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4A44 Vasculitis International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01 Vasculitides represent a heterogenous group of diseases of multifactorial aetiology characterised by inflammatory lesions of vessels. These lesions consist of fibrinoid necrosis (necrotizing arteritis), giant cell infiltration without necrosis, immunoglobulins deposit or leukocytoclastic infiltration. The spectrum and severity of the systemic vaculitides is broad, from life or sight threatening fulminant disease to relatively minor skin disease.
code elsewhere sections/codes in this section (4A44-4A44) postcoordination Add Stem and/or Extension codes to form a cluster code which adds detail to the condition.
Associated with - multiple selections are allowed [select] 1E51.0 Chronic hepatitis B – 1E51.00 Chronic hepatitis B with human immunodeficiency virus co-infection – 1E51.0Y Other specified chronic hepatitis B – 1E51.0Z Chronic hepatitis B, unspecified 1E51.1 Chronic hepatitis C 2A20 Non mast cell myeloproliferative neoplasms – 2A20.0 Chronic myelogenous leukaemia, BCR-ABL1-positive – 2A20.1 Chronic neutrophilic leukaemia – 2A20.2 Primary myelofibrosis – 2A20.3 Chronic eosinophilic leukaemia, not elsewhere classified – 2A20.4 Polycythaemia vera – 2A20.5 Non mast cell myeloproliferative neoplasm, unclassifiable – 2A20.Y Other specified non mast cell myeloproliferative neoplasms – 2A20.Z Non mast cell myeloproliferative neoplasms, unspecified –– 2A20.00 Chronic myelogenous leukaemia with blast crisis –– 2A20.01 Chronic myelogenous leukaemia, Philadelphia chromosome (Ph1) positive –– 2A20.02 Chronic myelogenous leukaemia, t(9:22)(q34; q11) –– 2A20.03 Naegeli-type monocytic leukaemia –– 2A20.0Y Other specified chronic myelogenous leukaemia, BCR-ABL1-positive –– 2A20.0Z Chronic myelogenous leukaemia, BCR-ABL1-positive, unspecified 2A21 Mastocytosis – 2A21.0 Systemic mastocytosis – 2A21.1 Cutaneous mastocytosis – 2A21.2 Mast cell sarcoma – 2A21.3 Extracutaneous mastocytoma – 2A21.Y Other specified mastocytosis – 2A21.Z Mastocytosis, unspecified –– 2A21.00 Mast cell leukaemia –– 2A21.0Y Other specified systemic mastocytosis –– 2A21.0Z Systemic mastocytosis, unspecified –– 2A21.10 Urticaria pigmentosa –– 2A21.1Y Other specified cutaneous mastocytosis –– 2A21.1Z Cutaneous mastocytosis, unspecified 2A22 Other and unspecified myeloproliferative neoplasms 2A30 Refractory anaemia 2A31 Refractory neutropaenia 2A32 Refractory thrombocytopenia 2A33 Refractory anaemia with ring sideroblasts 2A34 Refractory cytopenia with multi-lineage dysplasia 2A35 Refractory anaemia with excess of blasts 2A36 Myelodysplastic syndrome with isolated del(5q) 2A37 Myelodysplastic syndrome, unclassifiable 2A38 Refractory cytopenia of childhood 2A3Y Other specified myelodysplastic syndromes 2A3Z Myelodysplastic syndromes, unspecified 2A40 Chronic myelomonocytic leukaemia 2A41 Atypical chronic myeloid leukaemia, BCR-ABL1-negative 2A42 Juvenile myelomonocytic leukaemia – 2A42.0 Juvenile myelomonocytic leukaemia in complete remission – 2A42.Y Other specified juvenile myelomonocytic leukaemia – 2A42.Z Juvenile myelomonocytic leukaemia, unspecified 2A43 Refractory anaemia with ring sideroblasts associated with marked thrombocytosis 2A44 Myeloproliferative and myelodysplastic disease, unclassifiable 2A4Y Other specified myelodysplastic and myeloproliferative neoplasms 2A4Z Myelodysplastic and myeloproliferative neoplasms, unspecified 2A50 Myeloid/lymphoid neoplasm associated with PDGFRA