# Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)

    Excludes2: autoimmune disease (systemic) NOS (M35.9)
       certain conditions originating in the perinatal period (P00-P96)
       complications of pregnancy, childbirth and the puerperium (O00-O99)
       congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
       endocrine, nutritional and metabolic diseases (E00-E90)
       human immunodeficiency virus [HIV] disease (B20)
       injury, poisoning and certain other consequences of external causes (S00-T98)
       neoplasms (C00-D49)
       symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    This chapter contains the following blocks:
    D50-D53 Nutritional anemias
    D55-D59 Hemolytic anemias
    D60-D64 Aplastic and other anemias and other bone marrow failure syndromes
    D65-D69 Coagulation defects, purpura and other hemorrhagic conditions
    D70-D77 Other disorders of blood and blood-forming organs
    D78 Intraoperative and postprocedural complications of spleen
    D80-D89 Certain disorders involving the immune mechanism

# Nutritional anemias (D50-D53)Nutritional anemias (D50-D53)
## D50 Iron deficiency anemiaD50 Iron deficiency anemia

    Includes: asiderotic anemia
    hypochromic anemia
    D50.0 Iron deficiency anemia secondary to blood loss (chronic)
       Posthemorrhagic anemia (chronic)
       Excludes1: acute posthemorrhagic anemia (D62)
          congenital anemia from fetal blood loss (P61.3)
    D50.1 Sideropenic dysphagia
       Kelly-Paterson syndrome
       Plummer-Vinson syndrome
    D50.8 Other iron deficiency anemias
       Iron deficiency anemia due to inadequate dietary iron intake
    D50.9 Iron deficiency anemia, unspecified

## D51 Vitamin B12 deficiency anemiaD51 Vitamin B12 deficiency anemia

    Excludes1: vitamin B12 deficiency (E53.8)
    D51.0 Vitamin B12 deficiency anemia due to intrinsic factor deficiency
       Addison anemia
       Biermer anemia
       Pernicious (congenital) anemia
       Congenital intrinsic factor deficiency
    D51.1 Vitamin B12 deficiency anemia due to selective vitamin B12 malabsorption with proteinuria
       Imerslund (-Gr?sbeck) syndrome
       Megaloblastic hereditary anemia
    D51.2 Transcobalamin II deficiency
    D51.3 Other dietary vitamin B12 deficiency anemia
       Vegan anemia
    D51.8 Other vitamin B12 deficiency anemias
    D51.9 Vitamin B12 deficiency anemia, unspecified

## D52 Folate deficiency anemiaD52 Folate deficiency anemia

    Excludes1: folate deficiency without anemia (E53.8)
    D52.0 Dietary folate deficiency anemia
       Nutritional megaloblastic anemia
    D52.1 Drug-induced folate deficiency anemia
       Code first (T36-T50) to identify drug
    D52.8 Other folate deficiency anemias
    D52.9 Folate deficiency anemia, unspecified
       Folic acid deficiency anemia NOS

## D53 Other nutritional anemiasD53 Other nutritional anemias

    Includes: megaloblastic anemia unresponsive to vitamin B12 or folate therapy
    D53.0 Protein deficiency anemia
       Amino-acid deficiency anemia
       Orotaciduric anemia
       Excludes1: Lesch-Nyhan syndrome (E79.1)
    D53.1 Other megaloblastic anemias, not elsewhere classified
       Megaloblastic anemia NOS
       Excludes1: Di Guglielmo's disease (C94.0)
    D53.2 Scorbutic anemia
       Excludes1: scurvy (E54)
    D53. ​8 Other specified nutritional anemias
       Anemia associated with deficiency of copper
       Anemia associated with deficiency of molybdenum
       Anemia associated with deficiency of zinc
       Excludes1: nutritional deficiencies without anemia, such as:
          copper deficiency NOS (E61.0)
          molybdenum deficiency NOS (E61.5)
          zinc deficiency NOS (E60)
    D53.9 Nutritional anemia, unspecified
       Simple chronic anemia
       Excludes1: anemia NOS (D64.9)

# Hemolytic anemias (D55-D59)Hemolytic anemias (D55-D59)
## D55 Anemia due to enzyme disordersD55 Anemia due to enzyme disorders

    Excludes1: drug-induced enzyme deficiency anemia (D59.2)
    D55.0 Anemia due to glucose-6-phosphate dehydrogenase [G6PD] deficiency
       Favism
       G6PD deficiency anemia
    D55.1 Anemia due to other disorders of glutathione metabolism
       Anemia (due to) enzyme deficiencies, except G6PD, related to the hexose monophosphate [HMP] shunt pathway
       Anemia (due to) hemolytic nonspherocytic (hereditary), type I
    D55.2 Anemia due to disorders of glycolytic enzymes
       Hemolytic nonspherocytic (hereditary) anemia, type II
       Hexokinase deficiency anemia
       Pyruvate kinase [PK] deficiency anemia
       Triose-phosphate isomerase deficiency anemia
       Excludes1: disorders of glycolysis not associated with anemia (E74.8)
    D55.3 Anemia due to disorders of nucleotide metabolism
    D55.8 Other anemias due to enzyme disorders
    D55.9 Anemia due to enzyme disorder, unspecified
    D56 Thalassemia
       Excludes1: sickle-cell thalassemia (D57.4-)

