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)

Interleukin 4 (IL-4) is a pleiotropic type II cytokine which has been shown to have a direct killing effect on lymphoma and B-cell chronic lymphocytic leukaemia (B-CLL) cells in vitro. The clinical effects and toxicity of IL-4 treatment in patients with B-CLL were evaluated. Fourteen patients with B-CLL who were in partial remission after chemotherapy received one, two or three 8-week cycles of escalating doses (2, 4 or 6 microg/kg/d s.c.) of IL-4 for 3 d/week. Clinical response was analysed after each treatment cycle and toxicity was monitored continuously. Ten patients (71%) had progressive disease (PD) during IL-4 treatment. This was mainly attributable to an increase (two- to fourfold) of the blood lymphocyte count during IL-4 therapy. After cessation of IL-4 treatment, the lymphocytosis decreased spontaneously in 8 out of 12 evaluable patients. Splenomegaly remained unchanged in 7/7 patients, whereas enlarged lymph nodes were reduced by > 50% in 1/13 patients and by 25-50% in 4/13 patients. None of the patients achieved an objective tumour regression (complete or partial remission). A temporary increase (16-60%) of the platelet count was observed during IL-4 treatment. The platelet count decreased in 8/11 patients after the end of IL-4 therapy. World Health Organization (WHO) grade I/II fever, arthralgia and fatigue was observed in one-third of the patients and was more commonly seen with the highest dose (6 microg/kg/d). One patient developed pulmonary oedema and WHO grade III neutropenia was recorded in three patients. IL-4 was well tolerated by most patients in an outpatient setting. The anti-tumour activity observed in previous in vitro studies was not verified by the present in vivo trial which showed that IL-4 may instead increase the number of CLL cells in blood, indicating that IL-4 may have induced a stimulatory or antiapoptotic effect on the CLL cells in blood. These results may have important implications for the development of immunotherapy of CLL. In addition, the potential platelet-stimulatory effect of IL-4 warrants further studies.
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PMID:Interleukin 4 therapy for patients with chronic lymphocytic leukaemia: a phase I/II study. 1116 96

A prospective 12-month study was conducted throughout 1998 to determine the frequency of selected bacterial zoonoses as causes of fever among hospitalized Bedouins in southern Israel. One or more zoonoses were diagnosed in 30 (27%) of 110 patients admitted with fever. Brucellosis was diagnosed in 9 (8%), rickettsial infections in 20 (18%), and ehrlichiosis in 2 (2%), one of whom had also evidence of rickettsial spotted fever infection. None of the patients was diagnosed with Q fever. Compared with patients without zoonoses, patients with zoonoses were younger (P = 0.01), fewer of them had underlying conditions (P < 0.02), they had a longer febrile period prior to hospitalization (P = 0.04), a significantly higher proportion had arthralgia (P = 0.02), rash (P = 0.03), and splenomegaly (P = 0.04) and a lower proportion had pathological findings on chest auscultation (P < 0.01). Patients with zoonoses were found to have more commonly anaemia (P = 0.03) and leucopenia (P = 0.02) compared to the rest of the study population. Of the 30 patients with zoonoses 60% were misdiagnosed and only 57% received adequate antibiotic treatment. Zoonotic infections are a common cause of fever in adult Bedouins living in southern Israel. Because of the non-specific features of these diseases they are often misdiagnosed. Blood cultures and multiple serological tests should be used in the investigation of fever in such patients and tetracycline should be considered for initial empirical treatment.
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PMID:Zoonotic infections as causes of hospitalization among febrile Bedouin patients in southern Israel. 1149 Oct 3

From an association of nearly 50 years, the author had diagnosed biopsy proven 200 cases of sarcoidosis in Eastern India during the past three decades. It appears that most of these cases follow a distinct clinical pattern and presentation. The clinical course and prognosis differ considerably from that seen in Caucasians, Afro-Americans, South-African Bantus and Japanese. Males, above 40 years, coming largely from atopic and wealthier section of society (a particular business community with physicians, nurses with their families and other professionals). Patients present with constitutional symptoms (97%) like slow unrecognized fever with little malaise (fever-malaise dissociation in 70%), arthralgia (61%) or lone-myalgia (13%), appreciable loss of weight (33%), irritability, anorexia, respiratory symptoms (93%) like cough, dyspnea, etc., hepatomegaly (43.5%), splenomegaly (32.5%), lymphadenopathy (22%) with raised ESR (91%), hypergammaglobulinaemia (41.5%), hypercalciuria (40.5%), raised serum angiotensin converting enzyme (SACE) in 70.5% advance disease in chest radiograph (68%), positive 67-gallium scan and clinico-radiological dissociation in 81% (alarming looking chest radiograph with few physical signs). Course and prognosis also differ from the West. A different treatment schedule, avoiding oral prednisolone, has been found quite effective.
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PMID:Sarcoidosis: a journey through 50 years. 1243 38

