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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinical and laboratory data of 184 patients with immune neutropenia were evaluated. They suffered from autoimmune neutropenia (n = 165), alloimmune neonatal neutropenia (n = 18) and from transfusion-associated lung injury (n = 1). Autoimmune neutropenia was predominantly found in patients below 3 years. Patients were usually affected by benign bacterial infections. The peripheral blood count showed normal or diminished leukocyte counts with median absolute neutrophil counts of 285 cells/microliters. Bone marrow examination revealed in 60% of the cases a hypercellular marrow with a shift to the left. In 36% the bone marrow was normal and in 4% a hypocellular marrow was found. Spontaneous remission occurred in all newborns and, so far, in 4 patients with autoimmune neutropenia. Symptomatic treatment of the infections was sufficient in most of the patients.
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PMID:[Clinical importance of granulocyte-specific antibodies]. 128 51

The differential diagnosis of unexpected neutropenia following bone marrow transplantation includes several potentially life-threatening complications including graft rejection, overwhelming infection, relapse of the underlying neoplasm, and intrinsic graft failure. However, a number of recent reports document that the differential diagnosis also includes autoimmune neutropenia, which, although potentially life-threatening, often responds well to corticosteroids or splenectomy. Autoimmune neutropenia has been reported following both autologous and allogeneic bone marrow transplantation. Herein we report a 31-year-old woman who developed a rapidly falling neutrophil count 11 days following peripheral blood stem cell transplantation for non-Hodgkin's lymphoma. A laboratory evaluation supported a diagnosis of autoimmune neutropenia, and the neutropenia resolved following treatment with steroids and granulocyte-colony stimulating factor.
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PMID:Autoimmune neutropenia following peripheral blood stem cell transplantation. 138 19

Autoimmune neutropenia is thought to be an uncommon disorder in adults. Over a 2-year period, however, autoimmune neutropenia was diagnosed in seven adults in a county with a population of approximately 105,000 people. The median age of the patients was 52 years old (range of 22 to 81 years), and five of the seven patients were women. All seven patients had at least one other symptom of autoimmune disease. Three patients had splenomegaly, three patients had positive direct antiglobulin tests, and two patients had immune thrombocytopenia. Antibodies reacting to neutrophils were detected by either granulocyte agglutination (GA) or granulocyte immunofluorescence (GIF) testing in five of the seven patients. Antibodies in four patients reacted with an 80 kd neutrophil membrane glycoprotein, and antibodies from two people reacted with a 60 kd membrane glycoprotein. Three patients were given treatment with splenectomy, which resulted in only transient improvement in the neutrophil counts. Serious infections occurred in only three patients over the 2 years of observation. In summary, autoimmune neutropenia in adults may occur more often than appreciated. Most cases of autoimmune neutropenia in adults appear to be associated with other autoimmune phenomena.
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PMID:Autoimmune neutropenia in Sheboygan County, Wisconsin. 159 17

We report two siblings with autoimmune neutropenia of infancy. Patient 1 was hospitalized for viral gastroenteritis at 5 months of age, when his absolute neutrophil count was 684/mm3. Absolute neutrophil counts ranging from 0 to 930/mm3 continued but normalized spontaneously at 25 months of age. Patient 2, a younger brother of patient 1, had prolonged high grade fever at 5 months, when his absolute neutrophil count was 138/mm3. Absolute neutrophil counts ranging from 96 to 1,078/mm3 continued for the following 9 months. Antineutrophil antibodies were detected in the patients' sera. Our cases are siblings and each case was independently affected, since the diagnoses were made at 5 months of age and the interval between the diagnoses was 23 months. Autoimmune neutropenia of infancy is comparatively rare, so that we think that the disease in these siblings was due to a genetic abnormality.
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PMID:Two siblings with autoimmune neutropenia of infancy. 180 52

Autoimmune neutropenia is a rare immunohematological disorder occurring as an idiopathic as well as a symptomatic variety associated with different diseases. Autoimmune neutropenia has previously been reported predominantly in adults, but is now found more frequently in young children. The neutropenia usually leads to recurrent mild bacterial infections. Apart from blood count and bone marrow evaluation assessment of granulocyte-specific antibodies is essential for establishing the diagnosis. As the infectious complications in these patients often are not life-threatening and neutropenia in children is usually self-limiting, therapy with antibiotics is sufficient in most patients. In more severe cases interventions with high doses of intravenous immunoglobulin or corticosteroid therapy are indicated.
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PMID:[Autoimmune neutropenia: clinical aspects, diagnosis and therapy]. 265 4

Neutrophil specific antigens (NA) are expressed exclusively on human neutrophils and were identified using alloantibodies. Neutrophil specific antigens are polymorphic, and several of them (NA1, NA2, NB1, NB2, NC1, ND1, NE1, and 9A), are thought to define genes at different loci. Feto-maternal incompatibility of NA has resulted in alloimmune neonatal neutropenia. Also, NA are the target antigens for autoantibody production in infants and young children with autoimmune neutropenia of infancy and chronic idiopathic neutropenia in adults. Autoimmune neutropenia can occur secondary to several other diseases, including AIDS. Numerous assays are useful in detecting granulocyte antibodies in patients with neutropenia. Among these assays, granulocyte agglutination (GA) and granulocyte immunofluorescence (GIF) are available in some clinical laboratories. Both IgG and IgM agglutinins are detected by GA: in addition, IgG, IgM, and IgA are detected by GIF. Immune neutropenia (IN) occurs in all age groups. Originally thought to be rare, IN is being increasingly recognized in recent years. Further investigations should lead to a greater understanding of the role of NA in immune neutropenias and to identify as yet unknown NA specificities. With the availability of reproducible and sensitive assays to detect granulocyte antibodies and the increasing knowledge and understanding of various disease aspects of IN, proper diagnosis and appropriate clinical management are being applied.
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PMID:Neutrophil antigens and antibodies in the diagnosis of immune neutropenias. 265 24

