/MDS Other of SPECIFIED CELL TYPE C94. Leukemia, C91. 0 - Acute erythroid leukemia LYMPHOID 2 - Acute megakaryoblastic leukemia 0 - Not having 3 - achieved remission 0 - Acute lymphoblastic leukemia 4 - Acute panmyelosis with myelofibrosis 1 - In remission 1- Chronic lymphocytic leukemia of 8 - Other specified leukemias 2 - In relapse B-cell type 3 - Prolymphocytic leukemia of B-cell type 0 - Not having 6 - Myelodysplastic disease, not classified STOP 4 - achieved remission 5 - Adult T-cell /leukemia 1 - In remission (HTLV-1-associated) 2 - In relapse 6 - Prolymphocytic leukemia of T-cell type Leukemia of A - Mature B-cell leukemia Burkitt-type C95. Z - Other UNSPECIFIED 9 - Unspecified CELL TYPE 0 - Not having 0 - Acute achieved remission 1 - Chronic 1- In remission Leukemia, 9 - Unspecified C92. 2– In relapse MYELOID 0 - Acute myeloblastic leukemia Unspecified Malignant Neoplasms of 1 - Chronic , BCR/ABL- C96. positive 0 -Not having LYMPHOID, HEMATO 2 - Atypical chronic myeloid leukemia, achieved remission BCR/ABL-negative 1 - In remission POIETIC & RELATED TISSUE 3 - 2 - In relapse 0 - Multifocal and multisystemic (disseminated) 4 - Acute promyelocytic leukemia Langerhans-cell 5 - Acute myelomonocytic leukemia 2 - Malignant mast cell tumor 6 - with 11q23- 4 - Sarcoma of dendritic cells (accessory cells) abnormality 5 - Multifocal and unisystemic Langerhans-cell A - Acute myeloid leukemia with histiocytosis multilineage dysplasia 6 - Unifocal Langerhans-cell histiocytosis Z - Other myeloid leukemia A - Histiocytic sarcoma 9 - Unspecified Z - Other specified 9 - Unspecified

Leukemia,

MONOCYTIC C93. MYELODYSPLASTIC D46. 0 - Acute monoblastic/monocytic SYNDROME (MDS) leukemia 0 - Not having achieved remission 0 - Refractory without ring sideroblasts, so stated 1 - Chronic myelomonocytic leukemia 1 - Refractory anemia with ring sideroblasts 3 - Juvenile myelomonocytic leukemia 1- In remission 2– In relapse A - Refractory cytopenia with multilineage dysplasia Z - Other monocytic leukemia B - Refractory cytopenia with multilineage dysplasia and 9 - Unspecified ring sideroblasts C - with isolated del(5q) STOP chromosomal abnormality 4 - Refractory anemia, unspecified Z - Other myelodysplastic syndromes Poisoning by Adverse Effect of & Underdosing of, T45.1X 9 - Unspecified 0 - Not having achieved remission 2 - Refractory anemia ANTINEOPLASTIC & 1- 1 with excess of blasts IMMUNOSUPPRESSIVE DRUGS 2– 2 1- Poisoning by antineoplastic and Use T45.1X code for adverse effect, if applicable immunosuppressive drugs, A - initial encounter accidental (unintentional) D - subsequent encounter 5- Adverse effect of antineoplastic & S - sequela Multiple Myeloma & Malignant

immunosuppressive drugs PLASMA CELL C90. NEOPLASM 0 - Multiple Myeloma 0 - Not having achieved remission 1 - Plasma cell leukemia 1 - In remission 2 - Extramedullary 2 – In relapse 3 - Solitary plasmacytoma

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B-CELL LYMPHOMA

Lymphoma, Lymphoma,

HODGKIN C81. OTHER C85. 0 - Nodular 0 - Unspecified site NON-HODGKIN predominant 1 - Head, neck, face 0 - Unspecified site 1 - classical 2 - Intrathoracic 1 - Unspecified -B cell lymphoma 1 - Head, neck, face 2 - Mixed cellularity classical 3 - Intra-abdominal 2 - Mediastinal (thymic) large 2 - Intrathoracic 3 - Lymphocyte-depleted 4 - Axilla and upper limb B-cell 3 - Intra-abdominal classical 5 - Inguinal region and lower limb 8 - Other specified types of 4 - Axilla and upper limb 4 - Lymphocyte-rich classical 6 -Intrapelvic non-Hodgkin’s 5 - Inguinal region and lower limb 7 - Other classical 7 -Spleen 9 - Unspecified 6 -Intrapelvic 9 - Unspecified 8 - Multiple-sites 7 -Spleen 9 - Extranodal and solid organ site 8 - Multiple-sites 9 - Extranodal and solid organ site

