Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and pathologic findings in 24 patients with "angio-immunoblastic lymphadenopathy with dysproteinemia" (AILD) are presented. The patients' ages ranged from 44 to 80 years, with a median age of 68 years. The disease has an acute onset. In many respects, the clinical presentation is suggestive of malignant lymphoma. Generalized lymphadenopathy was always present. Hepatomegaly was found in 20 patients, splenomegaly in 17, constitutional symptoms in 20 and skin rashes in nine. Twenty patients had anemia, with positive Coombs' test in eight of 14 tested. Polyclonal hypergammaglobulinemia was found in 17 of 22 patients. Two patterns of evolution were recognizable: (1) long survival (24 to 67 months) without treatment or after the administration of intensive combination chemotherapy; and (2) rapid progression (one to 19 months) regardless of the treatment given. Sixteen patients died; postmortem examination in 10 cases showed the cause of death to be attributable to severe infection in eight patients, to renal disease in one and to cardiovascular disease in one. No evidence of malignant lymphoma was seen in any of these autopsies. Histologically, the disease is systemic, with specific lesions in the lymph nodes. The spleen, liver, bone marrow, skin and lung are also involved, but the changes are less characteristic than in the lymph nodes. In the patients in whom sequential biopsies were performed, a trend toward restoration of the nodal architecture was observed. AILD is a clinical-pathologic entity in a spectrum of yet to be defined immune reactions. The clinical, laboratory and pathologic manifestations of AILD are consistent with an autoimmune disorder, in which a deficiency of the T-cell regulatory functions probably predisposes to an abnormal proliferative and autoaggressive reaction of the B-cell system. Surgical staging procedures do not appear to be indicated. Intensive cytotoxic treatment may be hazardous in some patients, precipitating their death, but long survival after such therapy has been observed in others. Supportive therapy and small doses of steroids appear to be a safer therapeutic approach.
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PMID:Angio-immunoblastic lymphadenopathy. Diagnosis and clinical course. 119 Feb 54

Among 60 patients with AIDS seen at our institution, two had splenic abscesses due to Mycobacterium tuberculosis without pulmonary tuberculosis. In both cases splenic abscess was the first manifestation of AIDS; the patients had prolonged fever and had lost weight and experienced malaise; slight hepatomegaly was noted in both instances and peripheral lymphadenopathy in one. Chest radiography gave normal results in one case and showed hilar lymphadenopathy in the other. Ultrasonographic findings were characteristic: homogeneous hepatomegaly and splenomegaly, with multiple filling defects of variable size. Diagnosis required splenectomy in one case and biopsy of cervical lymph nodes in the other. In both cases Ziehl-Neelsen staining gave positive results; M. tuberculosis grew from a culture of splenic tissue of one patient and from a culture of lymph nodal tissue of the other. There was a rapid response to antituberculous therapy. Splenic tuberculosis seems to be a distinct extrapulmonary entity in patients with AIDS. Ultrasonographic images are useful for diagnosis and follow-up.
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PMID:Splenic abscesses due to Mycobacterium tuberculosis in patients with AIDS. 196 84

There are few data in the literature on the abdominal manifestations of sarcoidosis at computed tomography (CT). To determine whether differences in nodal distribution and appearance can be reliably used to distinguish between sarcoidosis and non-Hodgkin lymphoma (NHL), the authors retrospectively reviewed the abdominal and pelvic CT scans of 16 patients with biopsy-proved sarcoidosis and 20 patients with biopsy-proved NHL. Eleven of the 16 patients with sarcoidosis had abdominal and/or pelvic lymphadenopathy, which was common at all nodal sites except for the retrocrural and pelvic locations. There was a statistically significant lower frequency of retrocrual adenopathy in sarcoidosis than in NHL. Mean nodal size was significantly greater in NHL. Nodes tended to be confluent in NHL and discrete in sarcoidosis. Hepatomegaly was seen in six of the 16 patients (38%) with sarcoidosis and splenomegaly was present in nine of 15 (60%). CT depicted hepatic lesions in only three of eight patients (38%) with biopsy-proved hepatic involvement. Splenic lesions were seen at CT in five of the 15 patients (33%). The authors believe that the overlap in nodal appearance and distribution poses a limitation for use of these criteria in accurate disease characterization.
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PMID:Sarcoidosis: abdominal manifestations at CT. 198 30