Neutrophil antibodies were demonstrated in 119 of 121 infants and young children with chronic neutropenia, establishing the diagnosis of autoimmune neutropenia of infancy. The median age at diagnosis was 8 months (range 3 to 30 months), and the female/male ratio was 6:4. Autoimmune neutropenia of infancy was manifested by recurrent fever and infection. All patients had selective neutropenia (absolute neutrophil count 0 to 500), and many had monocytosis. Fifteen of 16 patients tested failed to respond to epinephrine and hydrocortisone stimulation. Bone marrow had myeloid hyperplasia and reduced mature neutrophils. Recovery occurred in all 81 patients who passed the age of 5 years, except for one patient who is recovering at 6 1/2 years. The median age at recovery was 30 months; 95% recovered before 4 years. The estimated median duration of disease was 20 months. Neutrophil antibodies were detected early in the neutropenic phase by a combination of immunofluorescence and agglutination tests. Ten percent of these antibodies had specificity for NA1 or NA2. Ten of the 12 serum samples with a strong reaction in the flow cytometer reacted only with neutrophils. Two also reacted with an unidentified subpopulation (30%) of lymphocytes. Lymphocyte subsets were normal in 10 patients investigated, and abnormal levels of circulating immune complexes were detected in sera from 11 of 25 (44%) patients tested. Temporary remission was induced in all of eight patients who received intravenous IgG therapy. Autoimmune neutropenia of infancy is probably the most common cause of chronic neutropenia in infancy and early childhood, can be diagnosed by immunologic techniques, and requires only conservative management; spontaneous cure appears to be the rule.
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PMID:Autoimmune neutropenia of infancy. 353 97

Autoimmune neutropenia (AIN) in children can be divided into 2 forms. In primary AIN, neutropenia is the sole abnormality, and although neutrophil counts are generally below 500 microL(-1), mild bacterial infections occur. Primary AIN is mostly seen in young children and shows a self-limited course. AIN occurring in association with autoimmune diseases (secondary AIN) often shows more severe infectious complications. We analyzed clinical and serological data from 28 pediatric patients with AIN to evaluate whether there is a possible relationship between specificity of the neutrophil autoantibodies and the clinical course of the disease. Specificity of the circulating antibodies was determined with the indirect granulocyte immunofluorescence test (GIFT) and a panel of phenotyped donor neutrophils. The samples were further analyzed in the monoclonal antibody immobilization of granulocyte antigens assay (MAIGA) for neutrophil antigen (NA)1, NA2, CD11a, and CD11b specificity. With the indirect GIFT, an antibody specificity was deduced in 26 of the 28 analyzed samples. In all but 3 sera from patients with primary AIN, NA1-(76%) or NA2-(10%) specific antibodies were detected with the indirect GIFT. In 2 samples, the reactivity in the indirect GIFT was too weak to draw conclusions, but the MAIGA showed NA1 and/or NA2 specificity of the antibodies. One serum, from a patient with primary AIN with a persistent neutropenia for more than 6 years, contained NA1, possibly pan-FcgammaRIIIb, and CD11a antibodies. In 4 sera from patients with primary AIN, weak antibodies with CD11a or CD11b specificity were detected with the MAIGA. Sera from 7 patients with secondary AIN contained in all cases antibodies with pan-FcgammaRIIIb specificity, as deduced from the indirect GIFT results and absorbance/elution experiments performed with 2 sera. The MAIGA confirmed this for only 1 of the 5 tested sera. Furthermore, CD11a antibodies were detected in 1 of the 5 tested sera. In conclusion, our results indicate that primary AIN is usually associated with NA-specific antibodies, whereas secondary AIN seems to be associated with pan-FcgammaRIIIb antibodies. Thus, characterization of the antibodies in sera from children with AIN discriminates patients with primary AIN from those with secondary AIN.
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PMID:Neutrophil antibody specificity in different types of childhood autoimmune neutropenia. 1047 6

Autoimmune neutropenia (AIN) has been reported in infants and children, but not in neonates. AIN is caused by antibodies produced by the patient against their own neutrophils; therefore, it differs from the more common alloimmune neonatal neutropenia and the neonatal neutropenia because of a maternal autoimmune disease in which antineutrophil antibodies of maternal origin cross the placenta. We observed 2 cases of congenital AIN in premature neonates. These are the youngest reported cases, and indicate that AIN can have a prenatal onset. Examination of the bone marrow biopsies revealed an increase in B lymphocytes and myeloperoxidase-positive cells with a maturation arrest at the myelocyte stage. Recombinant human granulocyte colony-stimulating factor effectively treated the neutropenia, as it does in infantile AIN. Ten months after the diagnosis, 1 of the patients still requires recombinant human granulocyte colony-stimulating administration.
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PMID:Congenital autoimmune neutropenia in two premature neonates. 1143 74

Autoimmune neutropenia (AIN) can occur during pregnancy. However, neonatal neutropenia occurring in an infant born to a mother with AIN has only rarely been documented. Recently, we have experienced two cases of AIN during pregnancy, both of which caused severe yet transient neonatal neutropenia (< 0.3 x 10(9)/l), probably as a result of transplacental maternal anti-neutrophil autoantibodies. The anti-neutrophil antibodies seemed to be against antigens other than NA1/NA2 because the autoantibodies did not bind to neutrophils of specific NA types selectively in the granulocyte indirect immunofluorescence test. Although AIN is a relatively uncommon disease, neonatal neutropenia caused by maternal AIN may not be quite as rare.
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PMID:Autoimmune neutropenia in pregnant women causing neonatal neutropenia. 1147 68


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