Lymphoma, C82. , FOLLICULAR MALIGNANT C88. 0 - Grade I 1 - Grade II 0 - Unspecified site IMMUNOPROLIFERATIVE 2 - Grade III unspecified 1 - Head, neck, face & OTHER B-CELLS 3 - Grade IIIa 2 - Intrathoracic 4 - Grade IIIb 3 - Intra-abdominal 0 - Waldenstrom macroglobulinemia 5 - Diffuse follicle center 4 - Axilla and upper limb 2 - lymphoma 5 - Inguinal region and lower limb 3 - Immunoproliferative small intestinal disease 6 - Cutaneous follicle center 6 -Intrapelvic 4 - Extranodal marginal zone B-cell lymphoma of mucosa- lymphoma 7 -Spleen associated lymphoid tissue MALT[ -lymphoma] 8 - Other types lymphoma 8 - Multiple-sites 8 - Other malignant immunoproliferative diseases 9 - Unspecified 9 - Extranodal and solid organ site 9 - Unspecified.

Lymphoma, Other SPECIFIED T/NK-CELL C86. Lymphoma, 0 - Extranodal NK/T-cell lymphoma, nasal type C83. 1 - Hepatosplenic T-cell lymphoma NON-FOLLICULAR 2 - Enteropathy-type (intestinal) - lymphoma 0 - Small cell B-cell Lymphoma 0 - Unspecified site 3 - Subcutaneous panniculitis-like-T-cell lymphoma 1 - 1 - Head, neck, face 4 - Blastic NK-cell lymphoma 3 - Diffuse Large -B cell 2 - Intrathoracic 5 - Angiommunoblastic -T cell lymphoma lymphoma 3 - Intra-abdominal 6 - Primary cutaneous CD-30-positive -T cell proliferations 5 - Lymphoblastic (diffuse 4 - Axilla and upper limb lymphoma) 5 - Inguinal region and lower limb 7 - 6 -Intrapelvic 8 - Other non-follicular 7 -Spleen Lymphoma (includes PEL/ 8 - Multiple-sites intravascular) 9 - Extranodal and solid organ site 9 - Unspecified

© Copyright 2015; Revised 9/24/15 HEMATOLOGY 2 MYELOPROLIF/MGUS Neoplasms of Uncertain Behavior of D45 LYMPHOID, HEMA- D47. VERA TOPOIETIC & RELATED TISSUE 0 - Histiocytic and mast cell tumors of uncertain behavior MONOCLONAL D47.2 1 - Chronic myeloproliferative disease GAMMOPATHY 2 - STOP 3 - Essential (hemorrhagic) 4 - Osteomyelofibrosis 9 - Unspecified Other & Unspecified Diseases of D75. Z - Other specified 1- Post-transplant lymphoproliferative & BLOOD- neoplasms disorder (PTLD) 9 - Other specified neoplasms FORMING ORGANS

For MYELOFIBROSIS, use D75.81 (MYELOFIBROSIS) 0 - Familial erythrocytosis 1 - Secondary polycythemia STOP ESSENTIAL D47.3 9 - Unspecified (HEMORRHAGIC) 8 - Other 1- MYELOFIBROSIS specified (Code first underlying disorder such as THROMBOCYTHEMIA diseases neoplasm. If applicable, code for associated of therapy-related MDS and for adverse effects blood and to identify drug) blood- 2 - Heparin induced thrombocytopenia (HIT) MYELOFIBROSIS D75.81 forming 9 - Other specified diseases of blood organs and blood-forming organs

Anemia, NUTRITIONAL FOLATE DEFICIENCY D52. Anemia, 0 –Dietary D50. 1 - Drug-induced IRON DEFICIENCY (Use additional code for adverse effects, 0 - Secondary to blood loss (chronic) if applicable, to identify drug) 1 - Sideropenic dysphagia 8 - Other 8 - Other iron deficiency anemias 9 - Unspecified 9 - Unspecified Anemias,