## D56.0 Alpha thalassemiaD56.0 Alpha thalassemia

       Alpha thalassemia major
       Hemoglobin H disease
       Severe alpha thalassemia
       Triple gene defect alpha thalassemia
       Excludes1: alpha thalassemia minor (D56.3)
          asymptomatic alpha thalassemia (D56.3)
          hydrops fetalis due to hemolytic disease (P56.-)
    D56.1 Beta thalassemia
       Beta thalassemia major
       Cooley' ​s anemia
       Homozygous beta thalassemia
       Severe beta thalassemia
       Thalassemia intermedia
       Excludes1: beta thalassemia minor (D56.3)
          delta-beta thalassemia (D56.2)
          sickle-cell beta thalassemia (D57.4-)
    D56.2 Delta-beta thalassemia
       Homozygous delta-beta thalassemia
       Excludes1: delta-beta thalassemia minor (D56.3)
    D56.3 Thalassemia minor
       Alpha thalassemia minor
       Alpha thalassemia trait
       Beta thalassemia minor
       Delta-beta thalassemia minor
       Excludes1: alpha thalassemia (D56.0)
          beta thalassemia (D56.1)
          delta-beta thalassemia (D56.2)
    D56.4 Hereditary persistence of fetal hemoglobin [HPFH]
    D56.8 Other thalassemias
       Hb-Bart's disease
       Excludes1: sickle cell anemia (D57.-)
          sickle-cell thalassemia (D57.4)
    D56.9 Thalassemia, unspecified
       Mediterranean anemia (with other hemoglobinopathy)
       Thalassemia (minor) (mixed) (with other hemoglobinopathy)

## D57 Sickle-cell disordersD57 Sickle-cell disorders

    Use additional code for any associated fever (R50.81)
    Excludes1: other hemoglobinopathies (D58.-)
    D57.0 Hb-SS disease with crisis
       Sickle-cell disease NOS with crisis
       Hb-SS disease with vasoocclusive pain
       D57.00 Hb-SS disease with crisis, unspecified
       D57.01 Hb-SS disease with acute chest syndrome
       D57.02 Hb-SS disease with splenic sequestration
    D57.1 Sickle-cell disease without crisis
       Hb-SS disease without crisis
       Sickle-cell anemia NOS
       Sickle-cell disease NOS
       Sickle-cell disorder NOS
    D57.2 Sickle-cell/Hb-C disease
       Hb-SC disease
       Hb-S/Hb-C disease
       D57.20 Sickle-cell/Hb-C disease without crisis
       D57.21 Sickle-cell/Hb-C disease with crisis
          D57. ​211 Sickle-cell/Hb-C disease with acute chest syndrome
          D57.212 Sickle-cell/Hb-C disease with splenic sequestration
          D57.219 Sickle-cell/Hb-C disease with crisis, unspecified
             Sickle-cell/Hb-C disease with crisis NOS
    D57.3 Sickle-cell trait
       Hb-S trait
       Heterozygous hemoglobin S
    D57.4 Sickle-cell thalassemia
       Sickle-cell beta thalassemia
       Thalassemia Hb-S disease
       D57.40 Sickle-cell thalassemia without crisis
          Sickle-cell thalassemia NOS
       D57.41 Sickle-cell thalassemia with crisis
          Sickle-cell thalassemia with vasoocclusive pain
          D57.411 Sickle-cell thalassemia with acute chest syndrome
          D57.412 Sickle-cell thalassemia with splenic sequestration
          D57.419 Sickle-cell thalassemia with crisis, unspecified
             Sickle-cell thalassemia with crisis NOS
    D57.8 Other sickle-cell disorders
       Hb-SD disease
       Hb-SE disease
       D57.80 Other sickle-cell disorders without crisis
       D57.81 Other sickle-cell disorders with crisis
          D57.811 Other sickle-cell disorders with acute chest syndrome
          D57.812 Other sickle-cell disorders with splenic sequestration
          D57.819 Other sickle-cell disorders with crisis, unspecified
             Other sickle-cell disorders with crisis NOS

## D58 Other hereditary hemolytic anemiasD58 Other hereditary hemolytic anemias

    Excludes1: hemolytic anemia of the newborn (P55.-)
    D58.0 Hereditary spherocytosis
       Acholuric (familial) jaundice
       Congenital (spherocytic) hemolytic icterus
       Minkowski-Chauffard syndrome
    D58.1 Hereditary elliptocytosis
       Elliptocytosis (congenital)
       Ovalocytosis (congenital) (hereditary)
    D58.2 Other hemoglobinopathies
       Abnormal hemoglobin NOS
       Congenital Heinz body anemia
       Hb-C disease
       Hb​D disease
       Hb-E disease
       Hemoglobinopathy NOS
       Unstable hemoglobin hemolytic disease
       Excludes1: familial polycythemia (D75.0)
          Hb-M disease (D74.0)
          hereditary persistence of fetal hemoglobin [HPFH] (D56.4)
          high-altitude polycythemia (D75.1)
          methemoglobinemia (D74.-)
    D58.8 Other specified hereditary hemolytic anemias
    D58.9 Hereditary hemolytic anemia, unspecified

