LEUKEMIA/MDS Leukemia, LYMPHOID 0 - Acute lymphoblastic leukemia 1- Chronic lymphocytic leukemia of B-cell type 3 - Prolymphocytic leukemia of B-cell type 4 - Hairy cell leukemia 5 - Adult T-cell lymphoma/leukemia (HTLV-1-associated) 6 - Prolymphocytic leukemia of T-cell type A - Mature B-cell leukemia Burkitt-type Z - Other lymphoid leukemia Leukemia, MYELOID 0 - Acute myeloblastic leukemia 1 - Chronic myeloid leukemia, BCR/ABLpositive 2 - Atypical chronic myeloid leukemia, BCR/ABL-negative 3 - Myeloid sarcoma 4 - Acute promyelocytic leukemia 5 - Acute myelomonocytic leukemia 6 -Acute myeloid leukemia with 11q23- abnormality A - Acute myeloid leukemia with multilineage dysplasia Z - Other myeloid leukemia C91. 0 - Not having achieved remission 1 - In remission 2 - In relapse C92. 0 -Not having achieved remission 1 - In remission 2 - In relapse Other Leukemias of SPECIFIED CELL TYPE 0 - Acute erythroid leukemia 2 - Acute megakaryoblastic leukemia 3 - Mast cell leukemia 4 - Acute panmyelosis with myelofibrosis leukemias 6 - Myelodysplastic disease, not classified Leukemia of UNSPECIFIED CELL TYPE 0 - Acute 1 - Chronic Un Malignant Neoplasms of LYMPHOID, HEMATO POIETIC & RELATED TISSUE 0 - Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis 2 - Malignant mast cell tumor 4 - Sarcoma of dendritic cells (accessory cells) 5 - Multifocal and unisystemic Langerhans-cell histiocytosis 6 - Unifocal Langerhans-cell histiocytosis A - Histiocytic sarcoma Z - Other C94. 0 - Not having achieved remission 1 - In remission 2 - In relapse C95. 0 - Not having achieved remission 1- In remission 2 In relapse C96. Leukemia, MONOCYTIC C93. 0 - Acute monoblastic/monocytic leukemia 1 - Chronic myelomonocytic leukemia 3 - Juvenile myelomonocytic leukemia Z - Other monocytic leukemia Poisoning by Adverse Effect of & Underdosing of, 0 - Not having achieved remission 1- In remission 2 In relapse ANTINEOPLASTIC & IMMUNOSUPPRESSIVE DRUGS 1- Poisoning by antineoplastic and immunosuppressive drugs, accidental (unintentional) 5- Adverse effect of antineoplastic & immunosuppressive drugs T45.1X A - initial encounter D - subsequent encounter S - sequela MYELODYSPLASTIC SYNDROME (MDS) 2 - Refractory anemia with excess of blasts 0 - Not having achieved remission 1-1 2 2 Use T45.1X code for adverse effect, if applicable D46. 0 - Refractory anemia without ring sideroblasts, so stated 1 - Refractory anemia with ring sideroblasts A - Refractory cytopenia with multilineage dysplasia B - Refractory cytopenia with multilineage dysplasia and ring sideroblasts C - Myelodysplastic syndrome with isolated del(5q) chromosomal abnormality 4 - Refractory anemia, un Z - Other myelodysplastic syndromes Multiple Myeloma & Malignant PLASMA CELL NEOPLASM 0 - Multiple Myeloma 1 - Plasma cell leukemia 2 - Extramedullary plasmacytoma 3 - Solitary plasmacytoma C90. 0 - Not having achieved remission 1 - In remission 2 In relapse HEMATOLOGY 1
