Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease, whose origin seems to lie in a acquired defect in the membrane of the pluri-potential hematopoietic cell. Chronic or intermittent acute hemolytic syndrome is the most frequent clinical manifestation, although in the literature there are also some references to the leukocytic and immunologic disorders of this disease. In this paper, we present the case of a 63-year-old patient with NPH who developed severe neutropenia and sustained febrile syndrome. In the past four years, she had suffered frequent episodes of fever and leukopenia, which apparently disappeared spontaneously. In the physical exploration, we observed hepatosplenomegaly. The hemogram showed mild iron deficiency anemia (hemoglobin 10.8 g/dl), severe neutropenia (neutrophil 0.3 x 10(9)/l) and significant reticulocytosis (610 x 10(9)/l). Iron deposits were greatly reduced in the marrow. Simultaneously to a new febrile episode and isolation of Escherichia coli in the urine, there was a severe anemization (hemoglobin 5 g/dl) and a significant thrombopenia (platelets 30 x 10(9)) resulting in a positive hemosiderinuria and sucrose test. The study of the leukocytic function showed a defect in the neutrophil chemotaxis, although a normal phagocytic capacity and microbicidal activity. In the following nine months, the patient had several severe infections, with intense but transitory pancytopenia, which always improved when treating the infection with antibiotics. The patient died due to a septic shock twelve months after the diagnosis. Recurrent febrile episodes and severe neutropenia are very rare in the PNH (less than 4% of the cases). The cause of these disorders is still unknown.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Recurrent infections, severe neutropenia and neutrophil chemotaxis defect in paroxysmal nocturnal hemoglobinuria]. 786 56

In a 77-year-old Caucasian female B-cell chronic lymphocytic leukaemia was diagnosed and classified as stage 1 according to the Rai classification. The disease remained stable and therefore no antileukaemic therapy had to be initiated. Four years after the initial diagnosis the patient developed hepatosplenomegaly, anaemia and leukopenia. Bone marrow biopsy revealed megakaryocytic myelosis supervening upon the pre-existing chronic lymphocytic leukaemia.
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PMID:Suppression of chronic lymphocytic leukaemia by megakaryocytic myelosis. 795 70

Out of 330 adult Systemic Lupus Erythematosus (SLE) cases who attended the Rheumatic Care Centre, Government General Hospital, 59 children were analysed. There was no case with onset before the age of 5 years. There were 49 females and 110 males (M:F = 1:4.9). The initial manifestations were fever (67%), arthritis (61%), skin rash (59%) and lymphadenopathy (27.1%). There was no case of Raynaud's phenomenon. Only 10.1% of patients presented with thrombocytopenic purpura. In the cumulative clinical features, arthritis in 86.6%, fever in 79.8%, skin rash in 69.4%, lymphadenopathy in 61% and hepatosplenomegaly in 39.9% were observed. Renal involvement was seen in 49.1%, neuropsychiatric manifestations in 27.1%, pleuropulmonary in 22% and cardiac manifestations in 10.2%. Anaemia was seen in 50.8%, leukopenia in 18.4%, thrombocytopenia in 11.8%, ANA in 100%, anti-dsDNA in 92.3%, anti-Sm in 34.7%, anti-SSA in 38.5%, anti-SSB in 15.4%, ACL in 30.8%, low C3 in 50% and false positive VDRL in 3.3%. Death occurred in 8 children, 3 due to infection, 2 due to renal causes, 1 due to cardiac and 2 due to central nervous system involvement.
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PMID:Childhood systemic lupus erythematosus in south India. 795 96

