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 clinical symptoms, response to therapy, and prognosis of T-zone lymphoma were analyzed in 32 cases. This recently defined lymphoma entity developed relatively quickly with generalized lymphadenopathy and general malaise. Hepatomegaly and/or splenomegaly and skin efflorescence were frequent presenting symptoms. A few patients showed hyperimmune reactions and occasionally severe autoimmune hemolytic anemia. The erythrocyte sedimentation rate was usually markedly elevated. There was sometimes a polyclonal increase in serum immunoglobulin, sometimes a reduction. Blood and bone marrow smears from a few patients showed occasional atypical lymphocytes. A remarkable finding was the frequent involvement of lung or pleura (40.5% of the patients). The prognosis is unfavorable. Most of the patients were in stages III or IV at the time of diagnosis. Massive infiltration of organs, resistance to routine therapy, and decreasing resistance to infection resulted in death soon after diagnosis. The probability of survival was 0.48 in the first year after diagnosis. The prognosis for patients in stages I and II was clearly better than that for patients in stages III and IV.
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PMID:[T-zone lymphoma--clinical symptoms, therapy, and prognosis (author's transl)]. 31 15

Mean red cell life-span in normal subjects and in patients with various hematological disorders was examined with 51Cr and DF32P. The results with 51Cr were corrected for 51Cr elution using correction factors. The results by the two methods agreed fairly well with each other. Elution rate in various hematological disorders was 2.3% per day or less except for the patients with extracorpuscular hemolytic agents such as autoimmune hemolytic anemia or congestive splenomegaly. It is concluded that estimates of mean red cell life-span by corrected 51Cr method are more useful and sufficient than uncorrected 51Cr or DF32P method in general hematological disorders.
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PMID:Evaluation of recommended methods for radioisotopes red cell survival studies. 99 47

A 59-year-old woman was admitted in February 1991, because of abdominal distension. On admission, she had splenomegaly, autoimmune hemolytic anemia, and idiopathic thrombocytopenic purpura. Evans syndrome had been diagnosed and daily prednisolone therapy had been performed. After this therapy, a rapid increase of lymphocytes was observed accompanied with contraction of spleen. The monoclonal proliferation of B-lymphocytes and rearrangement of JH and J kappa gene were detected and chronic lymphocytic leukemia was diagnosed. With reduction of prednisolone, the lymphocyte counts decreased and the size of the spleen returned to the previous state. It is suspected that the cause of rapid lymphocytosis in this case was due to the redistribution of lymphocytes from the spleen to the peripheral blood.
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PMID:[Chronic lymphocytic leukemia diagnosed after corticosteroid therapy for Evans syndrome]. 143 26

Autoimmune hemolytic anemia and severe giant cell hepatitis is an very uncommon association. Two patients aged 5 months and 2 years developed severe anemia, jaundice, hepatomegaly and splenomegaly. Laboratory tests demonstrated autoimmune hemolytic anemia and elevated amino-transferases. Diffuse giant cell transformation and distorted architecture was seen in liver biopsy. One patient had a cytomegalovirus infection. Early steroid therapy did not prevent fatal course with liver failure in one patient.
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PMID:[Autoimmune hemolytic anemia associated with severe giant cell hepatitis]. 204 9

Resident peritoneal macrophages from New Zealand Black (NZB) mice release O2- and H2O2 after adherence to a plastic surface without any chemical or particulate stimulant. This phenomenon is age dependent and more pronounced in animals with sever autoimmune disease. Significant differences were observed between the high and low breakage NZB sublines (HB and LB), which were previously developed by selective matings on the basis of chromosome breakage rates. The LB subline differs significantly from the HB subline with respect to autoimmune hemolytic anemia and tumor incidence. When the macrophages were stimulated with the tumor promoter TPA, the number of "responders" was higher in the HB than in the LB subline and correlated with the degree of splenomegaly, that is, with the severity of the disease. A negative response to agonist stimulation and very low spontaneous production of active oxygen species was observed in NZW and Swiss mice, which is the normal finding for resident macrophages according to data from the literature. The increased superoxide and hydrogen peroxide production by macrophages of NZB mice is discussed with respect to autoimmune disease and cancer.
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PMID:Superoxide and hydrogen peroxide production by macrophages of New Zealand black mice. 216 74

While anemia and a positive direct anti-globulin test are each frequently observed in the clinical syndrome of human immunodeficiency virus (HIV) infection, autoimmune hemolytic anemia has rarely been reported in this setting. A case of severe warm autoimmune hemolytic anemia (AIHA) with reticulocytopenia in a patient with AIDS-related complex is reported. Laboratory and clinical findings of severe hemolysis were present, including anhaptoglobinemia, microspherocytosis, splenomegaly, and transfusion dependence. Azidothymidine (AZT) therapy may have exacerbated this patient's anemia. Splenectomy produced a delayed but complete remission of the AIHA despite continuation of AZT therapy. Review of other reports of positive direct antiglobulin tests and autoimmune hemolytic anemia in patients with HIV infections suggests that autoantibodies may be a significant cause of anemia in this population and that the frequent lack of reticulocytosis, despite bone marrow erythroid hyperplasia, may lead to the underdiagnosis of AIHA in HIV-infected patients.
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PMID:HIV-associated autoimmune hemolytic anemia: report of a case and review of the literature. 175 45

