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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Liver function abnormalities have been noted in intensively treated hemophiliacs, and have led to less aggressive application of pooled plasma products by some physicians. In a prospective study, liver function was abnormal in 68 of 98 hemophiliacs. The abnormalities of hepatic function tended to persist over a 1-yr study period. There was no correlation between these abnormalities and the age of the patient, the presence of hepatitis-associated antigen or antibody, the presence or absence of splenomegaly (which was found in 26 of 98 patients), the number of infusions of plasma products, the type of hemophilia, or the type of product infused. Titers of antibodies to cytomegalovirus were generally higher in hemophilic patients than in a control group of healthy volunteers. These abnormalities did not suggest that a less aggressive infusion regimen was indicated for the hemophiliac, but did suggest the need for careful long-term observation of such patients.
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PMID:Health of the intensively treated hemophiliac, with special reference to abnormal liver chemistries and splenomegaly. 19 36

Classical sex-linked hemophilia (Hemophilia A) has been described as due to deficiency in the synthesis of Factor VIII procoagulant activity (VIII:C). The availability of immunological techniques provided the means of identifying Factor VIII-Related Antigen(VI-IIR:Ag) detectable by rabbit antibodies to F VIII, which is distinct from VIII:C detected by human anti-F VIII available from multitransfused patients. Hemophilia A is lacking in VIII:C but not VIIIR:Ag. Recently, a third function of the F VIII "complex" was discovered with the help of ristocetin (von Willebrand's Factor, VIIIR: RCo). This activity is reduced in von Willebrand's syndrome. Estimation of the titers of VIII:C and VIIIR:Ag provides a method for more accurate detection of hemophilic carriers. Newly available chromogenic substrates perhaps will give rise to more simplified assays of VIII:C. The development of cryoprecipitates and stable lyophilized concentrates of F VIII has greatly simplified and intensified maintenance therapy, and has opened a new era in treatment. Prophylactic therapy has been shown to be very helpful in certain "high risk" cases. The impact and benefits of home care and self-administration has been tremendous. However, the varying quality of cryoprecipitates and the high cost of more purified concentrates are still stumbling blocks in treatment regimes. Other problems exist. Spontaneous bleeding, especially central nervous system bleeding, account for the majority deaths by haemorrhage. Inhibitor kinetics have been well characterized. It is clear that there exists "low" and "high" responders. For the "high" responders, plasmapheresis, immunosuppressives and the infusion of Factor IX concentrates have been utilized with varying success. The prevention of hemophilic arthropathy and its progression by maintenance therapy seems to be still inadequate. The results of trials with more vigorous regimes are awaited. The complications of therapy still remain to be solved. Apart from the well-known complications wuch as hepatitis, haemolytic disease and F VIII inhibitors, the existence of previously unnoticed complications as splenomegaly, hypertension, renal disease and paradoxal bleeding have been recently realized. The role of altered fibrinogen, fibrin degradation products (FDP) and unclassified fibrinogen derivatives (UFD) present in cryoprecipitates and F VIII concentrates in the above complications needs to be further clarified. In conclusion, tremendous progress in various aspects of hemophilia has been achieved in developed countries. Comprehensive care can now be carried out in various centers. On the other hand, developing countries still face a number of basic problems. The concept that hemophilia is a "manageable" disease and that chronic crippling and death from exsanguination can be prevented, should be disseminated widely by various means...
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PMID:Recent advances in hemophilia. 52 46

The occurrence of a portal vein thrombosis in a haemophilia A patient is reported. The patient, a 53 year old male, had been followed by us for the past 20 years in our out-patient Clinic. He was hospitalized recently for a suspected hepatic cirrhosis. Severe ascites, hepato-splenomegaly together with weight loss and mild fever were present. During the hospitalization, an ultrasound and CT scan of the liver confirmed the cirrhotic pattern and showed the presence of a portal vein thrombosis. There were no changes in the underlying coagulation defect, in fact, the patient had recurrent haemarthrosis. Furthermore, with the ultrasound examination, some focal hepatic lesions--probably due to a hepatocellular carcinoma--were also observed. The patient died because of massive haematemesis due to rupture of oesophageal varices.
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PMID:Portal vein thrombosis in a patient with severe haemophilia A and post-hepatitis liver cirrhosis. 166 75