## D59 Acquired hemolytic anemiaD59 Acquired hemolytic anemia

    D59.0 Drug-induced autoimmune hemolytic anemia
       Code first (T36-T50) to identify drug
    D59.1 Other autoimmune hemolytic anemias
       Autoimmune hemolytic disease (cold type) (warm type)
       Chronic cold hemagglutinin disease
       Cold agglutinin disease
       Cold agglutinin hemoglobinuria
       Cold type (secondary) (symptomatic) hemolytic anemia
       Warm type (secondary) (symptomatic) hemolytic anemia
       Excludes1: Evans syndrome (D69.41)
          hemolytic disease of newborn (P55.-)
          paroxysmal cold hemoglobinuria (D59.6)
    D59.2 Drug-induced nonautoimmune hemolytic anemia
       Drug-induced enzyme deficiency anemia
       Code first (T36-T50) to identify drug
    D59.3 Hemolytic-uremic syndrome
    D59.4 Other nonautoimmune hemolytic anemias
       Mechanical hemolytic anemia
       Microangiopathic hemolytic anemia
       Toxic hemolytic anemia
    D59.5 Paroxysmal nocturnal hemoglobinuria [Marchiafava-Micheli]
       Excludes1: hemoglobinuria NOS (R82.3)
    D59.6 Hemoglobinuria due to hemolysis from other external causes
       Hemoglobinuria from exertion
       March hemoglobinuria
       Paroxysmal cold hemoglobinuria
       Use additional code (Chapter 20) to identify external cause
       Excludes1: hemoglobinuria NOS (R82.3)
    D59.8 Other acquired hemolytic anemias
    D59.9 Acquired hemolytic anemia, unspecified
       Idiopathic hemolytic anemia, chronic

# Aplastic ​and other anemias and other bone marrow failure syndromes (D60-D64)
## D60 Acquired pure red cell aplasia [erythroblastopenia]D60 Acquired pure red cell aplasia [erythroblastopenia]

    Includes: red cell aplasia (acquired) (adult) (with thymoma)
    Excludes1: congenital red cell aplasia (D61.01)
    D60.0 Chronic acquired pure red cell aplasia
    D60.1 Transient acquired pure red cell aplasia
    D60.8 Other acquired pure red cell aplasias
    D60.9 Acquired pure red cell aplasia, unspecified

## D61 Other aplastic anemias and other bone marrow failure syndromesD61 Other aplastic anemias and other bone marrow failure syndromes

    Excludes1: neutropenia (D70.-)
    D61.0 Constitutional aplastic anemia
       D61.01 Constitutional (pure) red blood cell aplasia
       Excludes1: acquired red cell aplasia (D60.9)
          Blackfan-Diamond syndrome
          Congenital (pure) red cell aplasia
          Familial hypoplastic anemia
          Primary (pure) red cell aplasia
          Red cell (pure) aplasia of infants
       D61.09 Other constitutional aplastic anemia
          Fanconi's anemia
          Pancytopenia with malformations
    D61.1 Drug-induced aplastic anemia
       Code first (T36-T50) to identify drug
    D61.2 Aplastic anemia due to other external agents
       Code first (T51-T65) to identify cause
    D61.3 Idiopathic aplastic anemia
    D61.8 Other specified aplastic anemias and other bone marrow failure syndromes
       D61.81 Pancytopenia
       Excludes1: pancytopenia (due to) (with):
          aplastic anemia (D61.-)
          bone marrow infiltration (D61.82)
          congenital (pure) red cell aplasia (D61.01)
          drug induced (D61.1)
          hairy cell leukemia (C91.4-)
          human immunodeficiency virus disease (B20.-)
          leukoerythroblastic anemia (M61.82)
          myelodysplastic syndromes (D46.-)
          myeloproliferative disease (D47.1)
       D61.82 Myelophthisis
          Leukoerythroblastic ​anemia
          Myelophthisic anemia
          Panmyelophthisis
          Code also the underlying disorder, such as:
             malignant neoplasm of breast (C50.-)
             tuberculosis (A15.-)
          Excludes1: idiopathic myelofibrosis (D47.1)
             myelofibrosis NOS (D75.81)
             myelofibrosis with myeloid metaplasia (D47.4)
             primary myelofibrosis (D47.1)
             secondary myelofibrosis (D75.81)
       D61.89 Other specified aplastic anemias and other bone marrow failure syndromes
    D61.9 Aplastic anemia, unspecified
       Hypoplastic anemia NOS
       Medullary hypoplasia

## D62 Acute posthemorrhagic anemiaD62 Acute posthemorrhagic anemia

    Excludes1: anemia due to chronic blood loss (D50.0)
       blood loss anemia NOS (D50.0)
       congenital anemia from fetal blood loss (P61.3)

## D63 Anemia in chronic diseases classified elsewhereD63 Anemia in chronic diseases classified elsewhere

    D63.0 Anemia in neoplastic disease
       Code first neoplasm (C00-D49)
       Excludes1: anemia due to antineoplastic chemotherapy (D64.81)
    D63.1 Anemia in chronic kidney disease
       Erythropoietin resistant anemia (EPO resistant anemia)
       Code first underlying chronic kidney disease (CKD) (N18.-)
    D63.8 Anemia in other chronic diseases classified elsewhere
       Code first underlying disease, such as:
          diphyllobothriasis (B70.0)
          hookworm disease (B76.0-B76.9)
          hypothyroidism (E00.0-E03.9)
          malaria (B50.0-B54)
          symptomatic late syphilis (A52.79)
          tuberculosis (A18.89)

