Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032285 (pneumonia)
 54,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 70-year-old man, who had been diagnosed as primary myelofibrosis in 1987 at Tokyo Women's Medical School, was admitted to our hospital because of left hip-joint pain in May 1988. Physical examinations revealed marked hepatosplenomegaly and multiple reddish papules on the skin. The peripheral blood showed marked leukoerythroblastosis and severe anemia with poikilocytosis. Bone marrow aspirations were dry tap. Needle biopsy of iliac crest showed a diffuse fibrosis. Biopsy of the papules showed an extramedullary haematopoiesis. He was treated with low dose Ara-C for seven days. Although hepatosplenomegaly and skin papules were reduced, he died of pneumonia a month later. At autopsy, leukemic cells massively infiltrated into the multiple organs, including bone marrow. The cells were identified with megakaryoblast, as those were positive for factor VIII related antigen. As far as we know, this is the third case of primary myelofibrosis transformed into acute megakaryoblastic leukemia.
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PMID:[Primary myelofibrosis with extramedullary haematopoiesis of the skin transformed into acute megakaryoblastic leukemia]. 232 83

Serum specimens from 252 recipients of factor VIII and/or factor IX (237 hemophiliacs and 15 nonhemophiliacs), 99 blood transfusion recipients, 269 chronic diseases, and 366 healthy subjects (included blood donors, hospital personnel and family members of hemophiliacs) were tested for reactivity to lymphadenopathy-associated virus/T-lymphotropic retrovirus type III (LAV/HTLV-III) by ELISA test kit (Abbott), and the presence of the antibodies to LAV/HTLV-III was confirmed by indirect immunofluorescence assay (IFA). Seropositivity rates were 38.4% (91 of 237) in hemophiliacs, 13.3% (2 of 15) in nonhemophiliac recipients of factor VIII and/or factor IX, and 8% (2 of 27) in male homosexuals. None of the other patients and healthy individuals had antibodies to LAV/HTLV-III. In serological follow-up study on 40 seronegative and 10 seropositive hemophiliacs, 4 of the seronegative hemophiliacs converted to seropositive, and 7 of seropositive hemophiliacs had the antibody titers elevated by 2 to 4 fold within the period for 3.5 to 13 months after first blood samplings. Comparative analysis of the absolute numbers of T helper (T4) and T suppressor (T8) lymphocytes between the seropositive and seronegative hemophiliacs revealed that more than 50% of both seropositive and seronegative hemophiliacs had lower T4 lymphocyte numbers and higher T8 lymphocyte numbers than those of normal subjects. Particularly, over 80% of seropositive hemophiliacs had lower T4 lymphocyte numbers. Two Japanese AIDS cases (a hemophiliac B and a male homosexual) were found in the present study. Both died of pneumonia, and were confirmed officially as AIDS cases.
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PMID:Prevalence of antibodies to AIDS-associated retrovirus in in- and out-patients in Japan. 310 40

A 71-year-old previously healthy woman developed unexplained thrush, onychomycosis, Pneumocystis carinii pneumonia, and a T-cell defect consistent with the acquired immunodeficiency syndrome. Her only apparent risk factor was infrequent, monogamous sexual contact with her husband, a 74-year-old hemophiliac who had received factor VIII concentrate and subsequently died due to P. carinii pneumonia. She first developed probable signs of the syndrome (recurrent thrush and onychomycosis) 1 year before her husband became ill. These findings suggest that the syndrome can be transmitted heterosexually by an asymptomatic person, and that the female sexual partners of hemophiliacs treated with factor VIII concentrate may be at risk of acquiring the syndrome; frequent sexual contact or several sexual partners are not necessary for transmission to occur; and the syndrome can occur in elderly people if exposed to risk factors. This case further supports the theory that the syndrome in hemophiliacs is due to an infectious agent that can be transmitted heterosexually as well as parenterally.
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PMID:The acquired immunodeficiency syndrome in the wife of a hemophiliac. 622 3

