1/41. Two case studies of chronic idiopathic neutropenia preceding acute myeloid leukaemia.Chronic idiopathic neutropenia is regarded as a benign disorder without risk of malignant transformation. We present two patients with chronic idiopathic neutropenia who showed disease progression to acute myeloid leukaemia. sequence analysis of the granulocyte-colony stimulating factor receptor (G-CSFR) gene from leukaemic DNA did not reveal any mutations and microsatellite analysis provided no evidence of microsatellite instability or loss of constitutional heterozygosity. These case studies suggest that chronic idiopathic neutropenia may constitute a preleukaemic condition in some patients. Alterations of the G-CSFR or defective dna mismatch repair do not appear to be involved in malignant transformation.- - - - - - - - - - ranking = 1keywords = chronic idiopathic, idiopathic (Clic here for more details about this article) |
2/41. Multiple autoimmune haemopoietic disorders and insidious clonal proliferation of large granular lymphocytes.We report a patient with clonal proliferation of CD3 8 TCRalphabeta large granular lymphocytes (LGL) presenting multiple episodes of autoimmune cytopenia, including autoimmune neutropenia, idiopathic thrombocytopenic purpura, autoimmune haemolytic anaemia, and pure red cell aplasia. Each disorder appeared separately or as a combination during an 11-year clinical course. The increase of blood CD3 8 TCRalphabeta LGL was detected 6 years after the initial diagnosis of cytopenia, but the absolute number of LGL cells was always < 1.0 x 109/l. LGL cells were of monoclonal origin and had a chromosomal abnormality. LGL cells transiently responded to cyclosporine A therapy, which was also effective on all of these autoimmune cytopenias. Accordingly, an undetectable level of proliferation of a clonal LGL population could cause various autoimmune haemopoietic disorders.- - - - - - - - - - ranking = 4.2479441326315E-5keywords = idiopathic (Clic here for more details about this article) |
3/41. A case of balamuthia mandrillaris meningoencephalitis.balamuthia mandrillaris is a newly described pathogen that causes granulomatous amebic encephalitis, an extremely rare clinical entity that usually occurs in immunosuppressed individuals. We report a case of pathologically proven Balamuthia encephalitis with unusual laboratory and radiologic findings. A 52-year-old woman with idiopathic seizures and a 2-year history of chronic neutropenia of unknown cause had a subacute illness with progressive lethargy, headaches, and coma and died 3 months after the onset of symptoms. Cerebrospinal fluid (CSF) glucose concentrations were extremely low or unmeasurable, a feature not previously described (to our knowledge). Cranial magnetic resonance imaging scans showed a single large temporal lobe nodule, followed 6 weeks later by the appearance of 18 ring-enhancing lesions in the cerebral hemispheres that disappeared after treatment with antibiotics and high-dose corticosteroids. The initial brain biopsy specimen and analysis of CSF samples did not demonstate amebae, but a second biopsy specimen and the postmortem pathologic examination showed Balamuthia trophozoites surrounded by widespread granulomatous inflammation and vasculitis. The patient's neutropenia and antibiotic use may have caused susceptibility to this organism. Amebic meningoencephalitis should be considered in cases of subacute meningoencephalitis with greatly depressed CSF glucose concentrations and multiple nodular lesions on cerebral imaging. Arch Neurol. 2000;57:1210-1212- - - - - - - - - - ranking = 4.2479441326315E-5keywords = idiopathic (Clic here for more details about this article) |
4/41. Chronic idiopathic neutropenia associated with gingival enlargement.A girl with chronic idiopathic neutropenia who developed gingival enlargement at seven years of age is presented. Intraoral examination revealed generalized gingival inflammation with a tendency to bleeding and inflammatory gingival enlargement localized to the anterior region. A considerable amount of bacterial plaque was noted on the teeth. There were also 4-5 mm pocket depths around the first molars. Radiographic examination also indicated the presence of incipient bone loss around the first molars in both jaws. The patient, who was diagnosed as localized prepubertal periodontitis with generalized gingival inflammation and anterior gingival enlargement, accentuates the importance of evaluation of periodontal status in patients with chronic idiopathic neutropenia, to avoid the destruction of supporting structures of the dentition.- - - - - - - - - - ranking = 0.33348909128486keywords = chronic idiopathic, idiopathic (Clic here for more details about this article) |
5/41. Sustained remission of platelet counts following monoclonal anti-CD20 antibody therapy in two cases of idiopathic autoimmune thrombocytopenia and neutropenia.Idiopathic autoimmune thrombocytopenia and neutropenia (ITN) is a primary haemocytopenic disorder clinically characterised by recurrent mucocutaneous bleeding episodes and infections. Unlike in simple idiopathic thrombocytopenic purpura, the platelet deficiency of ITN tends to be chronic and difficult to treat. We describe two patients with ITN who obtained sustained remission of their platelet counts after therapy with the chimeric monoclonal anti-CD20 antibody Rituximab. In one of two cases, Rituximab also induced prolonged normalisation of the neutrophil count and disappearance of auto-antibodies. Our observations indicate that disturbed B-cell function plays a central role in the pathogenesis of ITN. Anti-CD20 antibody therapy seems to constitute a safe and efficient alternative to corticosteroids for the management of ITN patients with chronic thrombocytopenia.- - - - - - - - - - ranking = 0.00021239720663158keywords = idiopathic (Clic here for more details about this article) |
