Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

The technique, and short and long term results, of splenectomy for massive splenomegaly are presented. The latter was defined as a spleen weighing in excess of 1.5 kg. Thirty-five of the 38 patients had a serious haematological disorder as the indication for the operation, usually non-Hodgkin's lymphoma or myelofibrosis. All operations were carried out through abdominal incisions. Accessible splenic attachments were divided, and the splenic artery was ligated in continuity, prior to posterior mobilization. Two patients (5.3%) died as a result of the operation, both deaths being due to sepsis. Septic and thrombo-embolic complications were common, and occurred both early and late after the operation. Overall, eight of the 12 deaths during the first postoperative year were due to the primary disease, whereas all of the five deaths after that time were due to causes other than the primary disease. Twenty-four patients lived at least 1 year, and 10 patients are alive for more than 5 years.
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PMID:Splenectomy for massive splenomegaly. 386 13

Hereditary spherocytosis is a clinically heterogeneous, genetically determined red blood cell membrane disorder resulting in hemolytic anemia. A deficiency of spectrin, the largest and most abundant structural protein of the erythrocyte membrane skeleton, results in the formation of spherocytes which lack the strength, durability, and flexibility to withstand the stresses of the circulation. Clinical manifestations of the disease are primarily dependent on the severity of hemolysis, which additionally results in an increased incidence of pigment gallstones. The likelihood of cholelithiasis is directly related to patient age and is uncommon before 10 years of age. Splenectomy is indicated in virtually every patient. When the disease is diagnosed in early childhood, the risk of overwhelming postsplenectomy sepsis makes it advisable to delay splenectomy until after 6 years of age if possible. At the time of splenectomy, it is important to identify and remove any accessory spleens. If gallstones are present, cholecystectomy should be performed. Although spherocytosis persists following splenectomy, hemolysis is alleviated and clinical cure of the anemia is achieved for most patients. Patients with recessively inherited spherocytosis are exceptions. Although they are significantly benefited by splenectomy, their anemia is not completely corrected. Splenectomy reduces hemolysis in all patients and thereby decreases the risk for development of pigment gallstones. Excision of an enlarged spleen removes the danger of traumatic rupture.
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PMID:Hereditary spherocytosis. Recent experience and current concepts of pathophysiology. 394 20

Classification of platelet disorders has been based on the platelet count. Addition of a second variable, mean platelet volume (MPV), to the routine blood count allows classification of patients into 9 categories: high, low, or normal MPV, and high, low or normal platelet count. We studied 1,244 adult inpatients. 1,134 had both platelet values normal. 11 patients had high MPV and low platelet count: all had hyperdestructive causes. 15 patients had high MPV and normal platelet count: 12 had heterozygous thalassemia, and three had iron deficiency. Seven patients had high MPV and high platelet count: causes included myeloproliferative disorders, inflammation, iron deficiency, and splenectomy, 25 patients had high platelet counts and normal MPV: the causes were inflammation, infection, sickle cell anemia, iron deficiency, or chronic myelogenous leukemia. 52 patients had an MPV that was inappropriately low for the platelet count (high, normal, or low). All had sepsis, splenomegaly, aplastic anemia, chronic renal failure, or a disease being treated with myelosuppressive drugs. High MPV thus appears correlated with myeloproliferative disease or thalassemia; and low MPV, with cytotoxic drugs or marrow hypoplasia. Addition of MPV to the platelet count allows subtler disorders to be detected (when the platelet count is normal), and allows distinction of the cause of thrombocytopenia.
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PMID:Use of mean platelet volume improves detection of platelet disorders. 407 87

A disease that is similar to human AIDS may occur in monkeys. Simian AIDS (SAIDS) was experimentally transmitted from 2 rhesus monkeys dying of the disease to 4 cytomegalovirus (CMV) antibody-negative rhesus monkeys. The inocula consisted of the supernatant fluid from 10% homogenates of various tissues with or without buffy-coat cells from blood. Lymphadenopathy, splenomegaly, neutropenia, polymyositis, and other signs of the disease appeared in recipients within a few weeks after inoculation. Two animals developed Kaposi-like "patch" and "plaque" skin lesions and one died of sepsis and profound lymphoid depletion. A second animal also died with lymphoid depletion. All animals became infected with CMV but antibody levels were low in two animals, appeared and then disappeared in one, and never developed in the second monkey which died.
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PMID:Experimental transmission of simian acquired immunodeficiency syndrome (SAIDS) and Kaposi-like skin lesions. 613 95