Neonatal group B streptococcal (GBS) sepsis and pneumonia cause lung endothelial cell injury. GBS invasion of the lung endothelium may be a mechanism for injury and the release of vasoactive eicosanoids. Pulmonary artery endothelial cells (PAEC) and lung microvascular endothelial cells (LMvEC) were isolated from neonatal piglets and were characterized as endothelial on the basis of morphology, uptake of acyl low-density lipoprotein, factor VIII staining, and formation of tube-like structures on Matrigel. PAEC and LMvEC monolayers were infected with COH-1 (parent GBS strain), isogenic mutants of COH-1 devoid of capsular sialic acid or all capsular polysaccharide, or a noninvasive Escherichia coli strain, DH5 alpha. Intracellular GBS were assayed by plate counting of colony-forming units resistant to incubation with extracellular antibiotics. All GBS strains invaded LMvEC significantly more than PAEC, showing that the site of lung endothelial cell origin influences invasion. DH5 alpha was not invasive in either cell type. Both isogenic mutants invaded PAEC and LMvEC more than COH-1 did, showing that GBS capsular polysaccharide attenuates invasion. Live GBS caused both LMvEC and PAEC injury as assessed by lactate dehydrogenase release; heat-killed GBS and DH5 alpha caused no significant injury. Supernatants from PAEC and LMvEC were assayed by radioimmunoassay for prostaglandin E2 (PGE2), the stable metabolite of prostacyclin (6-keto-PGF1 alpha), and the thromboxane metabolite thromoxane B2. At 4 h, live COH-1 caused no significant increases in eicosanoids from both PAEC and LMvEC. At 16 h, live COH-1, but not heat-killed COH-1, caused a significant increase in 6-keto-PGF1 alpha greater than PGE2 from LMvEC, but not PAEC. We conclude that live GBS injure and invade the lung microvascular endothelium and induce release of prostacyclin and PGE2. We postulate that GBS invasion and injury of the lung microvasculature contribute to the pathogenesis of GBS disease.
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PMID:Group B streptococci (GBS) injure lung endothelium in vitro: GBS invasion and GBS-induced eicosanoid production is greater with microvascular than with pulmonary artery cells. 780 66

A 77-year-old man was admitted because of high fever and general malaise. Whole-body CT showed large bilateral adrenal masses and para-aortic lymphadenopathy. Needle biopsy of the adrenal mass revealed mononuclear tumor cells within the lumens of small vessels, indicating the features of angioendothelial lymphoma. Immunohistochemically, these tumor cells were B cell lineage (positive for LCA, Mx-pan B, MB-1, and negative for factor VIII-related antigen, UEA-1, QB/ENDO 10). We used a modified CHOP therapy (500 mg of cyclophosphamide, 40mg of 4'-o-tetrahydropyranyl doxorubicin, 2mg of vindesine, and 50 mg of prednisolone) due to the patient's advanced age and general condition. Following the first course of this modified CHOP, the adrenal masses and para-aortic lymph nodes decreased dramatically in size, and his subjective symptoms also improved remarkably. Following three courses of modified CHOP, the patient developed dementia and disturbance of consciousness. Brain MRI revealed enhanced multiple nodular lesions. A diagnosis of multiple brain metastasis was made and modified CHOP and radiation therapy were begun, which relieved his symptoms for some time. However, he later died of pneumonia with severe leukopenia. A postmortem examination was performed. Microscopic examination showed no residual tumor cells in the adrenal gland. brain lymph nodes, or other organs. These results suggest that modified CHOP and radiation therapy may have beneficial anti-tumor effects against angioendothelial lymphoma.
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PMID:[Modified CHOP and radiation therapy in an aged patient with angioendothelial lymphoma]. 802 99

Plasma cell leukemia (PCL) is a very rare variant of multiple myeloma (MM) occurring in about 2% of newly diagnosed patients. Plasma cell leukemia may develop during the course of MM (secondary PCL) or it can occur without any prior sign of MM (primary PCL). We report a case of aggressive primary PCL with unusual clinical, cytogenetic and molecular features. A 36-year-old male patient was first seen because of fever and bone pain. On the skin of his chest, back, abdomen, and palpebras, there were nodular infiltrations resembling urticaria. White blood cell count was 10.8 x 10(9)/l with 41% plasmacytes. Bone marrow aspiration was hypercellular, 93.5% of cells were atypical plasmacytes and plasmablasts. The cytogenetic analysis of G-banded chromosomes in bone marrow cells yielded the trisomy 8. The skin biopsy specimen showed intensive infiltrates of uninucleated blastic cells similar to those found in the bone marrow. Immunophenotyping of bone marrow and skin neoplastic cells showed CD45+, CD45Ro+, CD68+, CD38+ and cytoplasmic kappa light chain +. The neoplastic cells stained negatively for lambda light chain, CD3, CD20, CD30, EMA, CD15, CD34, CD56 and factor VIII. The pattern of IgL genes rearrangement in the bone marrow aspirate, peripheral blood mononuclear cells, and skin specimens was examined by PCR analysis. All studied specimens showed three different IgK gene configurations suggesting that the neoplastic cells originated as a result of oligoclonal lymphoproliferation process. The patient received two courses of VAD (vincristine, doxorubicin, dexamethasone) without improvement and three courses of CHOP with only temporary stabilization of the disease. He died 5 months after the diagnosis of PCL because of disease progression and pneumonia.
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PMID:Aggressive primary plasma cell leukemia with skin manifestations, trisomy 8 and molecular oligoclonal features. 1214 88

