Tesch et al reported the Immune Deficiency and Dysregulation Activity (IDDA) score to evaluate patients with LRBA deficiency. The authors are from multiple institutions from around the world, European Society for Blood and Marrow Transplantation and European Society for Immunodeficiencies.
Parameters:
(1) subscore for graded manifestations
(2) subscore for additional manifestations
(3) Karnofsky or Lansky performance status
final score =
= (150 * (subscore for graded manifestations) / (Karnofsky or Lansky)) + (subscore for additional manifestations)
Interpretation:
• minimum final score: 0
• maximum final score: 745 (moribund)
• maximum final score seen in study: 108.6
• The lower the score the better the prognosis.
• An IDDA score prior to HSCT less than 15 was associated with 100% survival.
To read more or access our algorithms and calculators, please log in or register.
