Disease Subtype(s) HCT Disease Inserts Cellular Therapy Disease Inserts
Diseases of Immune Dysregulation, Familial Hemophagocytic Lymphohistiocytosis (FHL)
  • Familial Hemophagocytic Lymphohistiocytosis, Perforin deficiency (FHL2) (2511)
  • Familial Hemophagocytic Lymphohistiocytosis, UNC13D (FHL3) (2512)
  • Familial Hemophagocytic Lymphohistiocytosis, STX11 (FHL4) (2513)
  • Familial Hemophagocytic Lymphohistiocytosis, STXBP2 (FHL5) (2514)
  • Familial Hemophagocytic Lymphohistiocytosis, no mutation identified (2515)
  • Familial Hemophagocytic Lymphohistiocytosis, other mutations (2516)
Form 2039 & 2139 No disease inserts required
  • Langerhans cell histiocytosis (histiocytosis-X) (572)
Form 2040 & 2140
  • Hemophagocytosis (reactive or viral associated) (573)
  • Malignant histiocytosis (574)
  • Other histiocytic disorder (579)
  • Histiocytic disorder, not otherwise specified (570)
No disease inserts required
Diseases of Immune Dysregulation, Hemophagocytic Lymphohistiocytosis
  • Chediak-Higashi syndrome (456)
  • Griscelli syndrome type 2 (465)
  • Hermansky-Pudlak syndrome type 2 (466)
  • Other pigmentary dilution disorder (469)
Forms 2056 & 2156
Diseases of Immune Dysregulation, EBV susceptibility
  • SAP deficiency (XIAP-1) (458)
  • XIAP-2 deficiency (2420)
  • ITK deficiency (2421)
Forms 2034 & 2134
Last modified: Feb 17, 2026