6/41. Megakaryocyte emperipolesis in a child with chronic neutropenia: an unusual coexistence.The term emperipolesis defines the temporary presence of one cell within another's cytoplasm. In clinical use, megakaryocyte emperipolesis is the penetration of hematopoietic cells into the cytoplasm of megakaryocytes. The pathophysiological significance of megakaryocyte emperipolesis is uncertain. It has been described in association with neoplastic disorders, and in a few instances in idiopathic thrombocytopenic purpura, iron deficiency anemia, bleeding, and during the administration of recombinant human granulocyte colony-stimulating factor. However, megakaryocyte emperipolesis in a patient with chronic neutropenia has not been reported. In the current report, emperipolesis of hematopoietic cells within megakaryocytes in a boy with chronic neutropenia is described and the possible mechanisms are discussed.- - - - - - - - - - ranking = 4.2479441326315E-5keywords = idiopathic (Clic here for more details about this article) |
7/41. Neutrophilic dermatoses in two children with idiopathic neutropenia: association with granulocyte colony-stimulating factor (G-CSF) therapy.Painful neutrophilic skin lesions were observed in two children receiving granulocyte colony-stimulating factor (G-CSF) for treatment of idiopathic neutropenia. A girl with cystic fibrosis and cyclic neutropenia developed an erythematous papular eruption without fever or neutrophilia 7 months after commencing therapy with G-CSF. A skin biopsy specimen revealed microscopic, sterile, neutrophilic abscesses. A boy with chronic neutropenia and recurrent inflammatory skin lesions developed multiple erythematous nodules following administration of G-CSF. A biopsy specimen showed neutrophilic panniculitis. We believe that these skin eruptions belong to a spectrum of neutrophilic dermatoses that can be induced or aggravated by G-CSF therapy.- - - - - - - - - - ranking = 0.00021239720663158keywords = idiopathic (Clic here for more details about this article) |
8/41. Two patients with nonimmune chronic idiopathic neutropenia of adults developing acute myeloid leukemia with aberrant phenotype and complex karyotype but no mutations in granulocyte colony-stimulating factor receptor.It has been suggested that some cases of nonimmune chronic idiopathic neutropenia of adults (NI-CINA) may be considered preleukemic disorders. This paper describes two patients with NI-CINA who developed acute myeloid leukemia (AML) 34 and 64 months, respectively, following NI-CINA diagnosis. Patient 1 presented erythema nodosum and patient 2 polyarthritis of the large joints 9 and 2 months, respectively, before AML. Patient 1 had AML M4 disease associated with aberrant expression of CD7 and CD19 cell surface markers and one abnormal clone in bone marrow karyotype. Patient 2 had myeloid/natural killer (NK) cell leukemia with expression of CD7 and CD56 molecules and four derivative abnormal clones in the karyotype. Both patients had del(5)(q22q35) in common. No mutations in the transmembrane or the intracytoplasmic domain of the granulocyte colony-stimulating factor (G-CSF) receptor were found. The first patient had disease resistant to chemotherapy from the beginning of the treatment and the second following a brief complete hematological remission. On the basis of these observations, we concluded that a causal link of AML with the underlying NI-CINA cannot be presently justified, but the unusual findings noted in our patients prompt the description of additional cases for a further investigation of the relationships, if any, between these two granulocytic disorders.- - - - - - - - - - ranking = 0.83329793379889keywords = chronic idiopathic, idiopathic (Clic here for more details about this article) |
9/41. The association of Buschke-Ollendorf syndrome and nail-patella syndrome.Buschke-Ollendorf syndrome and nail-patella syndrome are both rare connective tissue disorders inherited in an autosomal dominant pattern and characterized by cutaneous and bone lesions. We describe a 3-year-old boy and his family who showed clinical features of both Buschke-Ollendorf syndrome and nail-patella syndrome. To our knowledge, this association has not been reported previously, suggesting that these two connective tissue disorders may share the same gene location with different mutations or involve different mutated genes that share downstream segments of their signaling pathways. Furthermore, this young patient is also affected by a chronic idiopathic neutropenia usually not observed in Buschke-Ollendorf syndrome or nail-patella syndrome.- - - - - - - - - - ranking = 0.16665958675978keywords = chronic idiopathic, idiopathic (Clic here for more details about this article) |
10/41. Granulocyte colony stimulating factor in the management of chronic neutropenia.OBJECTIVE: To report two cases of chronic neutropenic states successfully treated with granulocyte colony stimulating factor (G-CSF). CLINICAL FEATURES: A 23-year-old man with severe congenital cyclic neutropenia causing lifelong recurrent infections and consequent debilitation presented with intractable infected leg ulcers. A 56-year-old man with acquired idiopathic neutropenia presented with severe perianal infection and sepsis. INTERVENTION AND OUTCOME: Both patients were successfully treated with recombinant G-CSF. CONCLUSION: G-CSF is an agent with major clinical potential for the therapy of primary neutropenic states.- - - - - - - - - - ranking = 4.2479441326315E-5keywords = idiopathic (Clic here for more details about this article) |
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