Autoimmune neutropenia

Version: 3.0
Published: 20 October 2018
Condition for which Ig use is in exceptional circumstances only

View Superseded Version  Print
Specific Conditions
  • Autoimmune neutropenia
Indication for Ig Use
  • Severe autoimmune neutropenia unresponsive to treatment with G-CSF
  • Relapse of severe autoimmune neutropenia in a patient demonstrated to have previously responded to Ig therapy
Level of Evidence Insufficient data (Category 4a)
Description and Diagnostic Criteria Autoimmune neutropenia is a rare disorder caused by peripheral destruction of autoantibody-sensitised neutrophils by the reticuloendothelial system.

While autoantibodies to neutrophil surface antigens may be present they are not specific and do not need to be demonstrated.
Justification for Evidence Category First line treatment of autoimmune neutropenia is Granulocyte colony-stimulating factor (G-CSF) and antibiotics to treat any infection. In patients with severe infection, there are multiple small case reports and case series supporting the use of Ig therapy (Bux et al, 1991 and Bux et al, 1998 and Getta et al, 2015). Corticosteroids, cytotoxic and immunosuppressant medication and rarely, splenectomy have also been described (Shastri & Logue, 1993 and Capsoni et al, 2005 and Bux et al, 1998). Although some long lasting responses have occasionally been reported following Ig therapy, the overall response is generally short lived and providing an opportunity to treat severe underlying infection.
Diagnosis Requirements

A diagnosis must be made by a Haematologist.
Qualifying Criteria for Ig Therapy
Severe autoimmune neutropenia unresponsive to treatment with G-CSF
This indication should be used for new patients and those that have never received Ig therapy for this condition. For responding patients who have relapsed following Ig therapy please use the indication: Relapse of severe autoimmune neutropenia in a patient demonstrated to have previously responded to Ig therapy.
 
  • Persistent severe autoimmune neutropenia with a neutrophil count less than 0.5 x109/L
  • AND
  • Recurrent or severe bacterial infection(s) in the last six months
  • AND
  • Failure to respond to G-CSF treatment
AND
  • Non responsive to other immunosuppressant therapy
  • OR
  • Other immunosuppressant therapy is contraindicated or has resulted in unacceptable side effects or significant toxicity
Relapse of severe autoimmune neutropenia in a patient demonstrated to have previously responded to Ig therapy
This indication should be used for responding severe autoimmune neutropenia patients who have relapsed following immunoglobulin therapy. For new patients and those that have never received Ig therapy, please use the indication: Severe autoimmune neutropenia unresponsive to treatment with G-CSF.
 
  • Persistent severe autoimmune neutropenia
AND
  • Recurrent or severe bacterial infection(s) in the last six months
AND
  • Previous response following four weeks of Ig therapy demonstrated by improvement in neutrophil count (greater than 0.5x109/L) or a reduction in infections
  • AND
  • Previous response following four weeks of Ig therapy demonstrated by a reduction in ongoing infections
Review Criteria for Assessing the Effectiveness of Ig Use
Severe autoimmune neutropenia unresponsive to treatment with G-CSF
Review is not mandated for this indication however the following criteria may be useful in assessing the effectiveness of Ig therapy.
 
  • Improvement in neutrophil count compared to the qualifying assessment
  • AND
  • Reduction in ongoing infections compared to the qualifying assessment
Relapse of severe autoimmune neutropenia in a patient demonstrated to have previously responded to Ig therapy
Review is not mandated for this indication however the following criteria may be useful in assessing the effectiveness of Ig therapy.
 
  • Improvement in neutrophil count compared to the qualifying assessment
  • AND
  • Reduction in ongoing infections compared to the qualifying assessment
Dose
Severe autoimmune neutropenia unresponsive to treatment with G-CSF
  • Dose (IVIg) - Up to 2 g/kg in a single or divided dose weekly for 4 weeks
The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Refer to the current product information sheet for further information on dose, administration and contraindications.
 Dose Calculator
Relapse of severe autoimmune neutropenia in a patient demonstrated to have previously responded to Ig therapy
  • Relapse dose (IVIg) - Up to 2 g/kg in a single or divided dose weekly for four weeks
The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

Refer to the current product information sheet for further information on dose, administration and contraindications.
 Dose Calculator
Bibliography
Anderson, D, Ali, K, Blanchette, V, et al 2007, ‘Guidelines on the use of intravenous immune globulin for hematologic conditions’, Transfusion Medicine Reviews, vol. 21, no. 2, suppl. 1, pp. S9–56.

Bux, J, Kissel, K, Nowak, K, Spengel, U & Mueller-Eckhardt, C, 1991, ‘Autoimmune neutropenia, clinical and laboratory studies in 143 patients’, Annals of Hematology, vol.63, pp. 249-52.

Bux, J, Behrens, G, Jaeger, G et al, 1998, ‘Diagnosis and clinical course of Autoimmune neutropenia in infancy', analysis of 240 cases’ , Blood, vol. 91, no. 1, pp.18

Capsoni, F, Sarzi-Puttini, P & Zanella, A 2005, ‘Primary and secondary neutropenia’ Arthritis Research & Therapy', vol. 7, no.5, pp.208-14.

Getta, B, Ponniah, G, & Ling, S 2015, ‘Intravenous immunoglobulin induces short-term reversal of drug-induced autoimmune neutropenia’, Transfusion Medicine, vol. 25, pp.347-8.

Ontario Regional Blood Coordinating Network 2016. Ontario Intravenous Immune Globulin (IVIG) Utilization Management Guidelines, Version 3.0. [online]. Available from: http://transfusionontario.org/en/.

Shastri, KA & Logue, GL 1993, ‘Autoimmune neutropenia’, Blood, vol. 81, no.8, pp.1984-95.

UK Department of Health, 2011, ‘Clinical Guidelines for Immunoglobulin Use’, Second Edition Update Available from: https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/216671/dh_131107.pdf

UK Department of Health, 2011, ‘Clinical Guidelines for Immunoglobulin Use’ Second Edition Update. Summary Poster. Available from: http://igd.mdsas.com/wp-content/uploads/2016/04/DemandManagementPoster_v4_February2016.pdf