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)

The high frequency of pulmonary complications of haematological malignancy and the increasing number of patients treated for these disorders make it important that the respiratory physician has a structured diagnostic approach according to: 1 the immune deficiency due to the malignancy and/or the treatment administered; 2 the factors that can modify the risk of infection (anti infection prophylaxis and/or pre-emptive treatment); 3 co-morbidities; 4 extra-pulmonary manifestations. Two main situations can be identified: The patient is aplasic: Initially the pneumonias are predominantly of bacterial origin but may be fungal if the neutropenia is prolonged. The respiratory physician is faced with two problems: 1 the diagnosis of pneumonia; this may be helped by CT scanning; 2 The choice of antibiotics; this will depend on previous investigations. The patient is not aplasic: The lung disease may have many causes, mainly infectious but also drug related, tumoral, haemorrhagic or embolic. The main problem is the correct choice of investigations to establish an aetiological diagnosis. The collection of data according to a pre-established protocol based on simple factors (study of the notes and clinical examination) is one of the key elements for improving the prognosis of these patients whose management should be multidisciplinary following a pre-defined plan.
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PMID:[Management of the pulmonary complications of haematological malignancy]. 1734 2

Autoimmune cytopenias are well recognised in patients with primary immune deficiency, but treatment may be difficult. We report eight children with autoimmune cytopenias (autoimmune haemolytic anaemia, autoimmune thrombocytopenia, autoimmune neutropenia) complicating immune deficiency states (common variable immunodeficiency, Wiskott-Aldrich Syndrome, autoimmune lymphoproliferative syndrome, combined immunodeficiency) treated with between 1 and 3 courses of rituximab (anti-CD20). Responses occurred for 90% of treatments but relapse rates (after a median of 53 weeks) were high (78%). We conclude that rituximab is an effective treatment for autoimmune cytopenias in children with immune deficiencies, but repeated courses of treatment may be needed.
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PMID:Rituximab for the treatment of autoimmune cytopenias in children with immune deficiency. 1749 97

In our study, we tried to detect gastroenteritis causing intestinal protozoa in patients with immune deficiency and who suffered from diarrhea. We also tried to determine which laboratory methods should be used in detecting intestinal protozoon in these patients. Thirty-six immune deficient patients who had had diarrhea for more than 10 days and 44 immune deficient patients without diarrhea were included in the study. In stool samples taken from all cases, intestinal protozoa were detected using the conventional diagnostic methods including direct wet mount, trichrome and modified acid fast staining as well as serologic diagnostic methods such as ELISA, direct fluorescent antibody (DFA)] and the molecular method of polymerized chain reaction. In our study, we found that intestinal protozoan such as G. intestinalis; C. parvum, B. hominis and E. histolytica could be responsible for the long term diarrhea in patients with immune deficiency. If a pathogen is not detected in the feces by native Lugol (NL), DFA and MAF are suitable techniques for Cryptosporidium spp while ELISA or trichrome staining are suitable methods for E. histolytica. It was concluded in the study that the simple and inexpensive NL method is sufficient in the diagnosis of G. intestinalis and serological or molecular methods are unnecessary. Neutropenia in patients with immune deficiency did not enhance the frequent occurrence of intestinal protozoan infections; and also, in the cases with immune deficiency, it was found that the administration of steroid treatment was not a risk factor in intestinal protozoan disease.
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PMID:[Diagnosis of intestinal-protozoa in patients with immune deficiency]. 1915 5

Genetic deficiency of adenosine deaminase (ADA) can cause profound lymphopenia and result in the clinical presentation of severe combined immune deficiency (SCID). However, because of the ubiquitous expression of ADA, ADA-deficient patients often present also with nonimmunologic clinical problems, affecting the skeletal, central nervous, endocrine, and gastrointestinal systems. We now report that myeloid dysplasia features and bone marrow hypocellularity are often found in patients with ADA-SCID. As a clinical correlate to this finding, we have observed vulnerability to antibiotic-induced myelotoxicity and prolonged neutropenia after nonmyeloablative chemotherapy. We have also noted that, in the absence of enzyme replacement therapy, absolute neutrophil counts of patients with ADA deficiency vary inversely with the accumulation of deoxynucleotides. These data have significant implications for the application of standard and investigational therapies to patients with ADA-SCID and support further studies to investigate the possibility that ADA deficiency is associated with a stem cell defect. These trials were registered at www.clinicaltrials.gov as #NCT00018018 and #NCT00006319.
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PMID:Myeloid dysplasia and bone marrow hypocellularity in adenosine deaminase-deficient severe combined immune deficiency. 2172 47

