|  D89 |  | Other disorders involving the immune mechanism, not elsewhere classified   | 
	
		|  |  | 
	
		| Excludes1 | hyperglobulinemia NOS (R77.1) monoclonal gammopathy (of undetermined significance) (D47.2)
 
 |  
		| Excludes2 | transplant failure and rejection (T86.-) 
 |  | 
|  |  | 
	
		|  D89.0 |  | Polyclonal hypergammaglobulinemia |  
		|  |  | 
| Benign hypergammaglobulinemic purpura |  | Polyclonal gammopathy NOS |  |  | 
|  |  | 
	
		|  D89.1 |  | Cryoglobulinemia |  
		|  |  | 
| Cryoglobulinemic purpura |  | Cryoglobulinemic vasculitis |  | Essential cryoglobulinemia |  | Idiopathic cryoglobulinemia |  | Mixed cryoglobulinemia |  | Primary cryoglobulinemia |  | Secondary cryoglobulinemia |  |  | 
|  |  | 
	
		|  D89.2 |  | Hypergammaglobulinemia, unspecified |  | 
|  |  | 
	
		|  D89.3 |  | Immune reconstitution syndrome |  
		|  |  | 
| Immune reconstitution inflammatory syndrome [IRIS] |  
		| UseAdditionalCode | code for adverse effect, if applicable, to identify drug (T36-T50 with fifth or sixth character 5) 
 |  |  | 
|  |  | 
	
		|  D89.4 |  | Mast cell activation syndrome and related disorders |  
		|  |  | 
	
		| Excludes1 | aggressive systemic mastocytosis (C96.21) congenital cutaneous mastocytosis (Q82.2)
 (non-congenital) cutaneous mastocytosis (D47.01)
 (indolent) systemic mastocytosis (D47.02)
 malignant mast cell neoplasm (C96.2-)
 malignant mastocytoma (C96.29)
 mast cell leukemia (C94.3-)
 mast cell sarcoma (C96.22)
 mastocytoma NOS (D47.09)
 other mast cell neoplasms of uncertain behavior (D47.09)
 systemic mastocytosis associated with a clonal hematologic non-mast cell lineage disease (SM-AHNMD) (D47.02)
 
 |  |  |  |  | 
	
		|  D89.40 |  | Mast cell activation, unspecified |  
		|  |  | 
| Mast cell activation disorder, unspecified |  | Mast cell activation syndrome, NOS |  |  |  |  |  | 
	
		|  D89.41 |  | Monoclonal mast cell activation syndrome |  |  |  |  | 
	
		|  D89.42 |  | Idiopathic mast cell activation syndrome |  |  |  |  | 
	
		|  D89.43 |  | Secondary mast cell activation |  
		|  |  | 
| Secondary mast cell activation syndrome |  
		| CodeAlso | underlying etiology, if known 
 |  |  |  |  |  | 
	
		|  D89.44 |  | Hereditary alpha tryptasemia |  
		|  |  | 
	
		| UseAdditionalCode | code, if applicable, for: allergy status, other than to drugs and biological substances (Z91.0-)
 personal history of anaphylaxis (Z87.892)
 
 |  |  |  |  |  | 
	
		|  D89.49 |  | Other mast cell activation disorder |  
		|  |  | 
| Other mast cell activation syndrome |  |  |  | 
|  |  | 
	
		|  D89.8 |  | Other specified disorders involving the immune mechanism, not elsewhere classified |  |  |  | 
	
		|  D89.81 |  | Graft-versus-host disease |  
		|  |  | 
	
		| CodeFirst | underlying cause, such as: complications of transplanted organs and tissue (T86.-)
 complications of blood transfusion (T80.89)
 
 |  
		| UseAdditionalCode | code to identify associated manifestations, such as: desquamative dermatitis (L30.8)
 diarrhea (R19.7)
 elevated bilirubin (R17)
 hair loss (L65.9)
 
 |  |  |  |  | 
	
		|  D89.810 |  | Acute graft-versus-host disease |  
		|  |  |  |  |  |  |  | 
	
		|  D89.811 |  | Chronic graft-versus-host disease |  
		|  |  |  |  |  |  |  | 
	
		|  D89.812 |  | Acute on chronic graft-versus-host disease |  
		|  |  |  |  |  |  |  | 
	
		|  D89.813 |  | Graft-versus-host disease, unspecified |  
		|  |  |  |  |  |  |  |  | 
	
		|  D89.82 |  | Autoimmune lymphoproliferative syndrome [ALPS] |  |  |  |  | 
	
		|  D89.83 |  | Cytokine release syndrome |  
		|  |  | 
	
		| CodeFirst | underlying cause, such as: complications following infusion, transfusion and therapeutic injection (T80.89-)
 complications of transplanted organs and tissue (T86.-)
 
 |  
		| UseAdditionalCode | code for adverse effect, if applicable, to identify immune checkpoint inhibitors and immunostimulant drugs (T45.AX5) 
 |  
		| UseAdditionalCode | code to identify associated manifestations 
 |  |  |  |  | 
	
		|  D89.831 |  | Cytokine release syndrome, grade 1 |  |  |  |  | 
	
		|  D89.832 |  | Cytokine release syndrome, grade 2 |  |  |  |  | 
	
		|  D89.833 |  | Cytokine release syndrome, grade 3 |  |  |  |  | 
	
		|  D89.834 |  | Cytokine release syndrome, grade 4 |  |  |  |  | 
	
		|  D89.835 |  | Cytokine release syndrome, grade 5 |  |  |  |  | 
	
		|  D89.839 |  | Cytokine release syndrome, grade unspecified |  |  |  |  |  | 
	
		|  D89.84 |  | IgG4-related disease |  
		|  |  | 
| Immunoglobulin G4-related disease |  |  |  |  |  | 
	
		|  D89.89 |  | Other specified disorders involving the immune mechanism, not elsewhere classified |  
		|  |  | 
	
		| Excludes1 | human immunodeficiency virus disease (B20) 
 |  |  |  | 
|  |  | 
	
		|  D89.9 |  | Disorder involving the immune mechanism, unspecified |  
		|  |  |  |  |