Normal and autoimmune mice were studied with regard to signals eliciting differentiation and division of bone marrow stem cells. The erythropoiesis induced by anemia following serial bleedings was analyzed in young autoimmune New Zealand Black (NZB) mice and non-autoimmune strains. No difference in the response to the stimulus created by anemia was noted between the strains. After serial bleedings as a stimulus to stem cell proliferation, a five-fold increase in numbers of proliferating spleen cells occurred in both NZB and DBA/2 strains; the increased proliferating spleen cells in both strains were non-lymphoid. The bled animals had decreased percentages of B cells. The production of autoantibodies was not significantly altered by the experimentally induced anemia. In contrast, anti-immunoglobulin activation of resting B cells was increased in response to anemia. Young mice which had experimentally induced anemia had several characteristics in common with old autoimmune NZB mice. Both old NZB mice and young anemic animals had splenomegaly, increased numbers of proliferating spleen cells, decrease in splenic Ly 5+ cells and an increase in splenic colony forming units (CFUs). The anemic normal strains of animals lacked other characteristics of old NZB mice such as hyperimmunoglobulinemia or autoantibody production or elevated CD5+B cell numbers. This work supports the concept that the increase in spleen cell number, proliferating spleen cells, CFUs and the increased percentages of non-Ly-5 cells (which include erythroid precursors) found in the spleens of old NZB mice may in part result from their autoimmune hemolytic anemia.
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PMID:Effects of induced anemia in normal and autoimmune mice. 221 Aug 4

This review underscores the diversity of the clinical manifestations and hematopathological features of gamma heavy chain disease based on the detailed report of 16 patients evaluated in our chemical department, the analysis of 12 cases diagnosed in our laboratory, and the study of 81 cases previously reported. This condition is defined by the presence in the serum of immunoglobulin molecules composed of deleted gamma heavy chains devoid of light chains. The production by the monoclonal B cells of these peculiar proteins appears to result from multiple defects (deletions, insertions, and mutations) in both heavy and light chain genes leading to abnormal mRNA splicing. Gamma heavy chain disease is currently underdiagnosed. The diagnosis established by immunoelectrophoresis using specific antisera combined, in some instances, with the immunoselection procedure, can easily be missed on serum electrophoretic patterns: a narrow abnormal band suggestive of a monoclonal component was found in only 10 of our 28 cases. The amount of heavy chain disease protein in urine ranges from trace to 20 g/day and is usually moderate. Gamma heavy chain disease most often presents as a lymphoproliferative disorder featured by lymphadenopathies, splenomegaly, and constitutional symptoms. Extra-hematopoietic tumor localizations, such as cutaneous or subcutaneous involvement or thyroid tumor, may occur. Autoimmune disorders, notably rheumatoid arthritis and autoimmune hemolytic anemia or thrombocytopenic purpura, are frequent (26% of cases). There is no specific histological pattern. The most frequent is a pleomorphic malignant lymphoplasmacytic proliferation mainly seen in bone marrow and lymph nodes. Some cases present with a predominantly plasmacytic proliferation or chronic lymphocytic leukemia. Other patients are affected with non-Hodgkin lymphoma of various morphologic types. Immunocytologic studies showed that a gamma heavy chain disease protein may occur in the context of a double monoclonal lymphoproliferative process or in various B or T cell malignancies that are not directly involved in the production of the abnormal immunoglobulin. In some patients, the histologic appearance of the enlarged lymphoid organs showed only a moderate lymphoplasmacytic infiltration of uncertain malignancy. More important, some patients showed no evidence of an underlying lymphoproliferative disorder after several years of follow-up. The clinical course of gamma heavy chain disease varies from an asymptomatic state to a rapidly progressive malignancy. The choice of therapy should entirely rely on the underlying clinicopathologic features, without taking into account the presence of the abnormal protein.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Gamma heavy chain "disease": heterogeneity of the clinicopathologic features. Report of 16 cases and review of the literature. 250 55

Hepatic fibrin-ring granulomas were the main histological finding in the liver of a 38-year-old man with Epstein-Barr virus primary infection. The patient presented with fever, hepatomegaly, icterus, abnormal liver tests, autoimmune hemolytic anemia, and mononucleosis syndrome. There was neither enanthema nor lymphadenopathy or splenomegaly. Serologic tests disclosed an Epstein-Barr primary infection profile: anti-viral capsid antigen IgM antibodies and anti-early antigen antibodies were present, whereas anti-Epstein-Barr nuclear antigen antibodies were absent. There was no evidence for Q fever, Hodgkin's disease, or allopurinol-induced hepatitis, which are recognized causes of hepatic fibrin-ring granulomas. It is suggested that Epstein-Barr virus infection might be an additional cause of these peculiar hepatic granulomas.
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PMID:Epstein-Barr virus infection and hepatic fibrin-ring granulomas. 283 98

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.
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PMID:Angioimmunoblastic lymphadenopathy with dysproteinemia in Thailand. 332 19


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