B-CELL LYMPHOMA Lymphoma, HODGKIN 0 - Nodular lymphocyte predominant 1 - Nodular sclerosis classical 2 - Mixed cellularity classical 3 - Lymphocyte-depleted classical 4 - Lymphocyte-rich classical 7 - Other classical C81. 0 - Un site 1 - Head, neck, face 2 - Intrathoracic 3 - Intra-abdominal 4 - Axilla and upper limb 5 - Inguinal region and lower limb 6 -Intrapelvic 7 -Spleen 8 - Multiple-sites 9 - Extranodal and solid organ site Lymphoma, OTHER NON-HODGKIN 1 - Un B-cell lymphoma 2 - Mediastinal (thymic) large B-cell types of non-hodgkin s C85. 0 - Un site 1 - Head, neck, face 2 - Intrathoracic 3 - Intra-abdominal 4 - Axilla and upper limb 5 - Inguinal region and lower limb 6 -Intrapelvic 7 -Spleen 8 - Multiple-sites 9 - Extranodal and solid organ site Lymphoma, FOLLICULAR 0 - Grade I 1 - Grade II 2 - Grade III un 3 - Grade IIIa 4 - Grade IIIb 5 - Diffuse follicle center lymphoma 6 - Cutaneous follicle center lymphoma types lymphoma C82. Lymphomas, MALIGNANT 0 - Un site 1 - Head, neck, face 2 - Intrathoracic 3 - Intra-abdominal 4 - Axilla and upper limb 5 - Inguinal region and lower limb 6 -Intrapelvic 7 -Spleen 8 - Multiple-sites 9 - Extranodal and solid organ site IMMUNOPROLIFERATIVE & OTHER B-CELLS 0 - Waldenstrom macroglobulinemia 2 - Heavy chain disease 3 - Immunoproliferative small intestinal disease 4 - Extranodal marginal zone B-cell lymphoma of mucosaassociated lymphoid tissue [MALT-lymphoma] malignant immunoproliferative diseases. C88. Lymphoma, NON-FOLLICULAR 0 - Small cell B-cell Lymphoma 1 - Mantle cell Lymphoma 3 - Diffuse Large B-cell lymphoma 5 - Lymphoblastic (diffuse lymphoma) 7 - Burkitt lymphoma non-follicular Lymphoma (includes PEL/ intravascular) C83. 0 - Un site 1 - Head, neck, face 2 - Intrathoracic 3 - Intra-abdominal 4 - Axilla and upper limb 5 - Inguinal region and lower limb 6 -Intrapelvic 7 -Spleen 8 - Multiple-sites 9 - Extranodal and solid organ site Lymphoma, Other SPECIFIED T/NK-CELL C86. 0 - Extranodal NK/T-cell lymphoma, nasal type 1 - Hepatosplenic T-cell lymphoma 2 - Enteropathy-type (intestinal) T-cell lymphoma 3 - Subcutaneous panniculitis-like-t-cell lymphoma 4 - Blastic NK-cell lymphoma 5 - Angiommunoblastic T-cell lymphoma 6 - Primary cutaneous CD-30-positive T-cell proliferations HEMATOLOGY 2
MYELOPROLIF/MGUS Neoplasms of Uncertain Behavior of LYMPHOID, HEMA- TOPOIETIC & RELATED TISSUE 0 - Histiocytic and mast cell tumors of uncertain behavior 1 - Chronic myeloproliferative disease 2 - Monoclonal gammopathy 3 - Essential (hemorrhagic) thrombocythemia 4 - Osteomyelofibrosis Z - Other neoplasms For MYELOFIBROSIS, use D75.81 1- Post-transplant lymphoproliferative disorder (PTLD) neoplasms ESSENTIAL (HEMORRHAGIC) THROMBOCYTHEMIA D47. D47.3 MYELOFIBROSIS D75.81 POLYCYTHEMIA VERA MONOCLONAL GAMMOPATHY Other & Un Diseases of BLOOD & BLOOD- FORMING ORGANS (MYELOFIBROSIS) 0 - Familial erythrocytosis 1 - Secondary polycythemia diseases of blood and bloodforming organs D75. D45 D47.2 1- MYELOFIBROSIS (Code first underlying disorder such as neoplasm. If applicable, code for associated therapy-related MDS and for adverse effects to identify drug) 2 - Heparin induced thrombocytopenia (HIT) diseases of blood and blood-forming organs NUTRITIONAL ANEMIAS Anemia, IRON DEFICIENCY D50. 