## D64 Other anemiasD64 Other anemias

    Excludes1: refractory anemia (D46.-)
    D64.0 Hereditary sideroblastic anemia
       Sex-linked hypochromic sideroblastic anemia
    D64.1 Secondary sideroblastic anemia due to disease
       Code first underlying disease
    D64.2 Secondary sideroblastic anemia due to drugs and toxins
       Code first (T36-T65) to identify drug or toxin
    D64. ​3 Other sideroblastic anemias
       Sideroblastic anemia NOS
       Pyridoxine-responsive sideroblastic anemia NEC
    D64.4 Congenital dyserythropoietic anemia
       Dyshematopoietic anemia (congenital)
       Excludes1: Blackfan-Diamond syndrome (D61.01)
          Di Guglielmo's disease (C94.0)
    D64.8 Other specified anemias
       D64.81 Anemia due to antineoplastic chemotherapy
          Antineoplastic chemotherapy induced anemia
          Excludes1: anemia in neoplastic disease (D63.0)
             aplastic anemia due to antineoplastic chemotherapy (D61.1)
       D64.89 Other specified anemias
          Infantile pseudoleukemia
    D64.9 Anemia, unspecified

# Coagulation defects, purpura and other hemorrhagic conditions (D65-D69)Coagulation defects, purpura and other hemorrhagic conditions (D65-D69)
## D65 Disseminated intravascular coagulation [defibrination syndrome]D65 Disseminated intravascular coagulation [defibrination syndrome]

    Includes: afibrinogenemia, acquired
       consumption coagulopathy
       diffuse or disseminated intravascular coagulation [DIC]
       fibrinolytic hemorrhage, acquired
       fibrinolytic purpura
       purpura fulminans
    Excludes1: disseminated intravascular coagulation (complicating):
       abortion or ectopic or molar pregnancy (O00-O07, O08.1)
       in newborn (P60)
       pregnancy, childbirth and the puerperium (O45.0, O46.0, O67.0, O72.3)

## D66 Hereditary factor VIII deficiencyD66 Hereditary factor VIII deficiency

    Includes: classical hemophilia
       deficiency factor VIII (with functional defect)
       hemophilia NOS
       hemophilia A
    Excludes1: factor VIII deficiency with vascular defect (D68.0)

## D67 Hereditary factor IX deficiencyD67 Hereditary factor IX deficiency

    Includes: christmas disease
       factor IX deficiency (with functional defect)
       hemophilia B
       plasma ​thromboplastin component [PTC] deficiency

## D68 Other coagulation defectsD68 Other coagulation defects

    Excludes1: abnormal coagulation profile (R79.1)
       coagulation defects complicating:
       abortion or ectopic or molar pregnancy (O00-O07, O08.1)
       pregnancy, childbirth and the puerperium (O45.0, O46.0, O67.0, O72.3)

## D68.0 Von Willebrand's diseaseD68.0 Von Willebrand's disease

    Angiohemophilia
    Factor VIII deficiency with vascular defect
    Vascular hemophilia
    Excludes1: capillary fragility (hereditary) (D69.8)
       factor VIII deficiency NOS (D66)
       factor VIII deficiency with functional defect (D66)
    D68.1 Hereditary factor XI deficiency
       Hemophilia C
       Plasma thromboplastin antecedent [PTA] deficiency
       Rosenthal?s disease
    D68.2 Hereditary deficiency of other clotting factors
       AC globulin deficiency
       Congenital afibrinogenemia
       Deficiency of factor I [fibrinogen]
       Deficiency of factor II [prothrombin]
       Deficiency of factor V [labile]
       Deficiency of factor VII [stable]
       Deficiency of factor X [Stuart-Prower]
       Deficiency of factor XII [Hageman]
       Deficiency of factor XIII [fibrin stabilizing]
       Dysfibrinogenemia (congenital)
       Hypoproconvertinemia
       Owren's disease
       Proaccelerin deficiency
    D68.3 Hemorrhagic disorder due to circulating anticoagulants
       D68.31 Hemorrhagic disorder due to intrinsic circulating anticoagulants
          Hemorrhagic disorder due to intrinsic increase in antithrombin
          Hemorrhagic disorder due to intrinsic increase in anti-VIIIa
          Hemorrhagic disorder due to intrinsic increase in anti-IXa
          Hemorrhagic disorder due to intrinsic increase in anti-Xa
          Hemorrhagic disorder due to intrinsic increase in anti-XIa
          Hyperheparinemia
       D68.32 Hemorrhagic disorder due to extrinsic circulating anticoagulants
          Drug-induced hemorrhagic disorder
          Code first (T45.5-) to identify any administered anticoagulant
    D68.4 Acquired coagulation factor deficiency
       Deficiency ​of coagulation factor due to liver disease
       Deficiency of coagulation factor due to vitamin K deficiency
       Excludes1: vitamin K deficiency of newborn (P53)
    D68.5 Primary thrombophilia
       Primary hypercoagulable states
       Excludes1: lupus anticoagulant (D68.62)
          thrombotic thrombocytopenic purpura (M31.1)
       D68.51 Activated protein C resistance
          Factor V Leiden mutation
       D68.52 Prothrombin gene mutation
       D68.59 Other primary thrombophilia
          Antithrombin III deficiency
          Hypercoagulable state NOS
          Primary hypercoagulable state NEC
          Primary thrombophilia NEC
          Protein C deficiency
          Protein S deficiency
          Thrombophilia NOS
    D68.6 Other thrombophilia
       Other hypercoagulable states
       Excludes1: diffuse or disseminated intravascular coagulation [DIC] (D65)
          heparin induced thrombocytopenia (HIT) (D75.82)
          hyperhomocysteinemia (E72.11)
       D68.61 Anticardiolipin syndrome
          Antiphospholipid syndrome
          Excludes1: lupus anticoagulant syndrome (D68.62)
       D68.62 Lupus anticoagulant syndrome
          Lupus anticoagulant
          Presence of systemic lupus erythematosus [SLE] inhibitor
          Excludes1: anticardiolipin syndrome (D68.61)
             antiphospholipid syndrome (D68.61)
       D68.69 Other thrombophilia
          Hypercoagulable states NEC
          Secondary hypercoagulable state NOS
    D68.8 Other specified coagulation defects
       Excludes1: hemorrhagic disease of newborn (P53)
    D68.9 Coagulation defect, unspecified