C57/BL/6 mice infected with LP-BM5 MuLV virus developed an AIDS-like disease (MAIDS) with splenomegaly, leukopenia, thrombocytopenia, anemia, decreased numbers of helper/inducer and suppressor/cytotoxic T-cells and decreased production of interferon alpha. We have shown previously that HIV-associated Kaposi's sarcoma tissue contains high levels of prostaglandin E2 (PgE2), and this inhibits interferon synthesis through a cAMP-dependent second-messenger process. In this study we treated groups of MAIDS-infected mice with combinations of pentoxifylline, an agent which increases cAMP and inhibits phosphodiesterases, and sodium meclofenamic acid, a PgE2 inhibitor. Treated mice showed: 1) significantly higher total leukocyte and platelet counts, 2) higher total L3T4+ (helper/inducer) and Lyt-2+ (suppressor-cytotoxic) T-cell population. Pathologic examination also showed significantly less hepatosplenomegaly and lymphadenopathy in animals treated with pentoxifylline and meclofenamic acid. Partly, PgE2-induced suppression of interferon alpha production may mediate expression of retrovirus infection in this murine model of AIDS.
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PMID:Pentoxifylline and meclofenamic acid treatment reduces clinical manifestations in a murine model of AIDS. 830 44

Visceral leishmaniasis was experimentally induced in hamsters by the intracardiac inoculation of 10(7) amastigotes of Leishmania leishmania infantum of canine origin. At postinoculation (PI) days 7, 21, 42, and 63, hamsters were euthanatized. Body weights and total parasite numbers of the liver and spleen were determined. Gross and histologic evaluations of tissues were done. Dogs also were inoculated IV with 10(8) amastigotes/kg of body weight. Samples were obtained from dogs prior to infection and at biweekly PI intervals for CBC and serum chemical analysis, for lymphocyte blastogenic assay by use of blood leukocytes, and for ELISA to determine antileishmanial antibody titers. At PI week 12, dogs were necropsied; organ weights, tissue imprints of the liver and spleen, and histologic interpretations of tissues were obtained. Hamsters developed high parasite numbers within 7 days after inoculation, at which time the total parasite numbers in the liver (3.51 x 10(7) amastigotes) was observed to be approximately 11 times that in the spleen (2.93 x 10(6)). The liver had the highest parasite numbers throughout the infection period. Some infected hamsters became either cachectic and emaciated or ascitic. Two of the 10 infected hamsters died at PI days 54 and 58. Moderate to severe hepatosplenomegaly with granulomatous inflammatory reactions characterized by the presence of varied numbers of parasitized macrophages, giant cells, and hepatic Schaumann bodies were observed in infected hamsters. Infected dogs developed significantly altered hematologic values consisting of mild anemia and moderate leukopenia at PI weeks 8 to 12. Hyperproteinemia characterized by hyperglobulinemia (4.5 g/dl) was noticed at PI week 4.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Determination of virulence and pathogenesis of a canine strain of Leishmania leishmania infantum in hamsters and dogs. 842 54

We report a case of a female infant, from Acapulco Guerrero, Mexico. She had been sick for 45 days, with diarrhea and general malaise, fever during the last 20 days; at physical examination she was pale, with abdominal distention and hepatosplenomegaly. She had leukopenia, thrombocytopenia and anemia. The microscopic findings in the bone marrow sample were intracytoplasmic and extracellular bodies. Both bone marrow and blood cultures were positive for Histoplasma capsulatum. Seventy three pediatric cases of diseminated histoplasmosis have been described in the medical literature since 1934 to 1988. It is know that only about 1% of the persons that become infected will develop a diseminated disease.
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PMID:[Disseminated histoplasmosis in pediatrics. Report of a case]. 847 Nov 74

A newborn with symptoms of congenital cytomegalovirus infection (interuterine dystrophy, thrombocytopenia, leukopenia, hepatosplenomegaly, chronic pneumonia, pleocytosis in CSF) is described in whom tests for specific anti- CMV-IgM antibodies were negative. Changes typical for cytomegaloviral infection were found on autopsy. The authors discuss the difficulties in interpreting various serologic tests (CFT, anti-CMV IgM) in congenital infections, suggesting the necessity of introducing new diagnostic methods for better diagnosis and treatment.
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PMID:[Suppression of immunological response in a newborn with congenital cytomegalovirus infection: diagnostic difficulties]. 897 25