Anemia, D53. D51. OTHER NUTRITIONAL VITAMIN B12 0 - Protein deficiency anemia 1 - Other megaloblastic anemias, DEFICIENCY not elsewhere classified 0 - Intrinsic factor deficiency 2 - Scorbutic anemia 1 - Selective vitamin B12 malabsorption with proteinuria 8 - Other 2 - Transcobalamin II deficiency 9 - Unspecified 3 - Other dietary Vitamin B12 deficiency 8 - Other 9 - Unspecified IRON OVERLOAD & E83.1 OTHERS

0 - Disorder of Iron Metabolism, unspecified STOP

0 - Hereditary 1 - Due to repeated red 1 - Hemochromatosis blood cell transfusions 8 - Other 9 - Unspecified © Copyright 2015; Revised 9/24/15 HEMATOLOGY , OTHER ANEMIAS ACQUIRED D59. 0 -Drug-induced autoimmune Anemia due to (Use additional code for adverse effects, if applicable, to D55. identify drug) ENZYME DISORDERS 1 - Other autoimmune 0 - Due to glucose-6-phosphate dehydrogenase [G6PD] deficiency 2 - Drug-induced non-autoimmune 1 - Other disorders of glutathione metabolism (Use additional code for adverse effects, if applicable, to 2 - Disorders of glycolytic enzymes identify drug) 3 - Disorders of nucleotide metabolism 3 - Hemolytic-uremic syndrome 8 - Other anemias (Use additional code to identify associated: E. coli, 9 - Unspecified Pneumoncoccal pneumonia, or Shigella dysenteriae) 4 - Other non-autoimmune 5 - Paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli) 6 - Hemoglobinuria due to hemolysis from other external causes 8 - Other acquired hemolytic anemias D56. 9 - Unspecified 0 –Alpha 1 - Beta 2 - Delta-beta 3 - Minor Other Aplastic Anemias & Other 4 - HPFH (Hereditary persistence of fetal ) 5 - Hemoglobin E-beta D61. 8 - Other MARROW 9 - Unspecified FAILURE SYNDROMES 0– Constitutional 1 - Constitutional (pure) aplasia 9 - Other constitutional aplastic anemia SICKLE CELL D57. 0 - Antineoplastic 8 - Other DISORDERS 1- Pancytopenia chemotherapy specified induced 0 - Unspecified aplastic pancytopenia 0 - Hb-SS with crisis 1 - Acute chest syndrome STOP anemias & 1 - Other drug-induced 2 - Splenic sequestration other bone pancytopenia marrow 8 - Other pancytopenia 1 - Sickle cell without crisis failure 2 - Myelophthisis 3 - Sickle-cell trait STOP syndromes 9- Other specified

aplastic anemias STOP & other bone 2 - Hb-C disease 0 - Without crisis STOP 4 - Thalassemia marrow failure 8 - Other Sickle-Cell syndrome 1 - Acute chest 1 - With crisis syndrome 1 - Drug-induced aplastic anemia 2 - Splenic (If applicable, use additional code for adverse effects sequestration Use additional code for any associated fever 9 - Unspecified to identify drug) 2 - Aplastic anemia due to other external agents STOP (If applicable, code first toxic effects of substance chiefly non-medicinal as to source (T51-T65) 3 - Idiopathic aplastic anemia 9 - Unspecified OTHER ANEMIAS D64. 0 - Hereditary 1 - Secondary sideroblastic anemia due to disease 2 - Secondary sideroblastic anemia due to drugs Anemia in and toxins STOP D63. 3 - Other sideroblastic anemias CHRONIC DISEASES 4 - Congenital dyserythropoietic anemia 9 - Unspecified CLASSIFIED ELSEWHERE 0 - Anemia in neoplastic disease 8 –Other specified 1- Anemia due to (Code first neoplasm) anemias anti-neoplastic 1 - Anemia in chronic kidney disease chemotherapy (Code first underlying CKD) 9 -Other specified anemias 8 - Anemia in other chronic diseases classified elsewhere (Code first underlying disease such as: - Hypothyroidism - )