## D69 Purpura and other hemorrhagic conditionsD69 Purpura and other hemorrhagic conditions

    Excludes1: benign hypergammaglobulinemic purpura (D89.0)
       cryoglobulinemic purpura (D89.1)
       essential (hemorrhagic) thrombocythemia (D47.3)
       hemorrhagic thrombocythemia (D47.3)
       purpura fulminans (D65)
       thrombotic thrombocytopenic purpura (M31.1)
       Waldenström's hypergammaglobulinemic purpura (D89.0)
    D69. ​0 Allergic purpura
       Allergic vasculitis
       Nonthrombocytopenic hemorrhagic purpura
       Nonthrombocytopenic idiopathic purpura
       Purpura anaphylactoid
       Purpura Henoch(-Sch?nlein)
       Purpura rheumatica
       Vascular purpura
       Excludes1: thrombocytopenic hemorrhagic purpura (D69.3)
    D69.1 Qualitative platelet defects
       Bernard-Soulier [giant platelet] syndrome
       Glanzmann's disease
       Grey platelet syndrome
       Thromboasthenia (hemorrhagic) (hereditary)
       Thrombocytopathy
       Excludes1: von Willebrand's disease (D68.0)
    D69.2 Other nonthrombocytopenic purpura
       Purpura NOS
       Purpura simplex
       Senile purpura
    D69.3 Immune thrombocytopenic purpura
       Hemorrhagic (thrombocytopenic) purpura
       Idiopathic thrombocytopenic purpura
       Tidal platelet dysgenesis
    D69.4 Other primary thrombocytopenia
       Excludes1: transient neonatal thrombocytopenia (P61.0)
          Wiskott-Aldrich syndrome (D82.0)
       D69.41 Evans syndrome
       D69.42 Congenital and hereditary thrombocytopenia purpura
          Congenital thrombocytopenia
          Hereditary thrombocytopenia
          Code first: congential or hereditary disorder, such as:
             thrombocytopenia with absent radius (TAR syndrome) (Q87.2)
       D69.49 Other primary thrombocytopenia
          Megakaryocytic hypoplasia
          Primary thrombocytopenia NOS
    D69.5 Secondary thrombocytopenia
       Excludes1: heparin induced thrombocytopenia (HIT) (D75.82)
          transient thrombocytopenia of newborn (P61.0)
    D69.6 Thrombocytopenia, unspecified
    D69.8 Other specified hemorrhagic conditions
       Capillary fragility (hereditary)
       Vascular pseudohemophilia
    D69.9 Hemorrhagic condition, unspecified

# Other ​disorders of blood and blood-forming organs (D70-D77)
## D70 NeutropeniaD70 Neutropenia

    Includes: agranulocytosis
       decreased absolute neurophile count (ANC)
    Use additional code for any associated:
       fever (R50.81)
       mucositis (J34.81, K12.3-, K12.4, K92.81, N76.81)
    Excludes1: neutropenic splenomegaly (D73.81)
       transient neonatal neutropenia (P61.5)
    D70.0 Congenital agranulocytosis
       Congenital neutropenia
       Infantile genetic agranulocytosis
       Kostmann's disease
    D70.1 Agranulocytosis secondary to cancer chemotherapy
       Code first (T45.1-) to identify drug
       Code also underlying neoplasm
    D70.2 Other drug-induced agranulocytosis
       Code first (T36-T50) to identify drug
    D70.3 Neutropenia due to infection
    D70.4 Cyclic neutropenia
       Cyclic hematopoiesis
       Periodic neutropenia
    D70.8 Other neutropenia
    D70.9 Neutropenia, unspecified

## D71 Functional disorders of polymorphonuclear neutrophilsD71 Functional disorders of polymorphonuclear neutrophils

    Includes: cell membrane receptor complex [CR3] defect
       chronic (childhood) granulomatous disease
       congenital dysphagocytosis
       progressive septic granulomatosis

