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Autoimmune Neutropenia


Presentation

  • 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]
Lymphadenopathy
  • A 62-year-old man visited our hospital and was diagnosed with IgG4RD accompanied by interstitial pneumonitis, lymphadenopathy, and interstitial nephritis.[ncbi.nlm.nih.gov]
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]
Petechiae
  • The patient was referred to our hospital at 5 months of age because of epistaxis and generalized petechiae. Physical examination revealed moderate hepatosplenomegaly.[ncbi.nlm.nih.gov]
Epistaxis
  • The patient was referred to our hospital at 5 months of age because of epistaxis and generalized petechiae. Physical examination revealed moderate hepatosplenomegaly.[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]

Workup

  • 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]
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]

Treatment

  • 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]

Prognosis

  • […] 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]

Etiology

  • 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]

Epidemiology

  • […] 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]
Sex distribution
Age distribution

Pathophysiology

  • 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

  • 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]

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