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

We report a series of seven patients with reactive hemophagocytic syndrome, which was quite characteristic of its etiological spectrum. Infections were the leading cause, among them a case associated with HIV and another one with Salmonella enteritidis (a hitherto unreported association). The clinical findings consisted of fever, hepatomegaly, splenomegaly, lymphadenopathy, rash and pancytopenia. The diagnosis was carried out by bone marrow aspiration-biopsy except in two patients who were diagnosed at autopsy. The difficulty of the differentiation from malignant histiocytosis is discussed: one case of hemophagocytic syndrome due to diphenylhydantoin toxicity (the second reported one in the literature) was histologically undistinguishable from it. We think that, in any etiology, hemophagocytic syndrome is a reactive syndrome with variable intensity. The need for extensive microbiological investigation even in cases of histiocytosis of neoplastic appearance is emphasized.
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PMID:[Reactive hemophagocytic syndrome: analysis of a series of 7 cases]. 232 64

We had shown previously that the prevalence of human T-cell leukemia/lymphoma virus type I (HTLV-I)-antibody positivity is high in Jamaican non-Hodgkin's lymphoma (NHL) patients and that virus-positive patients have the clinical features and poor prognosis of adult T-cell leukemia/lymphoma (ATL). Sixty-two % of 45 NHL patients diagnosed consecutively between 2/1/82 and 1/31/84 and studied prospectively were HTLV-I-antibody positive. Skin involvement (38%), hypercalcemia (44%), and leukemia (40%) were unusually prevalent and there was a strong association (p less than 0.05) with HTLV-I-antibody positivity. Fifty-two % of the patients had bone marrow infiltration, and 74% of these patients were HTLV-I-antibody positive (p = 0.06). Lymphadenopathy (96%), hepatomegaly (60%), and splenomegaly (25%) were detected with about the same frequency as in other series of NHL patients with advanced disease, and 61-88% of these patients were HTLV-I-antibody positive. Patients were classified into those with "typical ATL" (NHL associated with 2 of the 4 features i) hypercalcemia; ii) histologically proven skin infiltration; iii) leukemia; and iv) bone marrow infiltration, providing that the morphology of infiltrating or leukemic cells was characteristic of ATL; those "consistent with ATL" (NHL associated with 1 of these 4 features); and "non-ATL" (NHL without any of these 4 additional features). Thirty-two (71%) of the NHL patients were ATL patients, i.e., had features typical of or consistent with ATL, and 78% of these were HTLV-I-antibody positive. HTLV-I provirus was detected in tumour cells of all HTLV-I-antibody positive patients tested. Three (23%) of the non-ATL patients were HTLV-I-antibody positive. There was no correlation between histopathological features and the clinical classification or HTLV-I-antibody positivity. Median survival of ATL and non-ATL patients was 16 and 53 weeks. Although the disease was usually fulminant, 34% of the ATL patients had a subacute or chronic course. Skin involvement and leukemia were prominent in these patients. Hypercalcemia was the chief prognostic determinant. Median survival of hypercalcemic and normocalcemic ATL patients was 13 and 86 weeks (p less than 0.05). Hypercalcemia caused 10 deaths, infections 12, and death was due to tumour progression in 4 patients. Infections were usually due to pyogenic organisms and only 2 patients had systemic opportunistic infections. Six (27%) of 22 chronic lymphocytic leukemic (CLL) patients were HTLV-I-antibody positive.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Adult T-cell leukemia/lymphoma in Jamaica and its relationship to human T-cell leukemia/lymphoma virus type I-associated lymphoproliferative disease. 610 Jun 52

Malaria and filariasis surveys were carried out as part of a broader general health survey between December, 1982 and May, 1983 in the Ok Tedi region of the Star Mountains, Western Province. Malaria, tropical splenomegaly syndrome (TSS) and anaemia were identified as significant health problems. Malaria slide positivity rates of 64.9% in children 2 to 9 years of age and 19.5% in adults 15 years and older indicate high levels of stable malaria transmission. Infections with Plasmodium falciparum were the most common (75.2%), but P. vivax (17.4%) and P. malariae (7.4%) were also encountered. Palpable splenomegaly occurred in 79.2% of adults and children over two years of age with more than 50% of the enlarged spleens grade III or greater (Hackett). Microfilariae (Wuchereria bancrofti) were present in 34.3% of night blood films, and estimated haemoglobin values were considerably below WHO standards. Data from the surveys provide a baseline against which to monitor changes in health status which might be expected to occur in conjunction with the development of a major mining project in the area.
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PMID:Malaria and filariasis in the Ok Tedi Region of the Star Mountains, Papua New Guinea. 659 55