## D72 Other disorders of white blood cellsD72 Other disorders of white blood cells

    Excludes1: basophilia (D72.824)
       immunity disorders (D80-D89)
       neutropenia (D70)
       preleukemia (syndrome) (D46.9)
    D72.0 Genetic anomalies of leukocytes
       Alder (granulation) (granulocyte) anomaly
       Alder syndrome
       Hereditary leukocytic hypersegmentation
       Hereditary leukocytic hyposegmentation
       Hereditary leukomelanopathy
       May-Hegglin (granulation) (granulocyte) anomaly
       May-Hegglin syndrome
       Pelger​Hu?t (granulation) (granulocyte) anomaly
       Pelger-Hu?t syndrome
       Excludes1: Chediak (-Steinbrinck)-Higashi syndrome (E70.330)
    D72.1 Eosinophilia
       Allergic eosinophilia
       Hereditary eosinophilia
       Excludes1: Löffler's syndrome (J82)
       pulmonary eosinophilia (J82)
    D72.8 Other specified disorders of white blood cells
       Excludes1: leukemia (C91-C95)
       D72.81 Decreased white blood cell count
          Excludes1: neutropenia (D70.-)
          D72.810 Lymphocytopenia
             Decreased lymphocytes
          D72.818 Other decreased white blood cell count
             Basophilic leukopenia
             Eosinophilic leukopenia
             Monocytopenia
             Other decreased leukocytes
             Plasmacytopenia
          D72.819 Decreased white blood cell count, unspecified
             Decreased leukocytes, unspecified
             Leukocytopenia, unspecified
             Leukopenia
             Excludes1: malignant leukopenia (D70.9)
          D72.82 Elevated white blood cell count
             Excludes1: eosinophilia (D72.1)
          D72.820 Lymphocytosis (symptomatic)
             Elevated lymphocytes
          D72.821 Monocytosis (symptomatic)
             Excludes1: infectious mononucleosis (B27.-)
          D72.822 Plasmacytosis
          D72.823 Leukemoid reaction
             Basophilic leukemoid reaction
             Leukemoid reaction NOS
             Lymphocytic leukemoid reaction
             Monocytic leukemoid reaction
             Myelocytic leukemoid reaction
             Neutrophilic leukemoid reaction
          D72.824 Basophilia
          D72.825 Bandemia
             Bandemia without diagnosis of specific infection
             Excludes1: ​confirmed infection - code to infection
                leukemia (C91.-, C92.-, C93.-, C94.-, C95.-)
          D72.828 Other elevated white blood cell count
          D72.829 Elevated white blood cell count, unspecified
             Elevated leukocytes, unspecified
             Leukocytosis, unspecified
          D72.89 Other specified disorders of white blood cells
             Abnormality of white blood cells NEC
    D72.9 Disorder of white blood cells, unspecified
       Abnormal leukocyte differential NOS

## D73 Diseases of spleenD73 Diseases of spleen

    D73.0 Hyposplenism
       Atrophy of spleen
       Excludes1: asplenia (congenital) (Q89.01)
          postsurgical absence of spleen (Z90.81)
    D73.1 Hypersplenism
       Excludes1: neutropenic splenomegaly (D73.81)
          primary splenic neutropenia (D73.81)
          splenitis, splenomegaly in late syphilis (A52.79)
          splenitis, splenomegaly in tuberculosis (A18.85)
          splenomegaly NOS (R16.1)
          splenomegaly congenital (Q89.0)
    D73.2 Chronic congestive splenomegaly
    D73.3 Abscess of spleen
    D73.4 Cyst of spleen
    D73.5 Infarction of spleen
       Splenic rupture, nontraumatic
       Torsion of spleen
       Excludes1: rupture of spleen due to Plasmodium vivax malaria (B51.0)
          traumatic rupture of spleen (S36.03-)
    D73.8 Other diseases of spleen
       D73.81 Neutropenic splenomegaly
          Werner-Schultz disease
       D73.89 Other diseases of spleen
          Fibrosis of spleen NOS
          Perisplenitis
          Splenitis NOS
    D73.9 Disease of spleen, unspecified

## D74 MethemoglobinemiaD74 Methemoglobinemia

    D74.0 Congenital methemoglobinemia
       Congenital NADH-methemoglobin reductase deficiency
       Hemoglobin-M [Hb-M] disease
       Methemoglobinemia, ​hereditary
    D74.8 Other methemoglobinemias
       Acquired methemoglobinemia (with sulfhemoglobinemia)
       Toxic methemoglobinemia
    D74.9 Methemoglobinemia, unspecified

## D75 Other and unspecified diseases of blood and blood-forming organsD75 Other and unspecified diseases of blood and blood-forming organs