rearrangement 2A51 Myeloid neoplasm associated with PDGFRB rearrangement 2A52 Myeloid or lymphoid neoplasms with FGFR1 abnormalities 2A5Z Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1, unspecified 2A60 Acute myeloid leukaemias and related precursor neoplasms 2A60.0 Acute myeloid leukaemia with recurrent genetic abnormalities 2A60.1 Acute myeloid leukaemia with myelodysplasia-related changes 2A60.2 Therapy-related myeloid neoplasms 2A60.3 Acute myeloid leukaemia, not elsewhere classified by criteria of other types 2A60.4 Myeloid proliferation associated with Down syndrome 2A60.5 Blastic plasmacytoid dendritic cell neoplasm 2A60.Y Other specified acute myeloid leukaemias and related precursor neoplasms 2A60.Z Acute myeloid leukaemias and related precursor neoplasms, unspecified – 2A60.20 Therapy related acute myeloid leukaemia or myelodysplastic syndrome – 2A60.2Y Other specified therapy-related myeloid neoplasms – 2A60.2Z Therapy-related myeloid neoplasms, unspecified – 2A60.30 Acute myeloid leukaemia with minimal differentiation – 2A60.31 Acute myeloid leukaemia without maturation – 2A60.32 Acute myeloid leukaemia with maturation – 2A60.33 Acute myelomonocytic leukaemia – 2A60.34 Acute monoblastic or monocytic leukaemia – 2A60.35 Acute erythroid leukaemia – 2A60.36 Acute megakaryoblastic leukaemia – 2A60.37 Acute basophilic leukaemia – 2A60.38 Acute panmyelosis with myelofibrosis – 2A60.39 Myeloid sarcoma – 2A60.3Y Other specified acute myeloid leukaemia, not elsewhere classified by criteria of other types – 2A60.3Z Acute myeloid leukaemia, unspecified – 2A60.40 Transient abnormal myelopoiesis – 2A60.41 Myeloid leukaemia associated with Down syndrome – 2A60.4Y Other specified myeloid proliferation associated with Down syndrome – 2A60.4Z Myeloid proliferation associated with Down syndrome, unspecified 2A61 Acute leukaemias of ambiguous lineage 2A70 Precursor B-lymphoblastic neoplasms – 2A70.0 B lymphoblastic leukaemia or lymphoma, not elsewhere classified – 2A70.1 B lymphoblastic leukaemia or lymphoma with t(9:22) (q34;q11.2); BCR-ABL1 – 2A70.Y Other B-lymphoblastic leukaemia/lymphoma with recurrent genetic abnormalities 2A71 Precursor T-lymphoblastic neoplasms 2A7Z Precursor lymphoid neoplasms, unspecified 2A80 Follicular lymphoma – 2A80.0 Follicular lymphoma grade 1 – 2A80.1 Follicular lymphoma grade 2 – 2A80.2 Follicular lymphoma grade 3 – 2A80.3 Primary cutaneous follicle centre lymphoma – 2A80.4 Paediatric type follicular lymphoma – 2A80.5 Follicular lymphoma in situ – 2A80.6 Follicular lymphoma of small intestine – 2A80.Y Other specified follicular lymphoma – 2A80.Z Follicular lymphoma, unspecified 2A81 Diffuse large B-cell lymphomas – 2A81.0 Primary mediastinal large B-cell lymphoma – 2A81.1 Intravascular large B-cell lymphoma – 2A81.2 Plasmablastic lymphoma – 2A81.3 Lymphomatoid granulomatosis – 2A81.4 T-cell/histiocyte rich large B-cell lymphoma – 2A81.5 Primary diffuse large B-cell lymphoma of central nervous system – 2A81.6 Epstein-Barr Virus-positive diffuse large B-cell lymphoma of the elderly – 2A81.7 Diffuse large B-cell lymphoma associated with chronic inflammation – 2A81.8 ALK-positive large B-cell lymphoma – 2A81.9 Primary effusion lymphoma – 2A81.A Primary cutaneous diffuse large B-cell lymphoma, leg type – 2A81.Y Other specified diffuse large B-cell lymphomas – 2A81.Z Diffuse large B-cell lymphoma, not otherwise specified 2A82 Mature B-cell neoplasm with leukaemic behaviour – 2A82.0 Chronic lymphocytic leukaemia or small lymphocytic lymphoma – 2A82.1 