Between 1956 and 1981, 306 splenectomies for hematologic diseases were performed at the UCLA Medical Center. Of these operations, more than 75% were performed for therapeutic reasons to control anemia, thrombocytopenia, neutropenia, or painful symptoms of splenomegaly. Of the 65 patients who had idiopathic thrombocytopenic purpura, 77% showed an excellent response, and of the 39 patients who had hereditary spherocytosis, 90% responded. Other diseases with predictably good response rates were autoimmune hemolytic anemias, Felty's syndrome, and hairy cell leukemia. Forty patients with Hodgkin's disease had splenectomies for diagnostic purposes the last 10 years. The overall morbidity and mortality were 24% and 6%, respectively, the most common complications being pneumonia, wound infections, and local postoperative bleeding, and the most common cause of death being sepsis. The review supports the thesis that in carefully selected patients, therapeutic splenectomy can have desirable palliative effects and that diagnostic splenectomy has a sufficiently low risk to warrant its consideration in patients with Hodgkin's disease.
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PMID:Splenectomy for hematologic disease. The UCLA experience with 306 patients. 673 25

Partial splenectomy for a large splenic cyst was performed in a seven-year-old girl referred for splenomegaly. The increased risk of sepsis in splenectomized patients induced the successful attempt at partial splenectomy. The surgical procedure described involved removal of the cyst while preserving as much of the spleen as possible. In view of the planocellular epithelial lining, the pathological diagnosis was of a rare congenital, intrasplenic cyst.
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PMID:Partial splenectomy for a splenic cyst. 673 90

A retrospective study was conducted to analyze the surgical aspects of the splenectomy in chronic myelogenous leukemia. Twenty patients, Philadelphia chromosome-positive, were initially treated with busulfan until remission was reached. Elective splenectomy was then performed and chromosomal studies repeated at four- and six-month intervals yielding the indication for cyclic intensive chemotherapy. There ws no mortality, one episode of gram-negative sepsis with shock, and five instances of minimal complications. The spleen weights averaged 265 g, ranging from 60 to 800 g. All patients had normal coagulation profiles at the time of surgery, but four of them developed a postoperative thrombocytosis without related complications. A specific correlation was noted between postoperative thrombocytosis and splenomegaly (average weight 570 g). Eleven patients showed a high postoperative leukemoid reaction (average 40,500 cells/cu mm). The interval between diagnosis and splenectomy was shorter (average 7.5 months) in this group than for the patients who had a lower granulocytosis (average 19.5 months).
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PMID:Early splenectomy in chronic myelogenous leukemia: surgical aspects. 694 Apr 64

The study of portal pressure during surgical exploration for biliary atresia in the series of 260 children operated at the Hospital Saint-Vincent de Paul since 1968 showed that portal hypertension could be demonstrated in 61.8% of the cases before the age of 3 months. Among the 54 children surviving without jaundice, splenomegaly was found in 35 cases; esophageal varices were seen in 14 children, more frequently after 5 years of age. Bleeding from esophageal varices occurred only in cases with recurrence of jaundice or moderate biliary retention, i.e. for 6 children. A portocaval shunt was constructed in 7 cases; all had bled except one. One child died two and a half years later from pneumococcal sepsis after a splenorenal shunt with splenectomy; another child had numerous recurrence of bleeding episodes. Five children have a functional shunt and have not rebled. When biliary atresia is cured by hepatoportoenterostomy, with complete disappearance of biliary retention, the risk of bleeding from esophageal varices is unlikely, in spite of persistent cirrhotic alterations of the liver; in these children, there should very seldom be an indication for a portocaval shunt.
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PMID:[Portal hypertension and biliary atresia (author's transl)]. 697 6

The records of eleven patients who underwent splenectomy for Gaucher's disease at The Mount Sinai Hospital from 1967 to 1981 have been reviewed. The major indications for operation were hypersplenism, pain, and mechanical problems associated with a massively enlarged spleen. There were no operative mortalities and the hematologic picture returned to normal in all cases. Because of the danger of overwhelming sepsis following splenectomy, partial splenectomy should be considered as a therapeutic modality in these patients.
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PMID:The surgical management of Gaucher's disease. 712 40


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