Splenectomy for massive splenomegaly and hypersplenism carries a significant morbidity and mortality. We have used partial splenic embolization (PSE) as an effective alternative to splenectomy. Ten PSE procedures were performed on nine patients without mortality and with minimal morbidity. The age of the patients ranged from 8 months to 32 years (mean 14 years). The causes of splenomegaly and hypersplenism included cystic fibrosis with cirrhosis (2), tyrosinemia and cirrhosis (1); thalassemia (1), hemophilia with Human Immune Deficiency Virus infection (2), chronic hepatitis with portal hypertension (1), malignant histiocytosis (1), and Wiskott-Aldrich Syndrome (1). All procedures were performed under local anesthesia with sedation. A percutaneous femoral artery approach to the splenic artery was used to deliver Ivalon sponge particles (280-800 microns) into the spleen. Splenic infarction was assessed by postembolization angiograms. All of the patients except one demonstrated improvement of hematologic parameters. In one patient, however, cytopenia improved only after a second embolization. In the total series, there was an early mean rise of 8,600/mm3 in the leukocyte count (range 2,900-14,900) and 212,000/mm3 in the platelet count (range 30,000-718,000). Follow-up ranged from 4 months to 7 years. Improvement of the blood picture has been persistent in seven of the eight patients who showed initial improvement. Transient procedural complications included fever (5), pleural effusion (2), pneumonia (1), and splenic abscess (1). One patient had paralytic ileus lasting for 10 days and one patient developed a streptococcal peritonitis 3 weeks after embolization. No patient developed pancreatitis or vascular compromise of other abdominal viscera.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Partial splenic embolization. An effective alternative to splenectomy for hypersplenism. 226 5

Patients with hemophilia are at a risk for the acquired immuno-deficiency syndrome (AIDS). As part of a surveillance program on AIDS, a group of patients with hemophilia was studied for clinical and immunological features associated with the syndrome. 30 patients in England who had received factor VIII treatment within 5 years were studied. 29 (male) had hemophilia A, and 1 female heterozygote had been treated for postoperative bleeding. 1 man had been tattooed over 10 years previously. No other risk factors for AIDS were present. 5 patients had recently developed splenomegaly or lymphadenopathy, and 1 of these also had mild thrombocytopenia. No opportunistic infection or unexplained loss of weight was noted. A basal cell carcinoma of the face in a 59-year old was recorded. 20 patients had detectable HTLV-III antibodies at the most recent date of testing. After 1981 approximately 15% of the subjects seroconverted each year until 1984. Most of the seropositive patients had had large amounts of factor VIII treatment, predominantly commercial concentrate from the US. 4 patients who seroconverted during 1984 had had little treatment, including a man who had used only 3250 units during 1983 from the same batch of commercial concentrate. 5 patients had used only products from the National Health Service over 5 years, and all were seronegative. The results failed to identify features that might help distinguish between self limiting HTLV-III infection and leading to AIDS or a carrier state.
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PMID:Seroconversion of human T cell lymphotrophic virus III (HTLV-III) in patients with haemophilia: a longitudinal study. 298 90

Early reports suggested that hemophiliacs with factor IX deficiency (Christmas Disease) may be at less risk for developing the acquired immunodeficiency syndrome (AIDS) than patients with classic hemophilia. We evaluated 12 factor IX deficient patients for clinical and immunologic abnormalities related to infection with the human immunodeficiency virus (HIV). Antibody to HIV was not detected in these patients prior to 1982. By 1985, 66 percent (eight of 12) patients were seropositive. All three concentrates available commercially before 1985 were associated with seropositivity. Furthermore, seropositive hemophiliacs had received on average significantly more factor IX concentrate than seronegative hemophiliacs (27,825 +/- 17,976 (S.D.) versus 1,250 +/- 1,500 factor units/year, (p less than 0.02). Half of the seropositive individuals had generalized lymphadenopathy with splenomegaly. Two seropositive patients have developed AIDS, one with cryptococcal meningitis and another with a large cell immunoblastic lymphoma. Infection with HIV has occurred with high frequency in hemophiliacs who received unmodified factor IX concentrates.
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PMID:The spectrum of human immunodeficiency virus infection in patients with factor IX deficiency (Christmas disease) 303 83