B-cell prolymphocytic leukaemia – 2A82.2 Hairy-cell leukaemia – 2A82.3 Splenic B-cell lymphoma or leukaemia, unclassifiable – 2A82.Y Other specified mature B-cell neoplasm with leukaemic behaviour – 2A82.Z Mature B-cell neoplasm with leukaemic behaviour, unspecified –– 2A82.00 Chronic lymphocytic leukaemia of B-cell type –– 2A82.0Y Other specified chronic lymphocytic leukaemia or small lymphocytic lymphoma –– 2A82.0Z Chronic lymphocytic leukaemia or small lymphocytic lymphoma, unspecified –– 2A82.10 B-cell prolymphocytic leukaemia in complete remission –– 2A82.1Y Other specified b-cell prolymphocytic leukaemia –– 2A82.1Z B-cell prolymphocytic leukaemia, unspecified 2A83 Plasma cell neoplasms – 2A83.0 Monoclonal gammopathy of undetermined significance – 2A83.1 Plasma cell myeloma – 2A83.2 Solitary plasmacytoma – 2A83.3 Extraosseous plasmacytoma – 2A83.4 Plasma cell leukaemia – 2A83.5 Monoclonal immunoglobulin deposition disease – 2A83.Y Other specified multiple myeloma and plasma cell neoplasms – 2A83.Z Plasma cell neoplasm, unspecified –– 2A83.50 Heavy chain deposition disease –– 2A83.51 Light and heavy chain deposition disease –– 2A83.52 Light chain deposition disease 2A84 Heavy chain diseases or malignant immunoproliferative diseases – 2A84.0 Alpha heavy chain disease – 2A84.1 Gamma heavy chain disease – 2A84.2 Mu heavy chain disease – 2A84.Y Other specified malignant immunoproliferative diseases – 2A84.Z Heavy chain diseases, unspecified 2A85 Other specified mature B-cell neoplasms or lymphoma – 2A85.0 Nodal marginal zone lymphoma – 2A85.1 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue of stomach – 2A85.2 Extranodal marginal zone B-cell lymphoma, primary site skin – 2A85.3 Extranodal marginal zone B-cell lymphoma, primary site excluding stomach or skin – 2A85.4 Lymphoplasmacytic lymphoma – 2A85.5 Mantle cell lymphoma – 2A85.6 Burkitt lymphoma including Burkitt leukaemia – 2A85.Y Further specified mature B-cell neoplasms or lymphoma 2A86 B-cell lymphoma, mixed features – 2A86.0 Malignant lymphoma of B cell type, not elsewhere classified – 2A86.1 B-cell lymphoma unclassifiable with features intermediate between Burkitt lymphoma and diffuse large B-cell lymphoma – 2A86.2 B-cell lymphoma unclassifiable with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma – 2A86.Y Other specified B-cell lymphoma, mixed features – 2A86.Z B-cell lymphoma, mixed features, unspecified 2A8Z Mature B-cell neoplasms, unspecified 2A90 Mature T-cell lymphoma, specified types, nodal or systemic – 2A90.0 T-cell prolymphocytic leukaemia – 2A90.1 T-cell large granular lymphocytic leukaemia – 2A90.2 Chronic lymphoproliferative disorders of NK-cells – 2A90.3 Aggressive NK cell leukaemia – 2A90.4 Systemic Epstein-Barr Virus-positive T-cell lymphoma of childhood – 2A90.5 Adult T-cell lymphoma or leukaemia, human T-cell lymphotropic virus type 1-associated – 2A90.6 Extranodal NK/T-cell lymphoma, nasal type – 2A90.7 Enteropathy associated T-cell lymphoma – 2A90.8 Hepatosplenic T-cell lymphoma – 2A90.9 Angioimmunoblastic T-cell lymphoma – 2A90.A Anaplastic large cell lymphoma, ALK-positive – 2A90.B Anaplastic large cell lymphoma, ALK-negative – 2A90.C Peripheral T-cell lymphoma, not otherwise specified – 2B00 Subcutaneous panniculitis-like T-cell lymphoma – 2B01 Mycosis fungoides – 2B02 Sézary syndrome – 2B03 Primary cutaneous CD30-positive T-cell lymphoproliferative disorders –– 2B03.0 Primary cutaneous CD30-positive anaplastic large cell lymphoma –– 2B03.1 Lymphomatoid papulosis – 2B0Y Other specified