0 - Secondary to blood loss (chronic) 1 - Sideropenic dysphagia iron deficiency anemias Anemia, VITAMIN B12 DEFICIENCY 0 - Intrinsic factor deficiency 1 - Selective vitamin B12 malabsorption with proteinuria 2 - Transcobalamin II deficiency 3 - Other dietary Vitamin B12 deficiency D51. Anemia, FOLATE DEFICIENCY 0 Dietary 1 - Drug-induced (Use additional code for adverse effects, if applicable, to identify drug) Anemias, OTHER NUTRITIONAL 0 - Protein deficiency anemia 1 - Other megaloblastic anemias, not elsewhere classified 2 - Scorbutic anemia IRON OVERLOAD & OTHERS 0 - Disorder of Iron Metabolism, un D52. D53. E83.1 1 - Hemochromatosis 0 - Hereditary 1 - Due to repeated red blood cell transfusions HEMATOLOGY
OTHER ANEMIAS Anemia due to ENZYME DISORDERS D55. 0 - Due to glucose-6-phosphate dehydrogenase [G6PD] deficiency 1 - Other disorders of glutathione metabolism 2 - Disorders of glycolytic enzymes 3 - Disorders of nucleotide metabolism anemias THALASSEMIA D56. 0 Alpha 1 - Beta 2 - Delta-beta 3 - Minor 4 - HPFH (Hereditary persistence of fetal hemoglobin) 5 - Hemoglobin E-beta SICKLE CELL DISORDERS 0 - Hb-SS with crisis 1 - Sickle cell without crisis 3 - Sickle-cell trait 2 - Hb-C disease 4 - Thalassemia Sickle-Cell 0 - Without crisis 1 - With crisis Use additional code for any associated fever OTHER ANEMIAS D57. 0 - Un 1 - Acute chest syndrome 2 - Splenic sequestration 0 - Hereditary sideroblastic anemia 1 - Secondary sideroblastic anemia due to disease 2 - Secondary sideroblastic anemia due to drugs and toxins 3 - Other sideroblastic anemias 4 - Congenital dyserythropoietic anemia 8 Other anemias 1 - Acute chest syndrome 2 - Splenic sequestration D64. 1- Anemia due to anti-neoplastic chemotherapy 9 -Other anemias Hemolytic Anemia, ACQUIRED D59. 0 -Drug-induced autoimmune (Use additional code for adverse effects, if applicable, to identify drug) 1 - Other autoimmune 2 - Drug-induced non-autoimmune (Use additional code for adverse effects, if applicable, to identify drug) 3 - Hemolytic-uremic syndrome (Use additional code to identify associated: E. coli, Pneumoncoccal pneumonia, or Shigella dysenteriae) 4 - Other non-autoimmune 5 - Paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli) 6 - Hemoglobinuria due to hemolysis from other external causes acquired hemolytic anemias Other Aplastic Anemias & Other BONE MARROW FAILURE SYNDROMES 0 Constitutional aplastic anemia aplastic anemias & other bone marrow failure syndromes 1 - Constitutional (pure) red blood cell aplasia constitutional aplastic anemia 1- Pancytopenia 2 - Myelophthisis 9- Other aplastic anemias & other bone marrow failure syndrome D61. 0 - Antineoplastic chemotherapy induced pancytopenia 1 - Other drug-induced pancytopenia pancytopenia 1 - Drug-induced aplastic anemia (If applicable, use additional code for adverse effects to identify drug) 2 - Aplastic anemia due to other external agents (If applicable, code first toxic effects of substance chiefly non-medicinal as to source (T51-T65) 3 - Idiopathic aplastic anemia Anemia in CHRONIC DISEASES CLASSIFIED ELSEWHERE 0 - Anemia in neoplastic disease (Code first neoplasm) 1 - Anemia in chronic kidney disease (Code first underlying CKD) 8 - Anemia in other chronic diseases classified elsewhere (Code first underlying disease such as: - Hypothyroidism - Tuberculosis) D63. HEMATOLOGY 4
