Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hairy cells from eight patients with hairy cell leukemia were evaluated with both light and transmission electron microscopy for their capacity to phagocytose zymosan, latex, staphylococcus aureus, and pseudomonas aeruginosa. In two patients, there was no phagocytosis of any of these substances; cells from three patients phagocytosed only latex; two, all except pseudomonas; and one, all 4 substances. Hairy cells became relatively smooth while in culture with staphylococcus, but no surface changes were noted during incubation with the other substances. Of the eight patients studied, one died of pseudomonas pneumonia and sepsis; pseudomonas was the only substance which her hairy cells did not phagocytose. The one patient whose hairy cells phagocytosed all 4 test substances developed a disseminated Mycobacterium intracellulare infection; culture of his hairy cells with this atypical myocbacterium showed no phagocytosis. Hairy cells have different phagocytic capabilities from patient to patient, and the evaluation of these capabilities in vitro might provide early identification of potential infectious complications.
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PMID:Hairy cell leukemia: differences in phagocytic capacity of cells in vitro. 3 38

Lymphadenopathy is an uncommon finding in hairy cell leukaemia (HCL). We report 12 HCL patients in whom relapse was associated with massive abdominal lymphadenopathy. All but one had long-standing HCL (range 3-25 years; median 10 years); in one it was discovered at presentation. Nine patients had been splenectomized and seven had previously been treated with 2'deoxycoformycin (DCF) and/or alpha-interferon (alpha IFN): three had achieved complete remission and four a partial response. The computerized tomography (CT) scan appearances were similar in all cases with a primary lymph node mass centred around the coeliac axis and involving upper para-aortic and retropancreatic regions. Histology and/or cytology confirmed nodal involvement by HCL in six patients. Large immature hairy cells were seen in both lymph nodes and bone marrow, suggesting a degree of transformation. Nine patients were treated with DCF: one had complete resolution, six responded with 50-90% reduction of the lymphadenopathy, one did not respond and one is still on treatment; alpha-IFN was used concomitantly or sequentially in two of the responders. One responding patient died of sepsis after four injections of DCF. Three patients received either alpha- or beta-IFN alone with no response. One elderly patient was not treated. Abdominal lymphadenopathy could be part of the natural history of HCL and/or may represent a transformation analogous to that seen in other low-grade lymphoproliferative disorders. Routine abdominal CT scanning should be part of the work up of all patients with HCL.
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PMID:Massive abdominal lymphadenopathy in hairy cell leukaemia: a report of 12 cases. 128 78

We administered one course of 2-chlorodeoxyadenosine (2CdA) at 4 mg/m2 daily for 7 days by continuous intravenous infusion to 46 patients with hairy cell leukemia. Complete remissions occurred in 36 patients (78%; 95% confidence limits, 63% to 89%), partial remissions in five (11%), and a minor response in one. One patient died of candida sepsis 3 weeks after beginning treatment and three patients were clearly resistant to therapy. These three either had morphologically atypical hairy cells, less than 20% of which expressed Ig light chain on the cell surface, or had failed prior treatment with deoxycoformycin and interferon-alpha. At a median of 37 weeks since discontinuation of therapy, recurrent thrombocytopenia has developed in one patient, whose marrow remains normal, while a bone marrow relapse has occurred in another patient, whose blood counts remain normal. Treatment produced a greater than 50% decrease in neutrophil count in 26 patients, which lasted 3 to 4 weeks and was associated with an increased incidence of febrile episodes. These episodes occurred in 21 patients but were associated with documented infection in only four patients. Decreases in the number of CD4+ lymphocytes appeared to occur regularly after treatment and have persisted for a median of 18 weeks without obvious clinical significance. Although years of follow-up will be needed, our results confirm Piro et al's observation (N Engl J Med 322: 1117, 1990) that 2CdA appears to be highly effective in the treatment of hairy cell leukemia.
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PMID:Treatment of hairy cell leukemia with 2-chlorodeoxyadenosine (2-CdA). 134 77