© Copyright 2015; Revised 9/24/15 HEMATOLOGY 4

NEUTROPENIA & OTHER WBC DISORDERS

Other Disorders of D70. D72. 0 – Congenital WHITE BLOOD CELLS 1 - Agranulocytosis secondary to chemotherapy 0 - Genetic anomalies of leukocytes (Code also underlying neoplasm. Use additional code for 1 - STOP adverse effect, if applicable, to identify drug.) 9 - Unspecified 2 - Other drug-induced agranulocytosis (Use additional code for adverse effect, if applicable, to

identify drug) 1 - Decreased 0 - Lymphocytopenia 3 - Neutropenia due to white blood 8 - Other 4 - cell count 9 - unspecified 8 - Other 9 - Unspecified 8 - Other specified 0 - Use additional code for any associated fever disorders (symptomatic)

& mucositis of white 2 - Elevated 1 - blood cells white blood (symptomatic) cell count 2 - Plasmacytosis Functional Disorders of 3 - D71 4 - POLYMORPHONUCLEAR 5 - Bandemia 8 - Other 9 - Unspecified 9 - Other specified STOP disorders

MYELOFIBROSIS, HLH, HIT, 2° POLYCYTHEMIA

Other & Unspecified Diseases of Other Specified Diseases D76. BLOOD & BLOOD- D75. W/Participation of FORMING ORGANS LYMPHORETICULAR & (MYELOFIBROSIS) RETICULOHISTIOCYTIC TISSUE 1 - Hemophagocytic lymphohistiocytosis 0 - Familial erythrocytosis 2 - Hemophagocytic syndrome, infection-associated 1 - Secondary polycythemia STOP (Use additional code to identify infectious agent 9 - Unspecified or disease) 3 - Other histiocytosis syndromes 8 - Other 1- MYELOFIBROSIS specified (Code first underlying disorder such as diseases neoplasm. If applicable, code for associated of therapy-related MDS and for adverse effects blood and to identify drug) blood- 2 - Heparin induced thrombocytopenia (HIT) forming 9 - Other specified diseases of blood organs and blood-forming organs

© Copyright 2015; Revised 9/24/15 HEMATOLOGY 5

ACUTE EMBOLISM & I82.4 HEPATIC THROMBOSIS THROMBOSIS OF DEEP VEINS OF I81: PORTAL VEIN THROMBOSIS LOWER EXTREMITY I82.0: BUDD-CHIARI SYNDROME 0 - Unspecified deep veins/ lower extremity 1 - Femoral vein 1 - Right Other 2 - Iliac vein 2 - Left D68. 3 - Popliteal vein 3 - Bilateral COAGULATION DEFECTS 4 - Tibial vein 9 - Unspecified 0 - Von Willebrand's disease 9 - Other specified 1- Hereditary factor XI deficiency Y - Proximal lower extremity 2 -Hereditary deficiency of other clotting factors Z - Distal lower extremity 4 - Acquired coagulation factor deficiency 8 - Other specified coagulation defects 9 - Unspecified POSTTHROMBOTIC I87.0 SYNDROME Hemorrhagic Disorders due to D68. 0 - Without complications 1 - Right lower extremity CIRCULATING 1 - With ulcer 2 - Leftlower extremity 2 - With 3 - Bilateral lower extremity ANTI-COAGULANTS 3 - With ulcer & inflammation 9 - Unspecified lower extremity 1 - Acquired 9 - With other complications 1- Hemorrhagic hemophilia disorder due 2 - Antiphospholipid antibody with hem- to intrinsic orrhagic disorder 3 - Hemorrhagic circulating disorder due 8 - Other Hemorrhagic anticoagulants, PULMONARY I26.9 to circulating disorder due to antibodies, anticoagulants intrinsic circulat- EMBOLISM W/O or inhibitors ing anticoagulants, ACUTE COR PULMONALE antibodies, 0 - Septic pulmonary embolism or inhibitors 2 - Saddle embolus of pulmonary artery 2 - Hemorrhagic 9 - Other disorder due to extrinsic STOP circulating NONPYOGENIC I67.6 anticoagulants THROMBOSIS OF INTRACRANIAL VENOUS Other Coagulation Defects, SYSTEM THROMBOPHILIA D68. 5 - Primary 1- Activated protein C resistance thrombophilia 2 - Prothrombin gene mutation HEPARIN INDUCED D75.82 9 - Other primary thrombophilia THROMBOCYTOPENIA 1- Antiphospholipid syndrome 6 - Other 2 - Lupus anticoagulant syndrome thrombophilia 9 - Other thrombophilia