NEUTROPENIA & OTHER WBC DISORDERS NEUTROPENIA 0 Congenital agranulocytosis 1 - Agranulocytosis secondary to cancer chemotherapy (Code also underlying neoplasm. Use additional code for adverse effect, if applicable, to identify drug.) 2 - Other drug-induced agranulocytosis (Use additional code for adverse effect, if applicable, to identify drug) 3 - Neutropenia due to infection 4 - Cyclic neutropenia Use additional code for any associated fever & mucositis Functional Disorders of POLYMORPHONUCLEAR NEUTROPHILS D70. D71 Other Disorders of WHITE BLOOD CELLS 0 - Genetic anomalies of leukocytes 1 - Eosinophilia disorders of white blood cells 1 - Decreased white blood cell count 2 - Elevated white blood cell count disorders D72. 0 - Lymphocytopenia 9 - un 0 - Lymphocytosis (symptomatic) 1 - Monocytosis (symptomatic) 2 - Plasmacytosis 3 - Leukemoid reaction 4 - Basophilia 5 - Bandemia MYELOFIBROSIS, HLH, HIT, 2 POLYCYTHEMIA Other & Un Diseases of BLOOD & BLOOD- FORMING ORGANS (MYELOFIBROSIS) 0 - Familial erythrocytosis 1 - Secondary polycythemia diseases of blood and bloodforming organs D75. 1- MYELOFIBROSIS (Code first underlying disorder such as neoplasm. If applicable, code for associated therapy-related MDS and for adverse effects to identify drug) 2 - Heparin induced thrombocytopenia (HIT) diseases of blood and blood-forming organs Other Specified Diseases W/Participation of D76. LYMPHORETICULAR & RETICULOHISTIOCYTIC TISSUE 1 - Hemophagocytic lymphohistiocytosis 2 - Hemophagocytic syndrome, infection-associated (Use additional code to identify infectious agent or disease) 3 - Other histiocytosis syndromes HEMATOLOGY 5
ACUTE EMBOLISM & I82.4 THROMBOSIS OF DEEP VEINS OF LOWER EXTREMITY HEPATIC THROMBOSIS I81: PORTAL VEIN THROMBOSIS I82.0: BUDD-CHIARI SYNDROME 0 - Un deep veins/ lower extremity 1 - Femoral vein 2 - Iliac vein 3 - Popliteal vein 4 - Tibial vein Y - Proximal lower extremity Z - Distal lower extremity 1 - Right 2 - Left 3 - Bilateral POSTTHROMBOTIC I87.0 SYNDROME 0 - Without complications 1 - With ulcer 2 - With inflammation 3 - With ulcer & inflammation 9 - With other complications 1 - Right lower extremity 2 - Left lower extremity 3 - Bilateral lower extremity lower extremity PULMONARY I26.9 EMBOLISM W/O ACUTE COR PULMONALE COAGULATION DEFECTS D68. Other 0 - Von Willebrand's disease 1- Hereditary factor XI deficiency 2 -Hereditary deficiency of other clotting factors 4 - Acquired coagulation factor deficiency coagulation defects D68. CIRCULATING ANTI-COAGULANTS Hemorrhagic Disorders due to 3 - Hemorrhagic disorder due to circulating anticoagulants 0 - Septic pulmonary embolism 2 - Saddle embolus of pulmonary artery NONPYOGENIC I67.6 THROMBOSIS OF INTRACRANIAL VENOUS SYSTEM D75.82 HEPARIN INDUCED THROMBOCYTOPENIA 2 - Hemorrhagic disorder due to extrinsic circulating anticoagulants Other Coagulation Defects, THROMBOPHILIA 5 - Primary thrombophilia 6 - Other thrombophilia 1- Hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors 1 - Acquired hemophilia 2 - Antiphospholipid antibody with hemorrhagic disorder Hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors D68. 1- Activated protein C resistance 2 - Prothrombin gene mutation primary thrombophilia 1- Antiphospholipid syndrome 2 - Lupus anticoagulant syndrome thrombophilia HEMATOLOGY 6
