Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hairy cell leukemia (HCL), a well-recognized chronic lymphoproliferative disorder, is frequently characterized by pancytopenia, monocytopenia, splenomegaly and marrow fibrosis, which typically leads to an unsuccessful bone marrow aspiration (dry tap). Patients with a high white cell count without neutropenia and/or monocytopenia, with an aspirable and hypercellular marrow, splenomegaly and neoplastic cells with hairy cell features have been recently recognized and classified as HCL variants. We report here the clinical, hematological and immunological features of 7 such cases. All patients presented splenomegaly with a high leukocyte count; 2 were anemic and only 1 thrombocytopenic. Five patients were treated with alpha-Interferon (alpha-IFN) but 4 failed to achieve any significant response; two of these were subsequently splenectomized and successfully treated with Chlorambucil. Splenectomy, followed by Chlorambucil, was performed at diagnosis in the remaining 2 cases, both of which achieved a partial response and are alive and well. Six out of the 7 patients are still alive. The recognition of these peculiar patients is also important because they most often do not respond to alpha-IFN, while splenectomy, followed by Chlorambucil, may be a reasonable therapeutic option for them.
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PMID:Hairy cell leukemia variant: a morphologic, immunologic and clinical study of 7 cases. 233 88

Fifty-four cases of Achromobacter xylosoxidans bacteremia diagnosed over a 10-year period in patients from 2 months to 87 years of age were reviewed. Fifty-two episodes were nosocomial. The most frequent underlying condition was neoplasm (solid or hematological). The source of infection was a contaminated intravenous catheter in 35 patients (60%) and pneumonia in 6 patients. Eight (15%) patients died. The only risk factors significantly associated with mortality were age over 65 years and neutropenia. The results of in vitro susceptibility studies of the isolates showed that antibiotic therapy with antipseudomonal penicillins or carbapenems would be a reasonable choice. An epidemiological study conducted in the hemodialysis unit showed Achromobacter xylosoxidans in tap water and on the hands of two healthcare workers but not in the hemodialysis systems. Patients were probably contaminated when healthcare workers manipulated the intravenous catheters without wearing gloves.
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PMID:Achromobacter xylosoxidans bacteremia: a 10-year analysis of 54 cases. 1275 Sep 59

Hairy cell leukaemia (HCL) is a rare, clonal, chronic lymphoproliferative disorder commonly seen in males in the middle years of life. Pancytopaenia with moderate to massive splenomegaly is the most common clinical presentation. Diagnosis is made on detecting the lymphocytes with abundant cytoplasm which spread into hair-like processes on peripheral blood and bone marrow smears, thus giving the name, "hairy cell leukaemia". The bone marrow aspirate is frequently a dry tap. The trephine biopsy has the characteristic features of a honey comb appearance and flow cytometry is typically CD103, CD25, FMC7, CD11c, gamma or kappa light chain positive with the classic B lymphocyte markers CD19, CD20, CD79a. Purine analogues followed by granulocyte-colony stimulating factor (G-CSF) to manage the febrile neutropenia is currently the treatment of choice. A 10 year disease free survival is recorded with these management strategies. Experimental use of anti CD20 and CD22 has also shown promising results in the treatment of this disease. We report four cases of HCL diagnosed in a span of two years at the Royal Hospital, Muscat, Oman.
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PMID:Hairy Cell Leukaemia in Oman: Four cases. 2174 80