Acquired hemophilia is a life-threatening condition associated with a high mortality, occurring mainly in elderly patients. We report on a 92-year-old male, administered two courses of 90 microg kg(-1) of recombinant activated factor VII (rFVIIa) due to failure of human factor VIII (FVIII) therapy, without side effects nor evidence of thrombotic complications. Despite the bleeding being completely under control, the patient died on day 11 of acute respiratory failure due to pneumonia. This case confirms previous reports on the complexity and severity of this disorder, showing that rFVIIa is an effective and safe agent for achieving hemostasis even in elderly patients with a FVIII inhibitor.
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PMID:Recombinant activated factor VII therapy in acquired hemophilia of the elderly. 1573 2

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with diverse manifestations. We suggest that intravenous immunoglobulin (IVIg) therapy may be beneficial and safe for various manifestations in SLE. A structured literature search of articles published on the efficacy of IVIg in the treatment of SLE between 1983 and 2005 was conducted. We searched the terms "IVIg," "intravenous immunoglobulin," "lupus," "SLE," and "systemic lupus erythematosus." The various clinical manifestations of SLE that were reported to be successfully treated by IVIg in case reports include autoimmune hemolytic anemia, acquired factor VIII inhibitors, acquired von Willebrand disease, pure red cell aplasia, thrombocytopenia, pancytopenia, myelofibrosis, pneumonitis, pleural effusion, pericarditis, myocarditis, cardiogenic shock, nephritis, end-stage renal disease, encephalitis, neuropsychiatric lupus, psychosis, peripheral neuropathy, polyradiculoneuropathy, and vasculitis. The most extensive experience is with lupus nephritis. There are only a few case series of IVIg use in patients with SLE with various manifestations, in which the response rate to IVIg therapy ranged from 33 to 100%. We suggest that IVIg devoid of sucrose, at a dose of 2 g/kg over a 5-d period given uniformly and at a slow infusion rate in patients without an increased risk for thromboembolic events or renal failure, is a safe and beneficial adjunct therapy for cases of SLE that are resistant to or refuse conventional treatment. The duration of therapy is yet to be established. Controlled trials are warranted.
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PMID:Intravenous immunoglobulin therapy and systemic lupus erythematosus. 1639 97

In severe haemophilia A, patients, start from the first years of life, with spontaneous bleeding and require transfusion. However, cardiac tamponade due to spontaneous pericardial bleeding is rare. An 11-year-old boy receiving haemophilia A treatment was referred to the Department of Paediatric Haematology with pneumonia, fever, dyspnoea, and palpitation. In his PA chest radiograph, pneumonic infiltration in the right lung and enlargement in the pericardial area were found. On his echocardiograph, pericardial effusion reaching 3.9 cm and other findings of tamponade were detected. APTT was outside the measurable range. It was deranged to > 120 seconds. The patient received 1000 U of factor VIII intravenously. A pericardial window was made via left anterior mini thoracotomy due to fluid drained. In his control echocardiograph taken after one month, no pathology was found. At 50th day, the patient showed left pleural serohaemorrhagic effusion, which was treated with tube thoracostomy. In haemophilia A patients, either pericardiocentesis or subxiphoid pericardial drainage or pericardial window creation via thoracotomy may be applied, depending on the primary pathology. In paediatric cases, pericardial window creation via mini thoracotomy can be an alternative treatment of choice considering complications such as recurring bleeding and effusion during pericardiocentesis.
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PMID:Acute cardiac tamponade due to spontaneous bleeding in a child with haemophilia A. 2072 9

Acquired hemophilia A, also known as acquired factor VIII deficiency, is an exceedingly rare bleeding diathesis that does not require any personal or family history of bleeding or clotting disorder. Because treatment is available, misdiagnosing or completely missing this diagnosis can be life threatening for patients. Clinicians should be aware that acquired forms of hemophilia do exist and are associated with high morbidity and mortality in elderly adults. We present a case of a 74-year-old man who was diagnosed with acquired factor VIII inhibitor during an admission for community-acquired pneumonia.
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PMID:Acquired factor VIII inhibitor (acquired hemophilia A) presenting as spontaneous blood-filled scrotum. 2160 23


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