Ecthyma gangrenosum is a skin manifestation of systemic sepsis commonly caused by Pseudomonas aeruginosa in patients with neutropenia or underlying immune deficiency. Although the usual outcome is poor, early recognition and appropriate systemic antibiotic treatment can lead to successful outcome. We report a case of a previously healthy lady with no apparent immune deficiency or neutropenia who had ecthyma gangrenosum of left lower limb in which the arterial line was placed.
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PMID:Ecthyma gangrenosum of a single limb. 2201 15

Primary immune thrombocytopenia (ITP) in childhood, typically presents as an acute self-limiting illness. However, secondary ITP is often a chronic disorder due to an underlying disease. Combined cytopenias in childhood, that is, secondary ITP occurring with auto-immune hemolytic anemia and/or auto-immune neutropenia, are often associated with disorders characterized by immune dysregulation. Such disorders include systemic lupus erythematosus, auto-immune lymphoproliferative syndrome, and common variable immune deficiency. Evans syndrome describes the combination of ITP, autoimmune hemolytic anemia, and/or autoimmune neutropenia. However, it is now clear that some patients with Evans syndrome have an underlying immunodeficiency. This report focuses on combined auto-immune cytopenias and highlights the challenges in their diagnosis and management.
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PMID:Auto-immune lymphoproliferative disorder and other secondary immune thrombocytopenias in childhood. 2310 1

Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive disease characterized by eczema, thrombocytopenia and immune deficiency. WAS gene mutations impair WAS protein function which cause WAS. The WAS-related disorders of X-linked thrombocytopenia (XLT) and X-linked congenital neutropenia (XLN) may have similar but less severe symptoms those are also caused by mutations of the same gene. We present two cases of WAS in neonates with WAS gene mutations. Early genetic diagnosis can help to the treatment and prevention this disease.
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PMID:Two cases of Wiskott-Aldrich syndrome in neonates due to gene mutations. 2330 16

The management of infections in haematology is dictated by the patient's type of acquired or induced immune deficiency (neutropenia, deficiency in cell-mediated or antibody-mediated immunity), and findings from clinical examination, laboratory studies, or morphologic investigations. The CT scan dominates in the initial management and follow-up of these patients, since clinical features very often appear to be non-specific. The radiologist's role is to guide the clinician towards a specific diagnosis such as aspergillosis or pneumocystosis, or to point them towards a non-infectious cause: tumour localisation, hypervolaemia, bronchiolitis obliterans suggestive of GVH disease, drug toxicity, or embolism.
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PMID:Infectious chest complications in haematological malignancies. 2331 63

A 15-year-old girl with combined immune deficiency syndrome, diagnosed with metastatic squamous cell cancer of the anus, had significant pain secondary to vulvar-perianal condyloma. Conventional treatment with oral and intravenous analgesics was limited by significant side effects of mental status changes and urinary retention leading to clinical deterioration that precluded attempts at chemotherapy. An intrathecal pump was implanted in the challenging setting of neutropenia. There was a drastic improvement in her quality of life and the ability to tolerate further chemotherapy. The option of an intrathecal pump for pain control extended our patient's ability to enjoy important quality time with family by several months.
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PMID:The use of an intrathecal pump to manage intractable cancer pain in a pediatric patient: a case report. 2365 66

Fever of undetermined origin always poses a challenging problem to the physician. Tuberculosis is an important health problem in developing countries. It is mostly seen in immune-compromised patients. And it is one of the common causes of fever of unknown origin. I am reporting a case of a splenic tuberculosis in 48 years old male who is not known of any immune deficiency state, he was presented with 3 weeks history of fever and found to have severe neutropenia and with negative work up for all hematological, rheumatological and malignant causes. A computerized tomography scan of the abdomen confirmed splenic enlargement with multiples hypo dense lesions consist with either splenic infection or splenic lymphoma so exploratory splenectomy was performed. Histological examination revealed granulomatouse inflammation with numerous acid fast bacilli consist with tuberculosis. He was started on four anti-tuberculouse drugs. in less than one week his fever subside with normalization of his neutrophilic count.
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PMID:Splenic tuberculosis presenting as fever of unknown origin with severe neutropenia. 2377 75


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