primary cutaneous mature T-cell or NK-cell lymphomas and lymphoproliferative disorders – 2B0Z Primary cutaneous T-cell lymphoma of undetermined or unspecified type 2B2Y Other specified mature T-cell or NK-cell neoplasms 2B2Z Mature T-cell or NK-cell neoplasms, unspecified 2B30 Hodgkin lymphoma 2B30.0 Nodular lymphocyte predominant Hodgkin lymphoma 2B30.1 Classical Hodgkin lymphoma 2B30.Z Hodgkin lymphoma, unspecified – 2B30.10 Nodular sclerosis classical Hodgkin lymphoma – 2B30.11 Lymphocyte-rich classical Hodgkin lymphoma – 2B30.12 Mixed cellularity classical Hodgkin lymphoma – 2B30.13 Lymphocyte depleted classical Hodgkin lymphoma – 2B30.1Z Classical Hodgkin lymphoma, unspecified 2B31 Histiocytic or dendritic cell neoplasms 2B31.0 Juvenile xanthogranuloma 2B31.1 Histiocytic sarcoma 2B31.2 Langerhans cell histiocytosis 2B31.3 Langerhans cell sarcoma 2B31.4 Interdigitating dendritic cell sarcoma 2B31.5 Follicular dendritic cell sarcoma 2B31.6 Indeterminate cell histiocytosis 2B31.7 Fibroblastic reticular cell tumour 2B31.Y Other specified histiocytic or dendritic cell neoplasms 2B31.Z Histiocytic or dendritic cell neoplasms, unspecified – 2B31.20 Langerhans cell histiocytosis involving the skin – 2B31.2Y Other specified Langerhans cell histiocytosis – 2B31.2Z Langerhans cell histiocytosis, unspecified 2B32 Immunodeficiency-associated lymphoproliferative disorders 2B32.0 Post-transplant lymphoproliferative disorder, early lesion 2B32.1 Reactive plasmacytic hyperplasia 2B32.2 Post-transplant lymphoproliferative disorder, Infectious mononucleosis-like 2B32.3 Polymorphic post-transplant lymphoproliferative disorder 2B32.Y Other specified immunodeficiency-associated lymphoproliferative disorders 2B32.Z Immunodeficiency-associated lymphoproliferative disorders, unspecified 2B33 Malignant haematopoietic neoplasms without further specification 2B33.0 Acute leukaemia, not elsewhere classified 2B33.1 Myeloid leukaemia 2B33.2 Chronic myeloid leukaemia, not elsewhere classified 2B33.3 Lymphoid leukaemia, not elsewhere classified 2B33.4 Leukaemia, unspecified 2B33.5 Malignant lymphoma, not elsewhere classified 2B33.Y Other malignant haematopoietic neoplasms without further specification 2B3Z Neoplasms of haematopoietic or lymphoid tissues, unspecified 4A40 Lupus erythematosus 4A40.0 Systemic lupus erythematosus 4A40.1 Drug-induced lupus erythematosus 4A40.Y Other specified lupus erythematosus 4A40.Z Lupus erythematosus, unspecified – 4A40.00 Systemic lupus erythematosus with skin involvement – 4A40.0Y Other specified systemic lupus erythematosus – 4A40.0Z Systemic lupus erythematosus, unspecified – EE7Y Other specified disorders of cutaneous connective tissue FA20 Rheumatoid arthritis – FA20.0 Seropositive rheumatoid arthritis – FA20.1 Seronegative rheumatoid arthritis – FA20.Z Rheumatoid arthritis, serology unspecified FA24 Juvenile idiopathic arthritis – FA24.0 Juvenile idiopathic oligoarthritis – FA24.1 Juvenile idiopathic polyarthritis – FA24.2 Juvenile psoriatic arthritis – FA24.3 Juvenile enthesitis related arthritis – FA24.4 Juvenile systemic arthritis – FA24.Y Other specified juvenile idiopathic arthritis – FA24.Z Juvenile idiopathic arthritis, unspecified –– FA24.00 Juvenile idiopathic oligoarthritis, onset persistent –– FA24.01 Juvenile idiopathic oligoarthritis, onset extended –– FA24.0Z Juvenile idiopathic oligoarthritis, onset unspecified NE60 Harmful effects of drugs, medicaments or biological substances, not elsewhere classified
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