The authors reviewed the clinical course of 31 consecutive patients with hairy cell leukemia seen at the University of California Los Angeles. The clinical presentation included varying degrees of pancytopenia, splenomegaly, and bone marrow infiltration with hairy cells. Ten patients were identified as having an "atypical" disease, which is defined as absence of palpable splenomegaly and/or marrow cellularity of less than 45%. These atypical patients had clinically milder disease and significantly less anemia than the usual patient (mean hemoglobin, 12.1 g/dl versus 9.4 g/dl; P = 0.016), although neutropenia and thrombocytopenia were comparable. Mortality and infection rates were similar in both groups. Infections were common in all patients, but opportunistic infections and septicemia were rare in patients prior to initiation of therapy. Two thirds of the patients who received corticosteroids and/or cytotoxic agents had serious infections, with a 50% mortality rate. Nearly 70% of the neutropenic patients (leukocyte count less than 1000) who received any form of treatment had a serious infection. The most important factors predicting mortality were chemotherapy and an age older than 50 years. Patients who survived 2 years with their disease had an excellent prognosis, and four patients in this series are alive and well with their disease for more than 10 years.
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PMID:Hairy cell leukemia. Disease pattern and prognosis. 673 79

Infection with Mycobacterium bovis (BCG) or injection of killed Corynebacterium parvum protected some strain B6D2 F1 (C57BL/6xDBA/2) mice but did not protect strain ICR or A mice from lethal challenge with Plasmodium berghei strain NYU-2. B6D2 mice were not protected against challenges delivered immediately after intravenous injection of these materials, but rather protection developed by day 7 and persisted through at least day 84. Infections in protected mice progressed to about 10% parasitemia in parallel with infections initiated with the same inoculum in untreated controls. However, infections in most of the protected mice were cleared subsequently, whereas infections in untreated controls were uniformly fatal. A small number of treated mice developed protracted high-level erythrocytic infections, which led to markedly delayed death. BCG-infected mice which survived P. berghei infections had a factor in their sera which protected passively immunized recipients from P. berghei. BCG-infected mice passively immunized with protective serum controlled P. berghei infections better than normal mice given the same amount of the same serum and challenged with the same P. berghei inoculum. The capacity of BCG-infected B6D2 mice to resist P. berghei infection was not directly related to the pattern of growth of BCG, to the degree of splenomegaly, or to the level of activation of macrophages (measured as microbicidal capacity) caused by BCG infection. Therefore, I concluded that (i) BCG infection or injection of killed C. parvum altered the immunological potential of B6D2 mice in such a way as to allow the production of measurable levels of a protective humoral factor in response to infection with P. berghei; (ii) BCG infection caused the generation of a capacity which, when expressed in the presence of immune serum, provided an anti-P. berghei capacity which was superior to that provided by BCG infection alone or immune serum in the absence of BCG infection; and (iii) not all strains of mice could be protected from P. berghei by BCG or C. parvum injection.
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PMID:Host defenses in murine malaria: nonspecific resistance to Plasmodium berghei generated in response to Mycobacterium bovis infection or Corynebacterium parvum stimulation. 702 24

Hairy-cell leukaemia is an indolent lymphoproliferative malignancy characterized by infiltration of the bone marrow, liver, spleen, and occasionally lymph nodes with a malignant B cell with hair-like cytoplasmic projections. This involvement leads to splenomegaly with secondary consumption of red cells, platelets and neutrophils as well as other complications of an enlarged spleen, including infarction-or-rarely rupture. The common haematological complications of anaemia, neutropenia and thrombocytopenia are due not only to the enlarged spleen but probably also to hairy cells in the bone marrow inducing cytokine-mediated suppression of haematopoiesis. Hepatic involvement, although frequent, only occasionally leads to liver dysfunction. Infections are a major cause of morbidity and mortality in patients with hairy-cell leukaemia, presumably owing to neutropenia and monocytopenia in these patients. The infections seen may be due to unusual pathogens, including Mycobacterium and Listeria. Autoimmune disease, including polyarthitis and vasculitis, occurs frequently and does not correlate with the severity of the disease. Other rare complications include bone involvement, meningitis and ascites. A wide range of secondary malignancies have been reported in patients with hairy-cell leukaemia, but it is still unclear whether the incidence is increased and whether they are related to the disease or treatment.
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PMID:Clinical manifestations and infectious complications of hairy-cell leukaemia. 1267 Apr 63

Infections with 3 species of malaria parasites are rarely encountered and observed in less than 0.05% of cases. We came across such an infection in four year-old, monozygote twin sisters, coming from Kinshasa (Democratic Republic of Congo). In both of them, parasitemia was low or very low for P. falciparum and P. ovale and of 0.1-0.2% for P. malariae. The twin sisters presented with an iron deficiency anaemia, associated with an heterozygous sickle-cell anaemia and a moderate splenomegaly. The biological tests results were similar. They responded well to treatment. We point out the difficulty in recognizing the concomitant presence of several species of hematozoaire on blood smear.
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PMID:[Triple malaria infection in twin sisters from the Democratic Republic of Congo]. 1283 24

