ITEM CRITERIA v2.1 PROPOSED REVISIONS TO THE CRITERIA SPECIALIST WORKING GROUP RATIONALE FOR ADDITIONS/CLARIFICATIONS Unchanged Condition Autoimmune neutropenia Autoimmune neutropenia Name Specialty Haematology Haematology Unchanged Category Exceptional circumstances Exceptional circumstances only Unchanged only Autoimmune neutropenia Specific Specific condition must be defined Conditions Level of Insufficient data (Category 4a) Insufficient data (Category 4a) Unchanged Evidence Justification First line treatment of Autoimmune neutropenia is G-CSF This section has been drafted in line with the for Evidence and antibiotics to treat any infection. In patients with severe literature Category 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. Severe autoimmune neutropenia unresponsive to Indications Two indications have been developed in order treatment with G-CSF to limit any subsequent treatment to responding patients only.
ITEM CRITERIA v2.1 PROPOSED REVISIONS TO THE CRITERIA SPECIALIST WORKING GROUP RATIONALE FOR ADDITIONS/CLARIFICATIONS Condition Name Autoimmune neutropenia Autoimmune neutropenia Unchanged Specialty Haematology Haematology Unchanged Category Exceptional circumstances only Exceptional circumstances only Unchanged Specific Conditions Autoimmune neutropenia Specific condition must be defined Level of Evidence Insufficient data (Category 4a) Insufficient data (Category 4a) Unchanged Justification for Evidence Category First line treatment of Autoimmune neutropenia is 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. This section has been drafted in line with the literature Indications Severe autoimmune neutropenia unresponsive to treatment with G-CSF Two indications have been developed in order to limit any subsequent treatment to responding patients only.
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