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Autoimmune Neutropenia (autoimmune + neutropenia)

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Medical Sciences100%

Selected Abstracts

Pyoderma Gangrenosum in Association with Autoimmune Neutropenia of Infancy

PEDIATRIC DERMATOLOGY, Issue 6 2008
Anisha J. Mehta M.R.C.P.
Histology showed changes consistent with pyoderma gangrenosum and the ulcer resolved rapidly with super-potent topical steroids under occlusion. Blood tests revealed a persistent neutropenia. Immunoglobulin G (IgG) antineutrophil antibodies were detected in the serum, directed against human neutrophil antigen (HNA)-1a. Bone marrow studies showed normocellular marrow with no evidence of dysplasia. T and B cell subsets and karotype analysis were normal. Autoimmune neutropenia is an uncommon self-limiting condition in young children. Pyoderma gangrenosum is rare in infants, although the buttocks are a common site of involvement in this age group. Pyoderma gangrenosum in infancy can be associated with systemic disease as in adults, particularly myelodysplasia and leukemia, arthritis and inflammatory bowel disease. However, the association of pyoderma gangrenosum and autoimmune neutropenia of infancy has not previously been reported. [source]

Cd8+/v,5.1+ large granular lymphocyte leukemia associated with autoimmune cytopenias, rheumatoid arthritis and vascular mammary skin lesions: successful response to 2-deoxycoformycin.

HEMATOLOGICAL ONCOLOGY, Issue 2 2002
E. Granjo
Abstract We report a case of CD8+/V,5.1+ T-cell large granular lymphocyte leukemia (T-LGL leukemia) presenting with mild lymphocytosis, severe autoimmune neutropenia, thrombocytopenia, polyarthritis and recurrent infections with a chronic disease course. Immunophenotyping showed an expansion of CD3+/TCR,,+/CD8+bright/CD11c+/CD57,/CD56, large granular lymphocytes with expression of the TCR-V,5.1 family. Southern blot analysis revealed a clonal rearrangement of the TCR ,-chain gene. Hematopoietic growth factors, high dose intravenous immunoglobulin and corticosteroids were of limited therapeutic benefit to correct the cytopenias. During the disease course, the patient developed a severe cutaneous leg ulcer and bilateral vascular mammary skin lesions. Treatment with 2-deoxycoformycin resulted in both clinical and hematological complete responses, including the resolution of vascular skin lesions. Combined immuno-staining with relevant T-cell associated and anti-TCR-V, monoclonal antibodies proved to be a sensitive method to assess the therapeutic effect of 2-deoxycoformicin and to evaluate the residual disease. Copyright © 2002 John Wiley & Sons, Ltd. [source]

Characterization of the bone marrow immunofluorescence test in childhood autoimmune neutropenia

INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 5 2009
S. W. LANE
Summary The bone marrow immunofluorescenece test (BMIFT) demonstrates autoantibodies to granulocytes and their precursors on fresh-frozen bone marrow slides. It may be used to differentiate childhood autoimmune neutropenia (AIN) from other causes of childhood neutropenia, even when circulating neutrophil counts are low. We sought to characterize the diagnostic utility of the BMIFT in childhood AIN. All BMIFT requests for investigation of children with neutropenia between January 1998 and May 2007 were reviewed. Patients were classified as AIN or nonautoimmune causes. Baseline demographic data, results of BMIFT, granulocyte immunofluorescence testing and bone marrow findings were collected from clinical records and the institutional laboratory database. Seventy-six children had BMIFT performed for investigation of neutropenia. There were 45 patients diagnosed with AIN, 28 with nonimmune neutropenia and three failed tests. The median age of children with AIN was 1.2 years (range 0.3,15.3), compared with 3.6 years (range 0.1,15.7) in the nonautoimmune group. The median neutrophil count in AIN was 0.3 × 109/l (0.9 × 109/l in nonautoimmune). BMIFT was positive in 24 of 45 patients with AIN and 0 of 28 with nonautoimmune neutropenia (sensitivity 53%, specificity 100%, positive predictive value (PPV) 100%, negative predictive value 57%). Ten patients had other autoimmune diatheses at diagnosis. The BMIFT is a simple, highly specific test with excellent PPV and thus is a clinically useful test to confirm AIN in children. [source]

Inverse relation between plasma G-CSF levels and neutrophil counts in a patient with autoimmune neutropenia treated with G-CSF

INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 2 2000
L.tH. Vlasveld
Summary The pharmacokinetic characteristics of granulocyte colony-stimulating factor (G-CSF) appears to be related to the neutrophil count. We report the case of an 81-year-old male with acquired antibody induced neutropenia treated with G-CSF. This produced a rapid increase in the neutrophil count which appeared to be associated with diminished trough plasma G-CSF levels. Our data appears to indicate that mature neutrophils may play a part in the clearance of G-CSF from plasma. [source]

Pyoderma Gangrenosum in Association with Autoimmune Neutropenia of Infancy

PEDIATRIC DERMATOLOGY, Issue 6 2008
Anisha J. Mehta M.R.C.P.
Histology showed changes consistent with pyoderma gangrenosum and the ulcer resolved rapidly with super-potent topical steroids under occlusion. Blood tests revealed a persistent neutropenia. Immunoglobulin G (IgG) antineutrophil antibodies were detected in the serum, directed against human neutrophil antigen (HNA)-1a. Bone marrow studies showed normocellular marrow with no evidence of dysplasia. T and B cell subsets and karotype analysis were normal. Autoimmune neutropenia is an uncommon self-limiting condition in young children. Pyoderma gangrenosum is rare in infants, although the buttocks are a common site of involvement in this age group. Pyoderma gangrenosum in infancy can be associated with systemic disease as in adults, particularly myelodysplasia and leukemia, arthritis and inflammatory bowel disease. However, the association of pyoderma gangrenosum and autoimmune neutropenia of infancy has not previously been reported. [source]

The effect of treatment with Campath-1H in patients with autoimmune cytopenias

BRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2001
F. Willis
We describe 21 patients with severe and life-threatening autoimmune cytopenias resistant to standard immunosuppression who were treated with the monoclonal antibody Campath-1H. Four patients had autoimmune neutropenia, four had autoimmune haemolytic anaemia, four had pure red cell aplasia, one had immune thrombocytopenia purpura (ITP), three had autoimmune haemolytic anaemia and ITP (Evan's syndrome), three had autoimmune pancytopenia (ITP, autoimmune neutropenia and autoimmune haemolytic anaemia), one had ITP (associated with acquired Glanzmann's disease) and autoimmune neutropenia, and one had ITP and red cell aplasia. Campath-1H was administered at a dose of 10 mg/d as an intravenous infusion for 10 d. Responses were seen in 15 patients, which were sustained in six. Relapse occurred in eight patients after Campath-1H treatment. Patients entering the study later, received cyclosporine after Campath-1H in an attempt to reduce the incidence of relapse. Three patients received a second course of Campath-1H; all responded but later relapsed. Fourteen patients are alive at a median of 12 months (range 4,61) after Campath-1H. Campath-1H represents an alternative therapeutic option for severe, refractory autoimmune cytopenias. [source]

Immune-mediated neutropenia in the neonate

ACTA PAEDIATRICA, Issue 2002
A Maheshwari
Alloimmune neonatal neutropenia, neonatal autoimmune neutropenia and autoimmune neutropenia of infancy have remained nebulous entities with difficulties in both clinical and laboratory identification. These disorders are reviewed in this article. [source]