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

A 55-year-old woman with an ovarian carcinoid presented with intermittent facial and cervical flushing for 10 years, watery diarrhea for 4 years, and abdominal pain without hepatomegaly. Markedly elevated systemic venous and arterial serotonin levels (830 ng/ml; nl = 50-200 ng/ml) were found. The highest serotonin levels were observed in the superior vena caval system, but serotonin as a marker for tumor localization was inaccurate and led to an unproductive neck exploration. The histological pattern of this tumor contained purely insular elements. No hepatic or nodal metastases were identified and the lesion was unilateral. Substance P levels were elevated in the venous drainage of the left ovary and in retrospect correctly localized the ovarian tumor. This peptide may prove to be another carcinoid tumor marker in addition to serotonin and 5-hydroxyindoleacetic acid. Substance P may also be an important mediator of symptoms in patients with carcinoid syndrome.
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PMID:Substance P in the localization of a carcinoid tumor. 620 86

A 63-year-old man developed generalized lymphadenopathy with skin rashes, fever, hepatomegaly and polyclonal hypergammaglobulinemia, twice, in February 1972 and in June 1979, after taking allopurinol for gout. Cervical lymph node biopsy, performed each time, showed the presence of immunoblasts and plasma cells, effaced nodal structure with involvement of the pericapsular tissue, rich vascularity and numerous mitoses, indicative of angio-immunoblastic lymphadenopathy with dysproteinemia (Frizzera, Moran and Rappaport). The existence of hypersensitivity to drugs, in particular, allopurinol in certain patients was emphasized, and induction of immunoblastic lymphadenopathy with various other therapeutic agents was briefly discussed.
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PMID:A case of angio-immunoblastic lymphadenopathy with dysproteinemia related to allopurinol. 645 13

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

We report a case of non-Hodgkin's lymphoma (NHL) presenting with acute renal failure. A-56-year-old male was admitted to our hospital on October, 1997 with fever and renal dysfunction. Physical examination showed no abnormality except for hepatomegaly. Body surface lymphadenopathy was not observed. Computed tomography (CT) of the abdomen showed markedly enlarged kidneys bilaterally and a mass of soft tissue density, which was considered as a swelling lymph node, around the aortic artery. The renal biopsy revealed parenchymal involvement of the NHL cells without normal tubulo-interstitial structure, but the glomeruli were almost intact. Our case rapidly fell into oliguria and acute renal failure, hence needed hemodialysis. After chemotherapy was performed, his renal function gradually improved and the kidney became smaller on subsequent CT. Unfortunately, the patient happened to suffer from methicillin-resistant staphylococcus aureus (MRSA) infection in a neutropenic state and died. Necropsy revealed recovery of the renal interstitium without residual NHL cells. Renal lymphoma without any other organ or nodal involvement is a rare type of NHL, which considered primary renal lymphoma (PRL). However, we believe this case to have been a result of lymphomatous infiltration of the kidneys in disseminated lymphoma.
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PMID:[A case of non-Hodgkin's lymphoma presenting with acute renal failure diagnosed by renal biopsy]. 1050 45

CD38 is a transmembrane glycoprotein expressed on the surface of leukemic cells in a significant percentage of patients with B-cell chronic lymphocytic leukemia (B-CLL). A recent study suggested that CD38 expression has prognostic value in CLL. Peripheral blood samples from 218 patients with B-CLL were analyzed by flow cytometry for CD38 expression on CD5/19(+) leukemic cells. Various patient characteristics were studied including age, sex, Rai and Binet stages, splenomegaly, hepatomegaly, hemoglobin (Hgb) level, beta-2 microglobulin (beta2M) level in the serum, number of nodal sites involved with disease, and length of survival. The Kaplan-Meier method was used to construct survival curves, and the log-rank statistic was used to compare these curves. CD38 was expressed in 20% or more of leukemic cells in 43% of the patients. Patients with high CD38 expression (20% or more) had significantly shorter survival times (P =.00005). Multivariate analyses showed that CD38 expression is an important prognostic factor associated with high incidence of lymph node involvement (P =.004), lower hemoglobin level (P =.001), hepatomegaly (P =.05), and high beta2M level (P =.00005). CD38 expression identified a group of patients with aggressive disease that was considered by Rai staging to be early-stage disease (Rai stages 0-II). Patients with CD38(+) samples have significantly aggressive disease regardless of their clinical stage. Measurement of CD38 expression by flow cytometry should become a routine test in the evaluation of patients with CLL.
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PMID:CD38 expression as an important prognostic factor in B-cell chronic lymphocytic leukemia. 1215 Jan 58


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