Clinical course and histopathologic features of a typical case of familial hemophagocytic lymphohistiocytosis (FHLH) are presented. FHLH, initially known as familial hemophagocytic reticulosis (FHR), is rare and without proper treatment is invariably rapidly fatal, usually accompanied by fever, anorexia, vomiting, irritability and pallor. Sporadic examples with prolonged survival have been reported. Other significant findings include hepatosplenomegaly, progressive anemia, leukopenia, thrombocytopenia, hyperlipidemia and hypofibrinogenemia. Varying degrees of hemophagocytosis by widely disseminated histiocytes in different organs and structures is one hallmark of the disease. Hemophagocytosis may also occur in viral and bacterial infections and in certain malignant processes. Very high parental consanguinity in FHLH was mentioned in two relatively recent reports. A probable immunologic defect has been the focus of recent investigations. The genetic defect is believed to be transmitted as an autosomal recessive trait.
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PMID:Familial hemophagocytic lymphohistiocytosis (FHLH). 909 88

A 27-year-old male suffered from Epstein-Barr virus (EBV)-related liver dysfunction with persistent hypogammaglobulinemia. IgG titers to EBV antigens were significantly high, while other hepatitis markers were negative. Liver biopsy disclosed active intralobular inflammation. Two years later, he manifested persistent fever, leukopenia, effusions and hypoproteinemia, and his general condition worsened progressively. The peripheral blood small lymphocytes predominantly expressed natural killer (NK)-like phenotypes (CD2+, CD7+, CD16+, CD56+). Hepatosplenomegaly and marked elevation of serum lactic dehydrogenase were observed. He died of respiratory failure at the age of 29. At autopsy, the liver (2190 g), spleen (860 g), small bowel and mesenteric lymph nodes showed massive infiltration of large atypical lymphoid cells in close association with hemophagocytic histiocytes. Involvement was mildly noted also in the bone marrow, lungs, gall-bladder and kidneys. The atypical cells belonged to CD30+ activated NK-type cells expressing CD2, cytoplasmic CD3 epsilon, CD7, CD45RO, CD56, HLA-DR and HLA-DQ. T cell receptors (TCR), surface CD3, CD4, CD5 and CD8 were not expressed. Epstein-Barr virus-related small nuclear RNA (EBER1) and Epstein-Barr virus-associated nuclear antigen 1 were detected in the nuclei of a significant number of atypical cells, while EBV-related latent membrane protein-1 was negative. EBER1 was also identified in the nuclei of non-neoplastic small lymphocytes at both biopsy and autopsy. Monoclonal integration of the EBV genome into the lymphoma cells was shown by Southern blot analysis. Clonal rearrangement of TCR was undetectable. Roles of chronic active EBV infection in the development of NK cell-type malignancy resembling malignant histiocytosis are discussed.
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PMID:Epstein-Barr virus (EBV)-induced CD30+ natural killer cell-type malignancy resembling malignant histiocytosis: malignant transformation in chronic active EBV infection associating hypogammaglobulinemia. 921 26

This report details a case of infection associated hemophagocytic syndrome (IAHS). A 20-year-old female was admitted to our hospital with persistent high fever in July, 1994. Physical examination revealed high body temperature (40 degrees C), marked hepatosplenomegaly and no superficial lymph node swelling. Laboratory examination revealed leukopenia and abnormal liver function on admission. Serum ferritin levels were surprisingly elevated. The coagulation tests showed high FDP and D-dimer. Specific viral antibody titers were not elevated such as Epstein-Barr virus or Cytomegalovirus. Bone marrow examination revealed histiocytic hyperplasia with hemophagocytosis, and the histiocytes were well matured. We diagnosed IAHS. Corticosteroids were administered on the 3rd hospital day (methylprednisolone 1 g/day, 3 days), but persistent high fever and laboratory findings did not improve. So we tried etoposide (etoposide 200 mg/day, 5 days) therapy on the 13th hospital day. After administration of etoposide, she failed to recover from severe leukopenia and suffered from meningitis. We administered G-CSF, gamma-globulin and antibiotics for intensive supportive therapy. As the leukocyte count increased, her symptoms and laboratory data improved. There was no hemophagocytosis in her bone marrow before discharge. Recently, etoposide is said to be effective for reactive monocytic proliferation. Administration of etoposide was very effective for IAHS, although corticosteroids, were ineffective.
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PMID:[A case of IAHS (infection associated hemophagocytic syndrome) successfully treated with etoposide]. 942 72


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