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

Eight cases of angio-immunoblastic lymphadenopathy were recently diagnosed at the University Hospital of Geneva. The disease was characterized by generalized adenopathy, hepatosplenomegaly, fever, and sometimes skin rash. Six patients had polyclonal dysproteinemia and two displayed autoimmune disorders, e.g. Coombs' positive hemolytic anemia. Lymph node biopsy was a decisive pointer for the diagnosis. Four patients died, two of them from immunoblastic lymphoma. Four other patients are in remission, the longest follow-up being 91 months. Analysis of 213 cases from the literature shows the following features:--Males and females are equally affected, with a predominance after the age of 50.--The onset is characterized by constitutional symptoms (75%) and fever (65%), sometimes following exposure to drugs.--The signs are generalized adenopathy (91%), splenomegaly (66%), hepatomeagly (65%), and skin rash (46).--Polyclonal hypergammaglobulinemia (76%) is found, together with anemia (78%) which is often autoimmune in origin (58%).--The course is often unfavourable and the mortality is over 55%, largely due to opportunistic infections and/or development of immunoblastic lymphoma.
 ...
PMID:[Angioimmunoblastic lymphadenopathy. 8 cases and review of the literature]. 35 86

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.
 ...
PMID:Angio-immunoblastic lymphadenopathy. Diagnosis and clinical course. 119 Feb 54

We present a six-year follow-up of a boy with a novel type of hypolipoproteinemia, with clinical and biochemical features distinct from classical hypoalphalipoproteinemias. There were abnormally low concentrations of total and high-density lipoprotein (HDL) cholesterol, apolipoprotein (apo) B, apo A-I, and apo A-II, and the phospholipids were decreased. The most striking abnormality was an extra fraction containing mainly phospholipids and apo A-I in the HDL3 subfraction. This fraction is reminiscent of concentric 20- to 50-nm-diameter lamellar phospholipid liposomes. Plasma lecithin:cholesterol acyltransferase activity was strongly decreased. We noted a persisting polyclonal hypergammaglobulinemia, hematological abnormalities (hemolytic anemia and thrombocytopenia), and a progressive splenomegaly. After the five-year follow-up, the patient had recurrent severe infections; moderate hematuria and proteinuria developed gradually. Treatment with corticosteroids and immunoglobulins improved thrombocytopenia and hypolipoproteinemia. These clinical and biochemical findings differ from those in the known primary and secondary hypo-alpha-lipoproteinemia syndromes. Although investigation of the relatives suggests a familial predisposition for hypo-alpha-lipoproteinemia, the subject's condition can be regarded as acquired.
 ...
PMID:Acquired hypolipoproteinemia. 158 35

Interleukin 6 (IL-6) is an important regulator of the acute phase response, T cell function, and terminal B cell differentiation. Excessive or inappropriate production of this cytokine may be involved in a variety of autoimmune and neoplastic disorders. To investigate the consequences of dysregulated synthesis of IL-6 in vivo, a high-titer recombinant retroviral vector produced in psi-2 packaging cells was used to introduce the coding sequences of murine IL-6 into mouse hematopoietic cells. Congenitally anemic W/Wv mice reconstituted with bone marrow cells transduced with the retroviral vector developed a syndrome characterized by anemia, transient granulocytosis, hypoalbuminemia, and polyclonal hypergammaglobulinemia, with marked splenomegaly and peripheral lymphadenopathy. Extensive plasma cell infiltration of lymph nodes, spleen, liver, and lung was noted. The similarity of these findings to those of multicentric Castleman's disease, taken together with the observation that lymph nodes from these patients elaborate large amounts of this cytokine, suggest that the inappropriate synthesis of IL-6 has a primary role in the pathogenesis of this systemic lymphoproliferative disorder.
 ...
PMID:Dysregulated interleukin 6 expression produces a syndrome resembling Castleman's disease in mice. 238 5

A retrospective clinical review of 41 patients with chronic myelomonocytic leukemia revealed a median age of 66 years and a male:female ratio of 2.4:1. The disease was preceded by a myelodysplastic syndrome of a different subtype in 24% of the patients and transformed into acute leukemia in 24%. Splenomegaly was present in 54% of the patients and reached massive proportions in 24%. Chromosomal abnormalities occurred in 34% of those studied, most commonly in the younger age group; the most frequent were trisomy 8, monosomy 7, and deletions involving the long arms of chromosomes 20 and X. Polyclonal hypergammaglobulinemia was detected in 47% of the patients in whom serum protein electrophoresis was done. The median survival was 3 years. With use of univariate analysis, the statistically significant prognostic determinants were hemoglobin level, the "modified Bournemouth score," and bone marrow blast cell percentage. When these factors were subjected to a multivariate analysis, only bone marrow blast cell percentage was an independent prognostic determinant. Orally administered hydroxyurea controlled leukocytosis and splenomegaly in some patients without affecting the overall prognosis.
 ...
PMID:Chronic myelomonocytic leukemia: natural history and prognostic determinants. 259 15

In 24 cases of thrombopenic purpura associated with human immunodeficiency virus infection the clinical, immunological and therapeutic features were evaluated. Thrombopenia resulted in clinical manifestations in 20 patients. Splenomegaly was found in only one fourth of patients. Antiplatelet antibodies were found in 9 patients, and thrombopenia was associated with anemia in 37% of cases and with leukopenia in 21%. Bone marrow examination showed megakaryocyte hyperplasia in two thirds of the patients. The major immunological abnormalities were an inverted helper/suppressor T lymphocytes ratio, a reduction in the number of helper T lymphocytes, polyclonal hypergammaglobulinemia, and increased serum concentrations of circulating immunocomplexes. The different therapeutic modalities, steroids, vincristine, danatrol and plasma exchange, resulted in short responses; only two patients had normal platelet counts. The median follow-up was 14 months; during this time three patients fulfilled the criteria of acquired immunodeficiency syndrome.
 ...
PMID:[Thrombopenic purpura associated with human immunodeficiency virus infection. Analysis of 24 cases]. 274 29