© Copyright 2015; Revised 9/24/15 HEMATOLOGY 6

Immunodeficiency associated with COMBINED D81. D82. OTHER MAJOR 0 - Severe combined [SCID] with DEFECTS reticular dysgenesis 0 - Wiskott-Aldrich syndrome 1 - SCID with low T- and B-cell numbers 1 - Di George's syndrome 2 - SCID with low or normal B-cell numbers 2 -Immunodeficiency with short-limbed stature 3 - Adenosine deaminase [ADA] deficiency STOP 3 -Immunodeficiency following hereditary defective 4 - Nezelof's syndrome response to Epstein-Barr virus 5 - Purine nucleoside phosphorylase [PNP] deficiency 4 -Hyperimmunoglobulin E [IgE] syndrome 6 - Major histocompatibility complex class I deficiency 8 - Other specified major defects 7 - Major histocompatibility complex class II deficiency 9 - Unspecified 9 - Unspecified

8 - Other 1 - Biotin- 0 - Biotinidase deficiency combined dependent 8 - Other biotin- COMMON VARIABLE D83. immuno- carboxylase dependent carboxylase IMMUNODEFICIENCY deficiencies deficiency Deficiency 9 - Unspecified 0 - With predominant abnormalities of B-cell numbers and function 9 - Other 1 - With predominant immunoregulatory T-cell disorders combined 2 -With autoantibodies to B- or T-cells STOP immuno- 8 - Other common variable immunodeficiency deficiencies 9 -Unspecified

Other Disorders Involving the GRAFT-VERSUS-HOST D89.81 D89. DISEASE IMMUNE 0 - Acute MECHANISM, NOT 1 - Chronic ELSEWHERE CLASSIFIED 2 - Acute on chronic 3 - Unspecified 0 - Polyclonal hypergammaglobulinemia 1 - Cryoglobulinemia 2 - Hypergammaglobulinemia, unspecified STOP 3 - Immune reconstitution syndrome DRY EYE SYNDROME HO4.12 9 - Unspecified 1 - Right lacrimal gland 8 - Other 0 - Acute 2– Left lacrimal gland 1 - Graft- specified 1 - Chronic 3 - Bilateral lacrimal glands versus- disorders 2 -Acute on chronic graft- 9 - Unspecified lacrimal gland host involving versus-host disease disease the 3 - Unspecified immune mechanism, not 2 - Autoimmune ENLARGED LYMPH R59. lymphoproliferative elsewhere STOP NODES classified syndrome [ALPS] 9 - Other specified disorders 0 - Localized 1 - Generalized

© Copyright 2015; Revised 9/24/15 HEMATOLOGY 8

DRUG-INDUCED PHENOMENON

AGRANULOCYTOSIS D70.1 TOXIC LIVER K71. SECONDARY TO CANCER DISEASE 0 - With cholestasis CHEMOTHERAPY 2 - With acute hepatitis STOP

1 - With hepatic necrosis 0 - Without coma 1 -With coma AGRANULOCYTOSIS D70.2 This is for toxic effects due to drug; DUE TO Does Not include ETOH NON-CHEMOTHERAPY DRUG Other & Unspecified G62. POLYNEUROPATHIES ANEMIA DUE TO D64.81 0 - Drug-induced 1 - Alcoholic STOP ANTINEOPLASTIC 2 - Due to other toxic agents CHEMOTHERAPY 9 - Unspecified (as used for AIDS) 1 -Critical illness 8 - Other specified 2 - Radiation-induced OTHER SECONDARY D69.59 9 - Other THROMBOCYTOPENIA * Due to drugs, EtOH, alloimmunization; NOT ITP POLYNEUROPATHY IN G63 DISEASES CLASSIFIED DRUG (OPIATE) K59.09 ELSEWHERE INDUCED (Includes myeloma induced neuropathy) CONSTIPATION ORAL MUCOSITIS, K12.3 ULCERATIVE DRUG (OPIATE) K59.09 0 -Unspecified 1 - Due to antineoplastic therapy INDUCED 2 - Due to other drugs CONSTIPATION 3 - Due to radiation 9 - Other

DRUG-INDUCED E27.3 DRUG-INDUCED E24.2 ADRENOCORTICAL CUSHING'S INSUFFICIENCY SYNDROME

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