We examined 35 patients with hemophilia to determine if there was an association between impaired cell-mediated immunity and the amount of factor concentrate use. There was a significant negative relationship between the logarithm of the helper-suppressor ratio and the logarithm of concentrate use determined over the previous one year, five years, and total lifetime. Similarly, the presence of splenomegaly was significantly associated with the logarithm of concentrate use for each time interval. Hypergammaglobulinemia, anergy, and lymphadenopathy were present in a high proportion of patients. Repeated study of 30 of these patients at eight to 14 months showed no significant changes in their T-cell subsets. At follow-up, 16 patients had lymphadenopathy with or without splenomegaly and four had splenomegaly alone. No significant associations between concentrate use during the study period and changes in T-cell subsets or clinical condition were found.
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PMID:Sequential clinical and immunologic abnormalities in hemophiliacs. 391 66

Hematologic contraindications to the continuation of pregnancy are discussed. Although maternal leukemia, including Hodgkin's disease, is not per se a contraindication, pregnancy should be interrupted when glucocorticoid, cytostatic, or abdominal X-ray treatments are necessary, because of their teratogenic and mutagenic effects on the fetus. Pronounced splenomegaly and abdominal involvement of leukemia or other malignant lymph node conditions, however, constitute absolute contraindications, even after Week 12 of pregnancy. In addition, hemophilia may be considered a genetic contraindication.
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PMID:[Contraindication for carrying pregnancy to full term from the hematologic viewpoint]. 471 45

Previous studies have demonstrated functional and histologic abnormalities of the liver, and, more recently, splenomegaly in patients with hemophilia. Since these observations usually were derived from hemophiliacs who had received intensive replacement therapy, the question was posed as to whether the frequency of splenic and hepatic abnormalities was secondary to the amount of therapy utilized. In this study, a variety of tests were employed to evaluate spleen and liver size and function to determine if abnormalities in these organs correlated with the intensity of the transfusion program. The study group was comprised of 25 hemophiliacs (mean factor replacement-18,361 U/year; median factor replacement-12,920 U/year). Over 70% of our patients had elevations of aspartate and alanine aminotransferase. Immunoglobulin and complement levels were normal in most subjects. Ninety-six percent had evidence of exposure to hepatitis B virus. Liver-spleen imaging suggested significant hepatic abnormalities in most of the patients as evidenced by inhomogeneity of tracer uptake in the liver in 33% and relatively increased colloid uptake in the spleen in 90%. Splenomegaly (palpable spleen or enlargement on liver-spleen imaging) was detected in 40% of our patients, and tended to occur in the more frequently transfused patients. These findings indicate that significant abnormalities of the spleen and liver can occur in hemophiliacs who have received moderate amounts of replacement therapy and that liver-spleen imaging may be a useful method for monitoring the development of hepatic and splenic abnormalities.
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PMID:Abnormalities of the spleen and liver in patients with hemophilia. 684 27

Persons with hemophilia or other HIV-1 risk factors may be more likely to have idiopathic CD4+ T-lymphocytopenia (ICL). We determined the frequency of ICL in prospectively followed cohorts of HIV-1 seronegative hemophilic men and seronegative female sex partners of HIV-1 infected hemophilic men, and examined factors potentially associated with ICL. Seven of 304 (2.3%) seronegative hemophilic men and one of 160 (0.6%) female partners met the ICL definition, but the condition resolved for two of the men and for the sole female partner. All five men with persistent ICL had lymphocytopenia (< 1,200 total lymphocytes/microliters) and < 300 total CD4+ lymphocytes/microliters; only one had a low CD4+ percentage. On the most recent measurement, 14.5% of the 304 seronegative hemophilic men had lymphocytopenia. Compared with matched hemophilic controls, men with persistent ICL more often had a history of liver disease (3/5 cases, 0/21 controls, P = 0.007) or splenomegaly (3/5 cases, 4/21 controls; P = 0.04), but not severe hemophilia, greater clotting factor concentrate exposure, high alanine aminotransferase levels, hepatitis B virus antigenemia, or detectable hepatitis C virus RNA in plasma. All five cases and 20/21 controls had antibodies to hepatitis C virus present in their serum. In this cohort of hemophilic men, ICL was related to lymphocytopenia associated with liver disease rather than selective loss of CD4+ lymphocytes.
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PMID:Idiopathic CD4+ T-lymphocytopenia in HIV seronegative men with hemophilia and sex partners of HIV seropositive men. Multicenter Hemophilia Cohort Study. 760 13


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