    Excludes2: acute lymphadenitis (L04.-)
       chronic lymphadenitis (I88.1)
       enlarged lymph nodes (R59.-)
       hypergammaglobulinemia NOS (D89.2)
       lymphadenitis NOS (I88.9)
       mesenteric lymphadenitis (acute) (chronic) (I88.0)
    D75.0 Familial erythrocytosis
       Benign polycythemia
       Familial polycythemia
       Excludes1: hereditary ovalocytosis (D58.1)
    D75.1 Secondary polycythemia
       Acquired polycythemia
       Emotional polycythemia
       Erythrocytosis NOS
       Hypoxemic polycythemia
       Nephrogenous polycythemia
       Polycythemia due to erythropoietin
       Polycythemia due to fall in plasma volume
       Polycythemia due to high altitude
       Polycythemia due to stress
       Polycythemia NOS
       Relative polycythemia
       Excludes1: polycythemia neonatorum (P61.1)
          polycythemia vera (D45)
    D75.8 Other specified diseases of blood and blood-forming organs
       D75.81 Myelofibrosis
          Myelofibrosis NOS
          Secondary myelofibrosis NOS
          Code first the underlying disorder, such as:
             malignant neoplasm of breast (C50.-)
          Use additional code, if applicable, for associated therapy-related myelodysplastic syndrome (D46.-)
          Use additional external cause code, if due to antineoplastic chemotherapy (T45.1-)
          Excludes1: acute myelofibrosis (C94.4-)
             idiopathic myelofibrosis (D47.1)
             leukoerythroblastic anemia (D61.82)
             myelofibrosis with myeloid metaplasia (D47.4)
             myelophthisic ​anemia (D61.82)
             myelophthisis (D61.82)
             primary myelofibrosis (D47.1)
       D75.82 Heparin induced thrombocytopenia (HIT)
       D75.89 Other specified diseases of blood and blood-forming organs
    D75.9 Disease of blood and blood-forming organs, unspecified

## D76 Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissueD76 Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue

       Excludes1: (Abt-) Letterer-Siwe disease (C96.0)
          eosinophilic granuloma (C96.6)
          Hand-Schüller-Christian disease (C96.5)
          histiocytic sarcoma (C96.a)
          histiocytosis X, multifocal (C96.5)
          histiocytosis X, unifocal (C96.6)
          malignant histiocytosis (C96.a)
          Langerhans-cell histiocytosis, multifocal (C96.5)
          Langerhans-cell histiocytosis NOS (C96.6)
          Langerhans-cell histiocytosis, unifocal (C96.6)
          leukemic reticuloendotheliosis or reticulosis (C91.4-)
          lipomelanotic reticuloendotheliosis or reticulosis (I89.8)
    D76.1 Hemophagocytic lymphohistiocytosis
       Familial hemophagocytic reticulosis
       Histiocytoses of mononuclear phagocytes
    D76.2 Hemophagocytic syndrome, infection-associated
       Use additional code to identify infectious agent or disease.
    D76.3 Other histiocytosis syndromes
       Reticulohistiocytoma (giant-cell)
       Sinus histiocytosis with massive lymphadenopathy
       Xanthogranuloma

## D77 Other disorders of blood and blood-forming organs in diseases classified elsewhereD77 Other disorders of blood and blood-forming organs in diseases classified elsewhere

    Code first underlying disease, such as:
       amyloidosis (E85.-)
       congenital early syphilis (A50.0)
       echinococcosis (B67.0-B67.9)
       malaria (B50.0-B54)
       schistosomiasis [bilharziasis] (B65.0-B65.9)
       vitamin C deficiency (E54)
    Excludes1: rupture of spleen due to Plasmodium vivax malaria (B51.0)
       splenitis, splenomegaly in:
       late syphilis (A52.79)
       tuberculosis (A18.85)

# Intraoperative ​and postprocedural complications of the spleen (D78)
## D78 Intraoperative and postprocedural complications of the spleenD78 Intraoperative and postprocedural complications of the spleen

    D78.0 Intraoperative hemorrhage and hematoma of spleen complicating a procedure
       Excludes1: intraoperative hemorrhage and hematoma of spleen due to accidental puncture or laceration during a procedure (D78.1-)
       D78.01 Intraoperative hemorrhage and hematoma of spleen complicating a procedure on the spleen
       D78.02 Intraoperative hemorrhage and hematoma of spleen complicating other procedure
    D78.1 Accidental puncture and laceration of spleen during a procedure
       D78.11 Accidental puncture and laceration of spleen during a procedure on the spleen
       D78.12 Accidental puncture and laceration of spleen during other procedure
    D78.2 Postprocedural hemorrhage and hematoma of spleen following a procedure
       D78.21 Postprocedural hemorrhage and hematoma of spleen following a procedure on the spleen
       D78.22 Postprocedural hemorrhage and hematoma of spleen following other procedure
    D78.8 Other intraoperative and postprocedural complications of spleen
       Use additional code, if applicable, to further specify disorder
       D78.81 Other intraoperative complications of spleen
       D78.89 Other postprocedural complications of spleen

# Certain disorders involving the immune mechanism (D80-D89)Certain disorders involving the immune mechanism (D80-D89)

    Includes: defects in the complement system
       immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
       sarcoidosis
    Excludes1: autoimmune disease (systemic) NOS (M35.9)
       functional disorders of polymorphonuclear neutrophils (D71)
       human immunodeficiency virus [HIV] disease (B20)