In this study we aimed to investigate the findings in patients with adult-onset Still's disease (AOSD) admitted with fever of unknown origin (FUO) during the last 18 years in our unit, in order to discover the ratio of such patients to all patients with FUO during the same period, and to determine the clinical features of AOSD in FUO. The number and the aetiologies of the patients with FUO diagnosed between 1984 and 2001, and the clinical features of those with AOSD, were taken from the patient files. The diagnosis of AOSD was reanalysed according to the diagnostic criteria of Cush et al. [11]. The presumed diagnoses before a diagnosis of AOSD was established were also noted. The chi(2) and Fisher's exact tests were used for statistical analysis. We studied 130 patients with a diagnosis of FUO, 36 (28%) of whom had collagen vascular diseases. Of these 36 patients, 20 (56%, 12 female, 8 male, mean age 34 years, range 16-65) had AOSD. Clinical and laboratory findings were as follows: fever (100%), arthralgia (90%), rash (85%), sore throat (75%), arthritis (65%), myalgia (60%), splenomegaly (40%), hepatomegaly (25%), lymphadenopathy (15%), anaemia (65%), neutrophilic leukocytosis (90%), increased erythrocyte sedimentation rate (100%), elevated transaminase levels (65%), a negative RF (100%), and a negative FANA (80%). Antibiotics had been prescribed in 18 (90%) of cases. The presumed infectious diagnoses were streptococcal tonsillitis/pharyngitis (50%), infective endocarditis (four patients), sepsis (two patients) and acute bacterial meningitis (two patients). The presumed non-infectious diagnoses were acute rheumatic fever (three patients), seronegative rheumatoid arthritis (two patients) and polymyositis (two patients). Sixteen patients were followed for a mean duration of 30 months (range 2-59). A remission was obtained with indomethacin in three cases (19%), and with prednisolone in the remainder. Relapse was detected in three cases (19%). AOSD is one of the most frequent aetiologies of FUO. During the diagnostic course of a patient with FUO, a maculopapular rash and/or arthralgia and/or sore throat should raise the suspicion of AOSD. Because the disease has heterogeneous clinical findings, certain bacterial infections (e.g. streptococcal pharyngitis and sepsis) are generally considered and the prescribing of antibiotics is common.
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PMID:Fever of unknown origin: a review of 20 patients with adult-onset Still's disease. 1274 Jun 70

Kikuchi-Fujimoto disease (KFD), also know as histiocytic necrotizing lymphadenitis, is a benign disorder characterized histologically by necrotic foci surrounded by histiocytic aggregates, and with the absence of neutrophils. KFD was recognized in Japan, where it was first described in 1972. The disease is most commonly affecting young women. The cause of the disease is unknown, and its exact pathogenesis has not yet been clarified. Many investigators have postulated viral etiology of KFD, connecting it with Epstein Barr virus, human herpes simplex virus 6 parvo B 19, but also with toxoplasmic infection. Kikuchi-Fujimoto disease is usually manifested with lymphadenopathy and high fever, and is associated with lymphopenia, splenomegaly, and hepatomegaly with abnormal liver function tests, arthralgia, and weight loss. The disease has the tendency of spontaneous remission, with mean duration of three months. Single recurrent episodes of KFD have been reported with many years' pauses between the episodes. Kikuchi-Fujimoto disease may reflect systemic lupus erythematosus (SLE), and self-limited SLE-like conditions. Final diagnosis could only be established on the basis of typical morphological changes in the lymph node, and lymph node biopsy is needed for establishing the diagnosis. Lymphadenopathy in a patient with fever of the unknown origin could provide a clue to the diagnosis of lymphoma, tuberculosis, metastatic carcinoma, toxoplasmosis and infectious mononucleosis. As KFD does not have any classical clinical features and laboratory characteristics, it may lead to diagnostic confusion and erroneous treatment. We described a case of KFD, and suggested that this disease should be considered as a possible cause of fever of the unknown origin with lymphadenopathy.
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PMID:[Kikuchi-Fujimoto disease]. 1460 43

