Symptoma

Autoimmune Neutropenia

Sixteen of the 18 patients presented with mild infectious symptoms; the other 2 patients were detected by chance and presented with no infectious symptoms. D-GIFT was positive in all patients, and I-GIFT was positive in 17 of these 18 patients. [ncbi.nlm.nih.gov]

  • Recurrent Otitis Media

    otitis media (81%), viral upper respiratory tract infection (67%), oral ulcers or gingivitis (53%), tonsillitis (39%), and sinusitis (37%). [19] A case of secondary autoimmune neutropenia. [emedicine.com]

    otitis media (81%), viral upper respiratory tract infection (67%), oral ulcers or gingivitis (53%), tonsillitis (39%), and sinusitis (37%). [21] A case of secondary autoimmune neutropenia. [emedicine.medscape.com]

  • Recurrent Urinary Tract Infection

    An infant developed recurrent urinary tract infections at 9 and 11 months of age. The identified uropathogens were Escherichia coli and Enterococcus faecalis, respectively. [ncbi.nlm.nih.gov]

  • Lethargy

    Autoimmune neutropenia Other names Autoimmune neutropenia of infancy and Primary autoimmune neutropenia Pronunciation au-toim-mune neu-trope-nia Specialty Hematology Symptoms Weak immune system, mouth ulcers, sore throat, lethargy, high fever and chills [en.wikipedia.org]

If it lasts longer than that, or if the child is very ill, then a full workup for neutropenia is in order. [emedicine.medscape.com]

Workup includes a peripheral blood smear analysis for cytology that shows the presence of larger lymphocytes characterized by abundant cytoplasm containing azurophilic granules. [connect.springerpub.com]

  • Helicobacter Pylori

    Recent studies have suggested a high prevalence of Helicobacter pylori infection in patients with immune thrombocytopenia. Eradication therapy for H. pylori led to an increase in platelet count in a significant number of patients. [ncbi.nlm.nih.gov]

    Improvement of thrombocytopenia in a patient with autoimmune pancreatitis after Helicobacter pylori eradication. Intern Med 2008;47:321-2. [PMID 18277039] Caposoni F, Sarzi-Puttini P, Zanella A. Primary and secondary autoimmune neutropenia. [pancreas.imedpub.com]

    Gupta V, Eden AJ, Mills MJ: Helicobacter pylori and autoimmune neutropenia. Clin Lab Haematol. 2002, 24: 183-185. 10.1046/j.1365-2257.2002.00441.x. 29. [arthritis-research.biomedcentral.com]

  • Viral Inclusion Body

    Viral inclusion bodies were found on urinary cytopathology and tubular cells in the urine as well as in the renal parenchyma on a renal biopsy. However, all viral cultures and serology were negative or normal. [ncbi.nlm.nih.gov]

  • HLA-DR2

    Although an association between NA1-specific autoantibodies and HLA-DR2 has been found, the occurrence of AIN in only one of monozygotic twins is strong evidence against a strictly hereditary mechanism. 26 27 Recently, an association between AIN and Parvovirus [bloodjournal.org]

The most effective treatment for primary P-AIN is a granulocyte colony-stimulating factor; however, no curative treatment has been reported. [ncbi.nlm.nih.gov]

Treatment and Prognosis Symptomatic treatment with antibiotics was sufficient in greater than 90% of the infectious events. [bloodjournal.org]

[…] fever and chills Usual onset Present from Birth Duration Disappears or weakens by age three Causes Autoimmune abnormality Diagnostic method Blood tests Treatment Granulocyte colony-stimulating factors (G-CSF) Medication Corticosteroids and antibiotics Prognosis [en.wikipedia.org]

CONCLUSIONS: Detection of NAIg is useful for the diagnosis, and the observation of changes in NAIg may be helpful one by one for prediction of the prognosis. [ncbi.nlm.nih.gov]

Prognosis The prognosis in primary chronic neutropenia is excellent. The condition usually lasts only 2-3 years before spontaneous resolution, and virtually all patients recover by age 5 years. Spontaneous recovery after 6-24 months is typical. [emedicine.com]

The therapeutic management depends on the etiology. Granulocyte growth factor is the main therapeutic option, raising the question of their long-term utilization safety. [ncbi.nlm.nih.gov]

AIN has a multifactorial etiology ranging from an idiopathic primary phenomenon to secondary disorders associated with established autoimmune diseases. [insights.ovid.com]

Whether autoimmune neutropenia is commonly triggered by similar etiologies is not known. A similar age group is affected, and recovery is expected in both. Some studies have shown an association with parvovirus B19 infection. [emedicine.com]

[…] normal, even during the neutropenic period, except when patients had infections. [10] On the other hand, Kobayashi et al found that the patient’s sera contained mildly but significantly elevated concentration of G-CSF compared with the control sera. [11] Epidemiology [emedicine.com]

[…] normal, even during the neutropenic period, except when patients had infections. [12] On the other hand, Kobayashi et al found that the patient’s sera contained mildly but significantly elevated concentration of G-CSF compared with the control sera. [13] Epidemiology [emedicine.medscape.com]

Brauner A. (1993) The epidemiology of benign neutropenia in Yemenite Jews. [ncbi.nlm.nih.gov]

Our case, invoving multiple hematological abnormalities, might help deepen our understanding of the pathophysiology of IgG4RD. [ncbi.nlm.nih.gov]

Helen A Papadaki and Charalampos Pontikoglou, Pathophysiologic mechanisms, clinical features and treatment of idiopathic neutropenia, Expert Review of Hematology, 1, 2, (217), (2008). [doi.org]

Pathophysiology The mechanism of neutropenia in autoimmune neutropenia is varied and not well understood. [cancertherapyadvisor.com]

Prevention of febrile neutropenia: use of prophylactic antibiotics. Br J Cancer. 2009 Sep. 101 Suppl 1:S11-4. [Medline]. [Full Text]. Krell D, Jones AL. Impact of effective prevention and management of febrile neutropenia. [medscape.com]

She also gets mouth ulcers and sore bleeding gums so needs to use a baby tooth brush to prevent her gums from bleeding. [raredisease.org.uk]

It is important for patients to maintain good dental hygiene in order to prevent gingivitis, stomatitis, and other mucous membrane infections of the mouth. [emedicine.com]

Management requires: annual bone marrow monitoring monthly CBC tests emotional support psychological therapy No specific prevention for neutropenia is known. [healthline.com]