A 67-year-old man was admitted in October 1987 with complaints of nausea, headache, dizziness and speech disturbance. Hematological examination showed pancytopenia. Bone marrow aspiration failed with a dry tap. A month later, the second aspiration showed hypocellular marrow containing 18.2% of lymphoma cells. Physical examination showed splenomegaly and lymph node swelling. Polyclonal hypergammaglobulinemia was not observed. A lymph node biopsy exhibited typical histology of immunoblastic lymphadenopathy (IBL)-like T cell lymphoma. Surface marker CD3 and CD4 positive cells were dominant. The patient complained of epigastric pain and occult blood was positive in stool. Gastrofiberscopic examination disclosed well differentiated adenocarcinoma in situ located on a polyp, and polypectomy was performed. Lymphoma was treated with cyclophosphamide, doxorubicin, vinblastine and prednisolone. Splenomegaly and lymph node swelling were reduced in size but the effect was temporary. Thereafter the patient has been treated with cyclophosphamide, doxorubicin, vindesine, prednisolone and etoposide every 3 weeks. This is our first case report of IBL-like T cell lymphoma associated with early gastric cancer.
 ...
PMID:[IBL-like T cell lymphoma associated with early gastric cancer: a case report]. 278 12

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a disease of unknown etiology and pathogenesis. It has the features of hyperimmunity and immune deficiency, and its behavior resembles malignant lymphoma. We report a review of 16 patients with AILD seen at Ramathibodi Hospital from 1982 to 1986. Thirteen patients had fever and seven had pruritus and rashes. Lymphadenopathy was found in all 16 cases; generalized in 14 and localized in 2. Hepatomegaly was present in 14 patients while only 7 had splenomegaly. Laboratory findings included autoimmune hemolytic anemia, lymphocytosis and polyclonal hypergammaglobulinemia. Pulmonary involvement was seen in 5 cases, and bone marrow showed the characteristic features of the disease in 9 cases. Two patients went on to develop diffuse lymphocytic, poorly differentiated lymphoma. Fourteen patients were treated with prednisolone initially. Five responded with complete recovery, eight responded with partial recovery, and one died with extensive involvement of the disease. Six of the patients that recovered partially were later treated with cyclophosphamide, vincristine and prednisolone. One patient recovered completely and two partially. Three died from extensive involvement. Two patients with malignant lymphoma were treated by combination chemotherapy. One case went to complete remission while the other died from infection. One patient was lost to follow up before any treatment was started.
 ...
PMID:Angioimmunoblastic lymphadenopathy with dysproteinemia in Thailand. 332 19

LGL leukemia results from a chronic, clonal proliferation of LGL. Chronic neutropenia with recurrent bacterial infection and splenomegaly are common clinical manifestations. Rheumatoid arthritis coexists in some of these patients, who thus resemble patients with Felty syndrome. Other hematologic abnormalities that may occur include pure red-cell aplasia and adult-onset cyclic neutropenia. Lymphoid infiltration of bone marrow, splenic red pulp cords, and hepatic sinusoids is characteristic; lymph node and skin involvement are rare. Multiple serologic abnormalities are frequently present, including positive tests for rheumatoid factor and/or antinuclear antibody, polyclonal hypergammaglobulinemia, and circulating immune complexes. Antineutrophil and antiplatelet antibodies are often present. Leukemic LGL exhibit phenotypic heterogeneity; the most common phenotype in our patients is CD2+, CD3+, CD8+, HNK-1+, CD16-. Despite markedly increased numbers of LGL, functional activity of the cells is usually decreased. The mechanism of cytopenias is uncertain: in pure red-cell aplasia, it appears to be due to suppressive effect on erythropoiesis by abnormal LGL, but in patients with chronic neutropenia it may be antibody-mediated. Although most patients appear to have a relatively benign clinical course, mortality from infections and progressive lymphoproliferation is substantial. Optimal therapy remains undefined. Some preliminary evidence suggests that LGL leukemia may be associated with infection with a retrovirus similar to HTLV-I. Although relatively rare, LGL leukemia is of interest because a better understanding of this disease process may contribute to our knowledge of autoimmune diseases, the immunoregulatory functions of LGL, and the mechanisms controlling normal hematopoiesis.
 ...
PMID:Large granular lymphocyte leukemia. Report of 38 cases and review of the literature. 362 48

A male patient, aged 23 years, with no notable previous history, was admitted to hospital because of fever, splenomegaly, and pancytopenia. The bone marrow was infiltrated by mature looking histiocytes displaying erythrophagocytosis. Other findings included polyclonal hypergammaglobulinemia, elevated transaminases, hypofibrinogenemia, and hypertriglyceridemia. A diagnosis of virus-associated hemophagocytic syndrome was made and virology studies attributed it to herpes simplex virus type 1. Therapy with acyclovir, high dose i.v. immunoglobulins, and vinblastine was unsuccessful. With the addition of prednisone, a remission, with disappearance of histiocytic marrow involvement, was obtained but was of short duration, and the outcome was fatal.
 ...
PMID:[Hemophagocytic syndrome associated with herpes simplex virus. Apropos of a case with a fatal outcome]. 380 39


1 2 3 Next >>