A 54-year-old female experienced morning stiffness and arthralgia of the hands from November 2001 and consulted her local doctor in January 2002. Hematological data showed liver dysfunction and antinuclear antibody was positive. In February 2002, the patient was admitted to our hospital to investigate these abnormalities of liver function further. The features of arthritis, thrombocytopenia with elevated platelet-associated IgG, positive antinuclear antibody, and anti-DNA antibody lead to a diagnosis of systemic lupus erythematosus. Liver biopsy revealed infiltration of Glisson's capsule by plasma cells, compatible with autoimmune hepatitis. We performed hepatic venography to investigate severe pancytopenia, remarkable splenomegaly and esophageal varices. Wedged hepatic venous pressure was mildly elevated, and hepatic veins displayed sharp angles, smooth walls and development of anastomosis with each other. These findings suggested idiopathic portal hypertension. Cytopenia and liver dysfunction gradually improved on treatment with 40 mg/day prednisolone. Esophageal varices were disappeared, and splenomegaly had improved after 6 months. As autoimmune factors are considered to underlie the development of idiopathic portal hypertension with systemic lupus erythematosus, steroid administration represents a therapeutic option in this condition.
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PMID:[Beneficial effect of glucocorticosteroids for esophageal varices due to idiopathic portal hypertension following systemic lupus erythematosus]. 1504 15

The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. Adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.
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PMID:A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis? 1546 68

This prospective study was carried out in two university hospitals between January 2000 and December 2002. The diagnosis of brucellosis was made with compatible clinical findings, positive Brucella agglutination > or =1/160 titres, and/or the isolation of Brucella species. The patients were followed up without intervention. One hundred and thirty-eight patients with active brucellosis were evaluated. Of the participants, 79 (57.2%) cases were acute, 23 (16.7%) sub-acute and 36 (26.1%) chronic. Brucella melitensis was isolated in the specimens of 24 (26.9%) out of 89 patients. The most frequent symptoms were fever (78.3%), arthralgia (77.5%) and sweating (72.5%). The most common physical findings were fever (40.6%), splenomegaly (36.2%), and hepatomegaly (26.8%). The osteoarticular involvement was found in 64 patients (46.4%). Ten (7.5%) patients had orchiepididymitis. Meningitis, pulmonary involvement, endocarditis, and hepatitis were found in five (3.6%), three (2.1%), two (1.5%) and one (0.7%) patient, respectively. Relative lymphomonocytosis was found in 80 cases (58.8%), anaemia in 46 (33.3%) and leucopoenia in 30 cases (21.7%). Clinical relapse was observed in 14 patients (10.1%).
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PMID:Clinical and laboratory features of brucellosis in two university hospitals in Southeast Turkey. 1648 39

The purpose of this study was to evaluate clinical and laboratory findings, treatment modalities and final outcomes of brucellosis in children and to compare our data with those of other studies performed in Greece. Fifty-two children treated for brucellosis in the Department of Pediatrics during the decade 1995-2004 were analyzed. Of the 52 children, 47 were reexamined during July 2005. Fever, arthritis or arthralgia, hepatomegaly and splenomegaly were the main findings. Young children had positive blood cultures and lower or negative antibody titers statistically significantly more often than did older children. Brucella abortus was isolated in 9 of 18 patients with positive blood cultures. Antibiotic treatment lasted for 28 days on average. There were no complications or relapses, except one, and the final outcomes were excellent.
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PMID:Presentation of childhood brucellosis in Western Greece. 1678 95

The aim of this study was to evaluate the brucellosis patients inhabiting in Mus province, in Eastern Anatolia of Turkey, retrospectively. The mean age of the patients (n: 87) was 38.1 +/- 12.4 years, and 45% of them were female. The transmission route was the consumption of unpasteurized fresh cheese (in 85%), and unboiled milk (in 45%). The most common symptoms were recorded as chills (89%), fever (87%), and arthralgia (81%). Splenomegaly (71%) and hepatomegaly (63%) were the predominant physical examination signs. Diagnosis was made based on the clinical features and positive Rose-Bengal test result (93%), however, blood cultures could not be performed due to insufficient laboratory equipment. In 92% of the patients at least one complication has been detected indicating delayed admission to the hospital, while the most common complications were sacroileitis (79%) and spondylitis (44%). Streptomycin+doxycyclin, streptomycin+doxycyclin+ ciprofloxacin, and streptomycin+doxycyclin+ rifampicin combination therapies were used in 62%, 24% and 14% of the patients, respectively, for six weeks, resulting with complete cure.
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PMID:[Retrospective evaluation of brucellosis cases inhabiting in Mus province]. 1700 61


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