COMBINED IMMUNODEFICIENCIES combined immunodeficiencies 1 - Biotindependent carboxylase deficiency combined immunodeficiencies D81. 0 - Severe combined immunodeficiency [SCID] with reticular dysgenesis 1 - SCID with low T- and B-cell numbers 2 - SCID with low or normal B-cell numbers 3 - Adenosine deaminase [ADA] deficiency 4 - Nezelof's syndrome 5 - Purine nucleoside phosphorylase [PNP] deficiency 6 - Major histocompatibility complex class I deficiency 7 - Major histocompatibility complex class II deficiency 0 - Biotinidase deficiency biotindependent carboxylase Deficiency Immunodeficiency associated with OTHER MAJOR DEFECTS 0 - Wiskott-Aldrich syndrome 1 - Di George's syndrome 2 -Immunodeficiency with short-limbed stature 3 -Immunodeficiency following hereditary defective response to Epstein-Barr virus 4 -Hyperimmunoglobulin E [IgE] syndrome major defects COMMON VARIABLE IMMUNODEFICIENCY 0 - With predominant abnormalities of B-cell numbers and function 1 - With predominant immunoregulatory T-cell disorders 2 -With autoantibodies to B- or T-cells common variable immunodeficiency 9 -Un D82. D83. GRAFT-VERSUS-HOST DISEASE 0 - Acute 1 - Chronic 2 - Acute on chronic 3 - Un D89.81 DRY EYE SYNDROME HO4.12 1 - Right lacrimal gland 2 Left lacrimal gland 3 - Bilateral lacrimal glands lacrimal gland ENLARGED LYMPH NODES 0 - Localized 1 - Generalized R59. Other Disorders Involving the IMMUNE MECHANISM, NOT ELSEWHERE CLASSIFIED 0 - Polyclonal hypergammaglobulinemia 1 - Cryoglobulinemia 2 - Hypergammaglobulinemia, un 3 - Immune reconstitution syndrome disorders involving the immune mechanism, not elsewhere classified 1 - Graftversushost disease D89. 2 - Autoimmune lymphoproliferative syndrome [ALPS] disorders 0 - Acute 1 - Chronic 2 -Acute on chronic graftversus-host disease 3 - Un HEMATOLOGY 8
DRUG-INDUCED PHENOMENON AGRANULOCYTOSIS SECONDARY TO CANCER CHEMOTHERAPY D70.1 TOXIC LIVER DISEASE 0 - With cholestasis 2 - With acute hepatitis K71. AGRANULOCYTOSIS D70.2 DUE TO NON-CHEMOTHERAPY DRUG ANEMIA DUE TO ANTINEOPLASTIC CHEMOTHERAPY OTHER SECONDARY THROMBOCYTOPENIA D69.59 * Due to drugs, EtOH, alloimmunization; NOT ITP DRUG (OPIATE) INDUCED CONSTIPATION DRUG (OPIATE) INDUCED CONSTIPATION D64.81 K59.09 K59.09 1 - With hepatic necrosis This is for toxic effects due to drug; Does Not include ETOH 0 - Without coma 1 -With coma Other & Un POLYNEUROPATHIES 0 - Drug-induced 1 - Alcoholic 2 - Due to other toxic agents (as used for AIDS) 1 -Critical illness 2 - Radiation-induced G62. POLYNEUROPATHY IN DISEASES CLASSIFIED ELSEWHERE (Includes myeloma induced neuropathy) ORAL MUCOSITIS, ULCERATIVE 0 -Un 1 - Due to antineoplastic therapy 2 - Due to other drugs 3 - Due to radiation G63 K12.3 DRUG-INDUCED ADRENOCORTICAL INSUFFICIENCY E27.3 DRUG-INDUCED CUSHING'S SYNDROME E24.2 9