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)

Splenectomy is a valuable method of management of approximately 80 per cent of patients with hairy cell leukemia who have hypersplenic complications develop; operation can be performed with minimal morality and morbidity. Removal of the spleen also provides useful palliation in patients with chronic lymphocytic leukemia who have symptomatic splenomegaly or autoimmune hemolytic anemia; success is less predictable when operation is performed for thrombocytopenia. Splenectomy is linked with a high mortality and is of little benefit in patients with chronic granulocytic leukemia except when operation is performed as a prerequisite to bone marrow transplantation. Several patients with acute leukemia who have signs of splenic infarction and intra-abdominal bleeding develop had a short survival period after splenectomy.
 ...
PMID:The limited role of splenectomy in patients with leukemia. 385 56

We investigated the relationship between the increased cell diameter of Lyt-2+ T cells and the development of autoimmune disease in aging NZB and NZB X NZW F1 hybrid (BW) mice. Individual animals were analyzed for Lyt-2+ T cell size (by narrow-angle forward light scatter), anti-erythrocyte autoantibodies, anemia, proteinuria, and splenomegaly. The peak light scatter of the Lyt-2+ T cells correlated with the level of anti-erythrocyte autoantibodies and severity of hemolytic anemia, but not with proteinuria or splenomegaly. The cell size of this T cell subset did not increase in old BW or in NZB mice homozygous for the xid gene (NZB.xid). The in vivo administration of bacterial lipopolysaccharide to young NZB mice did not stimulate the enlargement of Lyt-2+ T cells. Ly-2+ T cells from old NZB mice could be stimulated by concanavalin A (Con A) to express interleukin 2 (IL 2) receptors and to synthesize DNA in vitro. However, in vivo administration of Con A to old NZB mice did not induce the expression of IL 2 receptors on Lyt-2+ T cells. Further, in vivo T suppressor function was impaired in old NZB mice with enlarged Lyt-2+ T cells. Thus, the enlargement of Lyt-2+ T cells in old NZB mice appears related to impaired T cell function in vivo and is associated with the development of anti-erythrocyte autoantibodies and autoimmune hemolytic anemia.
 ...
PMID:Enlargement of Lyt-2-positive T cells is associated with functional impairment and autoimmune hemolytic anemia in New Zealand Black mice. 392 48

An indirect platelet radioactive antiglobulin test (PRAT) was used to evaluate serum antiplatelet antibody and complement activation in 114 patients with lymphoproliferative disease. Overall, 60% of serum samples gave a positive indirect PRAT. Positive PRAT was observed in 85% of patients with chronic lymphocytic leukemia (CLL) and megakaryocytic thrombocytopenia, as well as in about half the patients with CLL and a normal platelet count or amegakaryocytic thrombocytopenia. In contrast to findings in patients with idiopathic autoimmune thrombocytopenic purpura or in those with leukemia who were alloimmunized, positive PRAT in patients with lymphoproliferative disease was predominantly caused by C3-only sensitization of platelets. Results were analyzed in relation to other clinical and laboratory findings. There was no significant difference in antiglobulin sensitization pattern between CLL and other lymphomas, different cell type, stage of disease, presence or absence of splenomegaly, recent or distant chemotherapy, sex, or history of pregnancy. Previous transfusion did affect antiglobulin sensitization patterns, as did presence of autoimmune hemolytic anemia. A high frequency of low serum complements (CH50, C3, C4, and factor B) and increased circulating immune complexes was observed; these did not correlate with frequency or sensitization pattern of positive PRAT. Amounts of bound anti-C3 and anti-IgG were significantly higher in patients with lymphoproliferative disease and positive antiglobulin test than in normal individuals; there was, however, no correlation between amount of antiglobulin serum bound and platelet counts. There was an 87% correlation in direct and indirect PRAT in 24 patients tested.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Indirect platelet radioactive antiglobulin test in patients with lymphoproliferative disease. 394 93

In the past 10 years we have examined 20 children with inflammatory liver disease associated with high serum titers of anti-liver-kidney microsome antibody (anti-LKM). The first hepatic symptoms were progressive fatigue and jaundice, the fortuitous finding of hepatomegaly or splenomegaly with raised transaminase activity, or an acute hepatitis-like illness. At the time of diagnosis, hepatomegaly was present in 18 children, splenomegaly in 16, jaundice in nine, and ascites in two. Serum alanine transferase activities were elevated in all but two, who had already received steroids. Serum total gammaglobulin values were greater than 2.0 gm/dl in 16 children, prothrombin activity less than or equal to 60% in six, and serum titer of anti-LKM between 1:100 and 1:100,000. All children but one had cirrhosis, and histologic signs of aggressivity were present in 14. In 11 children one or more extrahepatic diseases were present, including type 1 diabetes, vitiligo, glomerulonephritis, autoimmune hemolytic anemia, hypoglycemia with hyperinsulinism, autoimmune thyroiditis, chronic mucocutaneous candidiasis with hypoparathyroidism, and multiple cutaneous and visceral telangiectasias. Treatment with prednisone and azathioprine improved the liver condition in 16 of the 18 patients given treatment. In eight of them discontinuation of treatment resulted in rapid relapse; 14 are still receiving treatment and have stable hepatic function with follow-up from 8 months to 6 1/2 years. Only two are free of treatment. Four children died, two in spite of immunosuppressive therapy, one during a relapse, and one of extrahepatic disease. These results indicate that this autoimmune inflammatory liver disease may have onset early in life, with several clinical patterns; is frequently associated with certain types of extrahepatic manifestations of autoimmune origin; and is a potentially fatal disease for which immunosuppressive treatment must be started early.
 ...
PMID:Liver disease associated with anti-liver-kidney microsome antibody in children. 395 Aug 19