Sarcoidosis is a granulomatous disease of unknown origin, with pulmonary findings in more than 90% of patients. Extrapulmonary involvement is common and all organs can be involved (especially lymph nodes, eyes, joints, central nervous system) but it is rare to find an isolated extrapulmonary disease (less than 10% of patients). Granulomatous inflammation of the spleen and the liver is common in patients with systemic sarcoidosis, while hepatosplenic enlargement is unusual and splenic involvement rare. We report two cases of systemic sarcoidosis, that onset with splenic and hepatosplenic disease, and one case with splenic sarcoidosis without pulmonary involvement. In the first case a 53-year-old woman with mild abdominal pain underwent sonography and CT, which revealed one hypoechoic/hypodense splenic lesion. Laboratory tests were normal. In order to exclude a lymphoma, splenectomy was performed: histology revealed a sarcoid granuloma. After surgery the patient was asymptomatic and now, after two years, disease is silent. The second case is a 66-year-old woman with a recent weight loss (8 kg in two months) and alterated liver function tests (AST 61 U/l, ALT 72 U/l, Alkaline phosphatase 748 U/l, g-GT 381 U/l). Since she had a familiar history of colon cancer, abdominal US scan, abdominal CT scan and MRI were performed and showed inter-aorto-caval lymphadenopathies and discreet multiple bilobar hepatic and splenic substitutive lesions, with no signs of primary tumor. Upper and lower GI endoscopy, full gynecological workup, complete set of tumor markers, bone marrow biopsy were performed. All resulted negative for neoplasia. Small pulmonary infiltrations were observed on chest-CT scan but cytology on BAL was normal. Infections were also excluded. An exploratory laparotomy showed whitish peritoneal, hepatic and splenic nodules. The histological exam revealed chronic granulomatous lesions typical for sarcoidosis. During a two-year follow-up after the splenectomy the patient feels well without any treatment. The third patient is a 32-year-old woman with mild epigastric pain after meals. Neck-thoracic CT, bone scintigraphy and upper GI endoscopy were negative. Abdominal US and MR showed splenomegaly with multiple splenic lesions. Splenectomy was performed and histological exam showed chronic granulomatous lesions typical for sarcoidosis. Further laboratory tests were normal, except for ACE (66 UI/l). After the surgery ACE became normal and now, three years later, the patient is still asymptomatic. We conclude that hepatosplenic involvement is less rare than it is thought. It is often oligosymptomatic or accompanied with unspecific manifestations and laboratory abnormalities. The diagnosis could be difficult; in fact typical laboratory findings of sarcoidosis such as ACE, lysozyme, calcium, were not diagnostic. Ultrasonography and CT were important but the diagnosis was established only with the histological examination of suspected lesions. This latter required to differentiate liver and/or spleen sarcoidosis from tuberculosis and other infections, primary biliary cirrhosis, metastasis or malignant lymphoma.
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PMID:Atypical sarcoidosis: case reports and review of the literature. 2138 7

This is a retrospective analysis of the medical records of 116 patients who presented to the gastroenterology division, department of medicine at King Khalid University Hospital (KKUH) in Riyadh, Kingdom of Saudi Arabia and subsequently had a histopathologic diagnosis of hepatic granulomas. Infections contributed to 56% and were represented mainly by schistosomiasis and to a lesser extent by tuberculosis, brucellosis and hydatid disease. Lymphomas (8%) were the major representative of noninfectious causes. The etiology of 25% of granulomas remained undetermined. Weight loss, fever, anorexia and abdominal pain were the most frequent presenting symptoms in 53, 45, 43 and 42% of patients, respectively. Ten percent of the patients were asymptomatic. Hepatomegaly and splenomegaly were the predominant physical findings in 55% and 43% of patients respectively. Hepatic granulomas in this study are mainly caused by infections. Schistosomiasis, tuberculosis and brucellosis represented the most common etiologic factors.
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PMID:Hepatic granulomas in an arab population: a retrospective study from a teaching hospital in Riyadh. 1986 28

The postgenomic era has revolutionized approaches to defining host-pathogen interactions and the investigation of the influence of genetic variation in either protagonist upon infection outcome. We analyzed pathology induced by infection with two genetically distinct Trypanosoma brucei strains and found that pathogenesis is partly strain specific, involving distinct host mechanisms. Infections of BALB/c mice with one strain (927) resulted in more severe anemia and greater erythropoietin production compared to infections with the second strain (247), which, contrastingly, produced greater splenomegaly and reticulocytosis. Plasma interleukin-10 (IL-10) and gamma interferon levels were significantly higher in strain 927-infected mice, whereas IL-12 was higher in strain 247-infected mice. To define mechanisms underlying these differences, expression microarray analysis of host genes in the spleen at day 10 postinfection was undertaken. Rank product analysis (RPA) showed that 40% of the significantly differentially expressed genes were specific to infection with one or the other trypanosome strain. RPA and pathway analysis identified LXR/RXR signaling, IL-10 signaling, and alternative macrophage activation as the most significantly differentially activated host processes. These data suggest that innate immune response modulation is a key determinant in trypanosome infections, the pattern of which can vary, dependent upon the trypanosome strain. This strongly suggests that a parasite genetic component is responsible for causing disease in the host. Our understanding of trypanosome infections is largely based on studies involving single parasite strains, and our results suggest that an integrated host-parasite approach is required for future studies on trypanosome pathogenesis. Furthermore, it is necessary to incorporate parasite variation into both experimental systems and models of pathogenesis.
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PMID:Role for parasite genetic diversity in differential host responses to Trypanosoma brucei infection. 2008 91


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