## D80 Immunodeficiency with predominantly antibody defectsD80 Immunodeficiency with predominantly antibody defects

    D80.0 Hereditary hypogammaglobulinemia
       Autosomal ​recessive agammaglobulinemia (Swiss type)
       X-linked agammaglobulinemia [Bruton] (with growth hormone deficiency)
    D80.1 Nonfamilial hypogammaglobulinemia
       Agammaglobulinemia with immunoglobulin-bearing B-lymphocytes
       Common variable agammaglobulinemia [CVAgamma]
       Hypogammaglobulinemia NOS
    D80.2 Selective deficiency of immunoglobulin A [IgA]
    D80.3 Selective deficiency of immunoglobulin G [IgG] subclasses
    D80.4 Selective deficiency of immunoglobulin M [IgM]
    D80.5 Immunodeficiency with increased immunoglobulin M [IgM]
    D80.6 Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia
    D80.7 Transient hypogammaglobulinemia of infancy
    D80.8 Other immunodeficiencies with predominantly antibody defects
       Kappa light chain deficiency
    D80.9 Immunodeficiency with predominantly antibody defects, unspecified

## D81 Combined immunodeficienciesD81 Combined immunodeficiencies

    Excludes1: autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
    D81.0 Severe combined immunodeficiency [SCID] with reticular dysgenesis
    D81.1 Severe combined immunodeficiency [SCID] with low T- and B-cell numbers
    D81.2 Severe combined immunodeficiency [SCID] with low or normal B-cell numbers
    D81.3 Adenosine deaminase [ADA] deficiency
    D81.4 Nezelof's syndrome
    D81.5 Purine nucleoside phosphorylase [PNP] deficiency
    D81.6 Major histocompatibility complex class I deficiency
       Bare lymphocyte syndrome
    D81.7 Major histocompatibility complex class II deficiency
    D81.8 Other combined immunodeficiencies
       D81.81 Biotin-dependent carboxylase deficiency
          Multiple carboxylase deficiency
          Excludes1: biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
          D81.810 Biotinidase deficiency
          D81.818 Other biotin-dependent carboxylase deficiency
             Holocarboxylase synthetase deficiency
             Other multiple carboxylase deficiency
          D81.819 Biotin-dependent carboxylase deficiency, unspecified
             Multiple carboxylase deficiency, unspecified
       D81. ​89 Other combined immunodeficiencies
    D81.9 Combined immunodeficiency, unspecified
       Severe combined immunodeficiency disorder [SCID] NOS

## D82 Immunodeficiency associated with other major defectsD82 Immunodeficiency associated with other major defects

    Excludes1: ataxia telangiectasia [Louis-Bar] (G11.3)
    D82.0 Wiskott-Aldrich syndrome
       Immunodeficiency with thrombocytopenia and eczema
    D82.1 Di George's syndrome
       Pharyngeal pouch syndrome
       Thymic alymphoplasia
       Thymic aplasia or hypoplasia with immunodeficiency
    D82.2 Immunodeficiency with short-limbed stature
    D82.3 Immunodeficiency following hereditary defective response to Epstein-Barr virus
       X-linked lymphoproliferative disease
    D82.4 Hyperimmunoglobulin E [IgE] syndrome
    D82.8 Immunodeficiency associated with other specified major defects
    D82.9 Immunodeficiency associated with major defect, unspecified

## D83 Common variable immunodeficiencyD83 Common variable immunodeficiency

    D83.0 Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function
    D83.1 Common variable immunodeficiency with predominant immunoregulatory T-cell disorders
    D83.2 Common variable immunodeficiency with autoantibodies to B- or T-cells
    D83.8 Other common variable immunodeficiencies
    D83.9 Common variable immunodeficiency, unspecified

## D84 Other immunodeficienciesD84 Other immunodeficiencies

    D84.0 Lymphocyte function antigen-1 [LFA-1] defect
    D84.1 Defects in the complement system
       C1 esterase inhibitor [C1-INH] deficiency
    D84.8 Other specified immunodeficiencies
    D84.9 Immunodeficiency, unspecified

## D86 SarcoidosisD86 Sarcoidosis

    D86.0 Sarcoidosis of lung
    D86.1 Sarcoidosis of lymph nodes
    D86.2 Sarcoidosis of lung with sarcoidosis of lymph nodes
    D86.3 Sarcoidosis of skin
    D86.8 Sarcoidosis of other sites
       D86.81 Sarcoid meningitis
       D86.82 Multiple cranial nerve palsies in sarcoidosis
       D86.83 Sarcoid iridocyclitis
       D86. ​84 Sarcoid pyelonephritis
          Tubulo-interstitial nephropathy in sarcoidosis
       D86.85 Sarcoid myocarditis
       D86.86 Sarcoid arthropathy
          Polyarthritis in sarcoidosis
       D86.87 Sarcoid myositis
       D86.89 Sarcoidosis of other sites
          Hepatic granuloma
          Uveoparotid fever [Heerfordt]
    D86.9 Sarcoidosis, unspecified

## D89 Other disorders involving the immune mechanism, not elsewhere classifiedD89 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
       Code first (T36-T50) to identify drug, if drug induced
    D89.8 Other specified disorders involving the immune mechanism, not elsewhere classified
       D89.81 Graft-versus-host disease
          Code first underlying cause, such as:
             complications of transplanted organs and tissue (T86.-)
             complications of blood transfusion (T80.89)
          Use additional 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]
       D89.89 Other specified disorders involving the immune mechanism, not elsewhere classified
          Excludes1: human immunodeficiency virus disease (B20)
    D89.9 Disorder involving the immune mechanism, unspecified
       Immune diseaseNOS