Reported is the unique occurrence of sarcoidosis, Hodgkin's disease, and autoimmune hemolytic anemia in the same patient. Although the diagnosis of sarcoidosis preceded that of Hodgkin's disease by 29 years, massive splenomegaly had been present for 13 years before Hodgkin's disease was diagnosed following splenectomy. The known associations of sarcoidosis and Hodgkin's disease as well as each disease with autoimmune hemolytic anemia are reviewed.
 ...
PMID:Sarcoidosis, Hodgkin's disease, and autoimmune hemolytic anemia. 395 37

We established two clones of monoclonal hybridoma, from a non-immunized NZB mouse, which produce IgM class hemagglutinating autoantibodies reactive with the exposed murine erythrocyte autoantigens. Absorption studies revealed that one monoclonal antibody exhibits cross-reactivity to chick erythrocytes and mouse liver, and the other antibody to rat erythrocytes and mouse brain. Optimal temperatures for the hemagglutination were 22 degrees C with the former and 4 degrees C with the latter. The specificity and nature of the autoantibodies are apparently distinct from any of the erythrocyte autoantibodies described to date in NZB mice, anti-X, anti-HB, anti-HOL or anti-I antibodies. Implantation of these hybridoma cells in BALB/c mice induced autoimmune hemolytic anemia associated with a marked splenomegaly. These findings provide evidence that the erythrocyte autoantibodies in NZB mice are more heterogenous than generally assumed and suggest that varieties of erythrocyte autoantibodies may be involved in the development of a naturally occurring hemolytic disease in NZB mice.
 ...
PMID:Hybridoma autoantibodies to erythrocytes from NZB mice and the induction of hemolytic anemia. 650 Jun 30

A relation between lymphoma and autoimmune neutropenia, unlike autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura, has not previously been well documented. We report a patient with a disorder presenting as autoimmune agranulocytosis, splenomegaly, and infiltration of the hepatic sinusoids by lymphocytes. Antineutrophil antibodies were present. Over a 2 1/2-year period, the illness progressed to an aggressive, poorly differentiated lymphocytic lymphoma with terminal liver failure and fibrosis. Peripheral blood lymphocyte markers identified the tumor as a proliferation of T-cells of the helper class. A review of previous literature disclosed other reports of similar patients who had neutropenia, a lymphoproliferative illness, and hepatic disease. Our case is representative of a previously unrecognized syndrome characterized by autoimmune neutropenia in the setting of a lymphoproliferative disorder of T cells, with a predilection for liver involvement.
 ...
PMID:Lymphoma with autoimmune neutropenia and hepatic sinusoidal infiltration: a syndrome. 698 Jun 14

The case report of a 52-year-old woman with an eight-year history of cyclic autoimmune hemolytic anemia and progressive splenic enlargement is presented. At laparotomy the enlarged spleen and splenic hilar lymph nodes showed lymphocyte-predominant Hodgkin's disease. No specific antitumor treatment was given and three years following splenectomy the patient is in excellent health and has not experienced any new hemolytic episodes. The patient illustrates the association between Hodgkin's disease and autoimmune hemolytic anemia as well as the extreme variation in the clinical picture of the disease.
 ...
PMID:Cyclic autoimmune hemolytic anemia as a presenting manifestation of splenic Hodgkin's disease. 706 69

In a retrospective study of 39 splenectomies, patients with increased blood cell breakdown (13 cases of idiopathic thrombocytopenic purpura (ITP), 5 cases of hereditary spherocytosis, 2 of Felty's syndrome and 2 of autoimmune hemolytic anemia) were compared with those patients also presenting decreased blood cell production [14 cases of myelofibrosis (MF) with splenomegaly and 3 cases of advanced chronic myelogeneous leukemia (CML)]. Platelet regeneration post-operatively was significantly (p less than 0.01) more rapid in the ITP than in the MF group. Only 1/22 patients in the ITP group had major post-operative complications as compared to 10/17 in the MF group. None of the patients in the ITP group died within 25 days of operation as compared to 5/17 in the MF group. Only 3/22 patients in the ITP group lost more than 800 ml of blood during the operation as compared to 8/17 with MF. No statistically significant higher blood loss was found in patients with less than 30 x 10(9) platelets/l preoperatively, compared to those with higher platelet counts. However, correlation between the splenic weights and amount of blood loss was statistically significant (p less than 0.01). Thus, splenectomy seems much better tolerated in patients with ITP, even if platelets are low, than in patients with myelofibrosis.
 ...
PMID:[Splenectomy in idiopathic thrombopenic purpura and in myelofibrosis. A retrospective study of platelet increase, hemorrhagic complications and mortality]. 719 36

Four children, aged 6 1/2 months to 2 years, presented with liver disease and autoimmune hemolytic anemia. Clinical signs included fever, jaundice, firm or hard hepatomegaly, and splenomegaly. Direct Coombs test results were of the mixed (IgG + C) type. Liver function tests showed high direct bilirubin transaminase, and serum gamma globulin values, and a prolonged prothrombin time. The liver histology was characterized by marked lobular fibrosis and giant cell transformation. The course of the disease was severe, resulting in the death of three patients from liver failure. However, the liver disease seemed responsive to corticosteroid treatment, which in one patient was clearly beneficial.
 ...
PMID:Severe giant cell hepatitis with autoimmune hemolytic anemia in early childhood. 729 42


<< Previous 1 2 3 4 5 6 7 8 Next >>