Fifty patients undergoing splenectomy for complications of hematologic malignancy were reviewed to define indications and results. Primary diseases included lymphoma (n = 14), chronic lymphatic leukemia (n = 13), hairy-cell leukemia (n = 12), myeloid metaplasia (n = 6), and other similar disorders (n = 5). Indications for splenectomy in these patients included cytopenia (n = 37), diagnostic laparotomy (n = 8), "small stomach" syndrome (n = 3), and abdominal pain (n = 2). Splenectomy was performed by the midline approach in 32 patients. In 40 patients, the splenic artery was ligated prior to mobilization of the spleen. The spleens averaged 1650 g; in eight patients accessory spleens were removed. Additional surgical procedures included liver biopsy (n = 30), lymph node biopsy (n = 15), and cholecystectomy (n = 3). Intraoperative blood loss averaged 750 ml. In 14 patients, drainage of the left subphrenic space was used. Splenectomy was effective in 36 of 50 patients. In seven patients, splenectomy was ineffective in correction of cytopenia. Seven mortalities were from bleeding (n = 2), pulmonary embolus (n = 2), postoperative sepsis (n = 2), and progression of primary disease (n = 1). Additional complications included reoperation for bleeding (n = 3), septic complications including pneumonia (n = 14), wound infection (n = 4), and intra-abdominal abscess (n = 2). Splenectomy for the patients with hematologic malignancy is generally effective. Meticulous hemostasis, timely administration of intraoperative platelets, surgical asepsis, and aggressive pulmonary care are essential to reduce morbidity and mortality.
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PMID:Splenectomy in hematologic malignancy. 646 89

A recent Ethiopian immigrant to Israel presented with pneumococcal sepsis, massive splenomegaly and lymph-adenopathy. Investigations revealed many features of both hairy cell leukaemia (HCL) and hyperreactive malarious splenomegaly (HMS). Proguanil therapy for HMS was followed by rapid, marked decrease in spleen size, disappearance of the tartrate-resistant acid phosphatase-positive cells characteristic of HCL, and increasing eosinophilia, but unchanged lymphadenopathy.
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PMID:Massive splenomegaly responsive to proguanil and with features of hairy cell leukaemia. 777 47

In order to describe the clinical features of HIV (non-AIDS), particularly injection drug use (IDU) related HIV, in patients attending the Regional Infectious Diseases Unit in Edinburgh, a prospective review utilizing the WHO staging system of the 680 HIV positive patients, 30% of whom were women and 68% were infected via IDU, was undertaken. Despite the fact that the majority of drug users and heterosexuals enrolled asymptomatic, by 1993, 71% of the patients had developed some HIV related clinical problem. The important clinical problems observed for the cohort were; minor skin problems, minor bacterial infections, major bacterial sepsis, oral thrush, oral hairy leucoplakia, significant weight loss of > 10%, HIV related thrombocytopenia and of course AIDS. Unlike previous reports from other areas, in Edinburgh drug users were not more likely than other risk groups to develop severe bacterial disease. Differences in morbidity and mortality rates by risk group but not by gender were noted but these may well be affected by the very different enrolment pattern observed in the various risk groups. The pre-AIDS mortality rates for drug users were remarkably similar to published rates from other centres.
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PMID:Clinical features of early HIV in the Edinburgh City Hospital cohort. 873 35

We present the case of a 74-y-old HIV-negative female who suffered simultaneously from multiple opportunistic infections and a Klebsiella pneumoniae sepsis during high-dose steroids for giant cell arteritis. The patient was treated with a purine analog due to hairy cell leukaemia 10 y previously. Purine analog therapy can lead to long lasting defects in cell-mediated immunity. In these patients, treatment with steroids should be closely monitored with CD4 counts.
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PMID:Multiple opportunistic infections after high-dose steroid therapy for giant cell arteritis in a patient previously treated with a purine analog. 1700 40