includes the "immunoblastic lymphadenopathy" of Lukes and to a large extent the "angioimmunoblastic lymphadenopathy" of Rappaport (except for the cases with active germinal centers), but in LgX there is another morphologic variant not mentioned either by Lukes or by Rappaport. To establish the morphologic diagnosis of LgX three typical changes of the affected lymph nodes are needed: effacement of the nodal architecture, absence of active germinal centers, and markedly increased proliferation of epithelioid venules. Cases with only partial effacement of nodal architecture, and especially with active germinal centers, were considered hyperimmune reactions (HR). This "hyperimmune reaction" may be an early stage of LgX: 4 of 39 cases showed later transformation into LgX. Based on cytologic aspects, five different variants of LgX are distinguished: immunoblastic predominance, plasma cell predominance, mixed cell type, epithelioid cell predominance and lymphocytic predominance. Case history and clinical picture of the five variants of LgX (172 patients) are reported and compared with HR (37 patients). In LgX the age peak is in the 7th decade; the youngest patient was 16 years old. HR are seen in children as well as in elderly people, the mean age being 49 years. In LgX males predominate and in HR females. In LgX the disorder is usually more generalized than in HR (80% versus 46% with generalized lymph node enlargement, 69% versus 24% with hepatomegaly and 62% versus 27% with splenomegaly). Skin involvement and high sedimentation rate are less frequent in HR. In LgX a Coombs-positive anemia is occasionally found; its origin is "aplastic" rather than hemolytic. Based on the data presented, some reflections relative to the etiology and pathogenesis of LgX are presented. Rubella virus should be considered a possible etiologic agent. In most cases with drug hypersensitivity, allergic reactions to drugs appear only in the course of the illness; in these cases drugs are ruled out as an etiologic factor. Occasionally, an augmented number of azurophil granulated lymphocytes (suppressor T-cells?) is observed in the blood, a fact that could be a pointer to the pathogenesis of LgX and possibly explain the high incidence of infections seen in this disorder.
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PMID:[History and clinical picture of lymphogranulomatosis X (including (angio)immunoblastic lymphadenopathy]. 726 65

We report 26 elderly patients (median age 68.3 years) who met diagnostic criteria for B-cell chronic lymphocytic leukaemia (B-CLL) but whose lymphocytes lacked CD5 expression. Haematological and clinical features of this CD5- series were compared with those of 333 CD5+ B-CLL patients from the same institute. No significant differences were observed regarding peripheral blood (PB) and bone marrow (BM) lymphocytosis, Hb level, platelet count, incidence of adenomegaly, hepatomegaly or splenomegaly or diffuse BM pattern. Due to an absence of nodal enlargements or to general clinical condition, lymph node biopsy was performed in only three patients, while spleen histology was examined in two cases following splenectomy. All histological results confirmed the clinical diagnosis of CLL. The distribution of the CD5- subjects according to the different staging categories proposed by Rai, Binet and Mandelli was similar to that of CD5+ subjects. Ten patients received standard chemotherapy with Chlorambucil (CHL) and Prednisone (PDN). All achieved partial remission, although one of these patients later died of disease progression; 80 months after diagnosis. We conclude that rare cases of CD5- lymphocytosis fulfilling all criteria for B-CLL may occur. Haematological and clinical features at presentation and the response to conventional treatment with Chlorambucil support our hypothesis of considering this disease as a less frequent subgroup of B-CLL.
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PMID:CD5 negative lymphocytosis mimicking typical B-chronic lymphocytic leukaemia. Description of 26 cases. 769 97

The existence of primary nodal marginal zone lymphomas (MZL) is controversial, as is their relationship to putative extranodal counterparts. Most nodal lymphomas with monocytoid B cell/marginal zone differentiation exhibit the morphologic and immunophenotypical characteristics of extranodal MALT-lymphomas. Splenic marginal zone lymphoma (SMZL) is also of putative marginal zone derivation, but it differs immunophenotypically from MALT lymphoma. To clarify the relationship between nodal and extranodal MZLs and to investigate the possible existence of a nodal variant of SMZL, 36 MZL initially considered to be primary nodal neoplasms were examined. Other low-grade lymphomas with marginal zone differentiation were excluded (small lymphocytic lymphoma/chronic lymphocytic leukemia [SLL/CLL], follicular lymphoma, and mantle cell lymphoma). Six nodal MZLs showed morphologic and phenotypic characteristics similar to those of SMZL, whereas 30 tumors were more similar to MALT-type lymphomas. The six tumors with SMZL features showed a polymorphic infiltrate surrounding residual germinal centers with absent or very attenuated mantle cuffs. These lymphomas were IgD positive (6/6) but cyclin D1 (0/5), CD5 (0/6), and CD23 (0/6) negative. Five of these patients came for treatment in stage I or II. No patient manifested splenomegaly, peripheral blood, and/or bone marrow infiltration either at diagnosis or during follow-up. Lymph nodes from 30 patients with MALT-type features showed a perisinusoidal and perivascular infiltration of monocytoid/centrocytoid cells and residual germinal centers with a relatively well-preserved mantle cuff. The neoplastic cells were negative for IgD (0/17), cyclin D1 (0/8), and CD5 (0/12). Seven of 16 (44%) patients with a detailed history and clinical follow-up had evidence of extranodal lymphoma. These observations suggest that most nodal B cell lymphomas with marginal zone differentiation are of the MALT type and that they are frequently associated with an extranodal component. In addition, a primary nodal counterpart of splenic MZL also exists, and may occur in the absence of splenomegaly.
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PMID:Primary nodal marginal zone lymphomas of splenic and MALT type. 1068 Sep 7

Six patients had blood and bone marrow manifestations characterized by the presence of morphologically immature or blastic B-lineage lymphoid cells expressing CD5 antigen. The median patient age was 70 years, and the male-to-female ratio was 5:1. The presence or degree of lymphadenopathy and splenomegaly was variable among this group at staging evaluation, although two patients did not have these features. One patient had an antecedent diagnosis of classical nodal mantle cell lymphoma, without prior morphologic blood or bone marrow involvement. Other patients lacked a history of underlying lymphoproliferative disorders. The median white blood cell count was 120 x 10(9)/L. Most patients had thrombocytopenia, whereas only one patient had neutropenia at presentation. Leukemic peripheral blood cells in these six cases were small to medium in size with fine or granular nuclear chromatin and small or inconspicuous nucleoli. The pattern of marrow involvement was interstitial or diffuse, with cells showing immature nuclear features resembling acute leukemia or blastic lymphoma. All tumors demonstrated a consistent immunophenotype of B-cell lineage, surface immunoglobulin positivity, and CD5 antigen expression. The progenitor cell-associated markers CD34 and TdT were not expressed, and CD23 antigen was either negative (three of four cases) or only weakly present (one of four cases). The presence of a karyotypic t(11;14)(q13;q32) was documented in one tumor, whereas two other cases had BCL-1 gene rearrangements by either polymerase chain reaction or Southern blot analysis. Cyclin D1 mRNA overexpression was noted in three of four cases tested. This patient group was characterized by very poor overall survival (median, 3 months; range, 0.5 to 6 months). The aggregate clinical, pathologic, and genetic data in these unusual cases are consistent with de novo or predominant leukemic presentations of blastic mantle cell lymphoma. Accurate diagnosis in such cases is greatly facilitated by cytogenetic studies or the demonstration of BCL-1/cyclin D1 abnormalities.
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PMID:Blastic mantle cell leukemia: an unusual presentation of blastic mantle cell lymphoma. 1126 37

SMZL has been included in the REAL classification of lymphoid neoplasms (1994) as an entity. It affects middle aged adults presenting with splenomegaly, mild lymphocytosis and in 40% of cases with the presence of monoclonal paraprotein. Our material includes 10 patients (6 women + 4 men). In all patients bone marrow biopsy was the initial material for histopathological examination. With characteristic intertrabecular nodular interstitial infiltrates of CD20+ small lymphocytes and corresponding clinical data (splenomegaly, lymphocytosis in peripheral blood and sporadic elevation of IgM levels) it was very suggestive of SMZL diagnosis. Splenectomy was the treatment of choice. The spleen showed micronodular white foci in all cases. Morphologically the pattern of white pulp involvement was observed with lymphoma infiltrates as well as small foci in the red pulp. The white pulp showed follicles surrounded by a wide marginal zone, resembling reactive splenic follicles. A corona-like rim consisted of medium size cells with abundant clear cytoplasm, resembling monocytoid B cells, often with plasmacytoid differentiation, or centrocyte-like cells. Smaller aggregates of these cells were present in the red pulp. Lymphoma cells were CD20+, bcl-2 and IgM+. In all cases monoclonal kappa light chains were found. Although SMZL is a disseminated lymphoma at diagnosis, its course is indolent and splenectomy is the treatment of choice. Clinical and histopathological differential diagnoses include hairy cell leukemia and secondary involvement of the spleen by infiltrates of nodal B-cell lymphomas.
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PMID:Primary splenic B-cell lymphoma (marginal zone lymphoma). Clinicopathological evaluation of 10 cases. 1124 89


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