IV. DISEASES OF THE BLOOD AND BLOOD-FORMING ORGANS Excludes: anemia complicating pregnancy or puerperium (648.2) 280 Iron deficiency anemias Anemia: Anemia: asiderotic Kelly-Paterson syndrome hypochromic Plummer-Vinson syndrome due to blood loss (chronic) Sideropenic dysphagia posthaemorrhagic Excludes: acute posthaemorrhagic anemia (285.1) 281 Other deficiency anemias 281.0 Pernicious anemia Anemia: Addison's Biermer's congenital pernicious Congenital intrinsic factor deficiency 281.1 Other vitamin B12 deficiency anemia Anemia: vegan's vitamin B12 deficiency (dietary) due to selective vitamin B12 malabsorption with proteinuria Syndrome: Imerslund's Imerslund-Gr„sbeck 281.2 Folate-deficiency anemia Folate or folic acid deficiency anemia: NOS dietary drug-induced Nutritional megaloblastic anemia Use additional E code, if desired, to identify drug 281.3 Other specified megaloblastic anemias not elsewhere classified 281.4 Protein-deficiency anemia Amino-acid-deficiency anemia 281.8 Anemia associated with other specified nutritional deficiency Scorbutic anemia ICD-9/ch04.TXT - pg. 1 U 281.9 Unspecified Anemia: megaloblastic NOS nutritional NOS simple chronic 282 Hereditary hemolytic anemias 282.0 Hereditary spherocytosis Acholuric (familial) jaundice Congenital spherocytosis Minkowski-Chauffard syndrome 282.1 Hereditary elliptocytosis Elliptocytosis (congenital) Ovalocytosis (congenital) (hereditary) 282.2 Anemias due to disorders of glutathione metabolism Anemia: enzyme-deficiency, drug induced erythrocytic glutathione deficiency glucose-6-phosphate dehydrogenase [G-6-PD] deficiency glutathione-reductase deficiency hemolytic nonspherocytic (hereditary), type I Favism 282.3 Other hemolytic anemias due to enzyme deficiency Anemia: hemolytic nonspherocytic (hereditary), type II hexokinase deficiency pyruvate-kinase [PK] deficiency triose phosphate isomerase deficiency 282.4 Thalassemias Cooley's anemia Mediterranean anemia (with other hemoglobinopathy) Sickle-cell thalassemia Thalassemia (alpha) (beta) (intermedia) (major) (minor) (mixed) (trait) (with other hemoglobinopathy) 282.5 Sickle-cell trait Hb-AS genotype Hemoglobin S [Hb-S] trait Heterozygous: hemoglobin S Hb-S ICD-9/ch04.TXT - pg. 2 Excludes: with other hemoglobinopathy (282.6-) with thalassemia (282.4) 282.6 Sickle-cell anemia Hb-S disease Sickle-cell/Hb-C disease Hb-S/Hb-C disease Sickle-cell/Hb-D disease Hb-S/Hb-D disease Sickle-cell/Hb-E disease Hb-S/Hb-E disease Excludes: sickle-cell thalassemia (282.4) 282.7 Other hemoglobinopathies Abnormal hemoglobin NOS Hemoglobinopathy NOS Congenital Heinz-body anemia Hereditary persistence of fetal Disease: hemoglobin [HPFH] Hb-C Unstable hemoglobin hemolytic disease Hb-D Hb-E Excludes: familial polycythemia (289.6) Hb-M disease (289.7) high-oxygen-affinity hemoglobin (289.0) 282.8 Other Stomatocytosis 282.9 Unspecified Hereditary hemolytic anemia NOS 283 Acquired hemolytic anemias 283.0 Autoimmune hemolytic anemias Autoimmune hemolytic disease (cold type) (warm type) Chronic cold hemagglutinin disease Cold agglutinin disease or hemoglobinuria Hemolytic anemia: cold type (secondary) (symptomatic) warm type (secondary) (symptomatic) Excludes: Evans' syndrome (287.3) hemolytic disease of newborn (773.-) 283.1 Non-autoimmune hemolytic anemias Hemolytic anemia: Hemolytic anemia: mechanical toxic microangiopathic Hemolytic-uraemic syndrome ICD-9/ch04.TXT - pg. 3 Use additional E code, if desired, to identify cause 283.2 Hemoglobinuria due to hemolysis from external causes Hemoglobinuria: from exertion march paroxysmal (cold) (nocturnal) due to other hemolysis Marchiafava-Micheli syndrome Use additional E code, if desired, to identify cause 283.9 Unspecified Acquired hemolytic anemia NOS Chronic idiopathic hemolytic anemia 284 Aplastic anemia 284.0 Constitutional aplastic anemia Aplasia, (pure) red cell: Blackfan-Diamond syndrome congenital Familial hypoplastic anemia of infants Fanconi's anemia primary Pancytopenia with malformations 284.8 Other Aplastic anemia (due to): Pancytopenia (acquired) drugs Red cell aplasia (acquired) infection (adult) (pure) (with thymoma) radiation toxic Use additional E code, if desired, to identify cause 284.9 Unspecified Anemia: Medullary hypoplasia aplastic (idiopathic) NOS hypoplastic NOS 285 Other and unspecified anemias 285.0 Sideroblastic anemia Anemia: Anemia, sideroblastic pyridoxine-responsive primary sidero-achrestic refractory sideroblastic secondary (drug-induced) acquired (due to disease) congenital sex-linked hypochromic hereditary vitamin B6-responsive ICD-9/ch04.TXT - pg. 4 Use additional E code, if desired, to identify cause if drug-induced 285.1 Acute posthemorrhagic anemia 285.8 Other specified anemias Anemia: dyserythropoietic (congenital) dyshematopoietic (congenital) leukoerythroblastic Infantile pseudoleukemia 285.9 Anemia, unspecified 286 Coagulation defects 286.0 Congenital factor VIII disorder Factor VIII (functional) Hemophilia: deficiency NOS A Excludes: factor VIII deficiency with vascular defect (286.4) 286.1 Congenital factor IX disorder Christmas disease Hemophilia B Deficiency: factor IX (functional) plasma thromboplastin component [PTC] 286.2 Congenital factor XI deficiency Hemophilia C Plasma thromboplastin antecedent [PTA] deficiency 286.3 Congenital deficiency of other clotting factors Congenital afibrinogenemia Deficiency: Deficiency of factor: AC globulin I or fibrinogen proaccelerin II or prothrombin Disease: V or labile Owren's VII or stable Stuart-Prower X or Stuart-Prower Dysfibrinogenemia (congenital) XII or Hageman Hypoproconvertinemia XIII or fibrin stabilizing 286.4 von Willebrand's disease Angiohemophilia Factor VIII deficiency with vascular defect Vascular hemophilia ICD-9/ch04.TXT - pg. 5 Excludes: factor VIII deficiency: NOS (286.0) with functional defect (286.0) hereditary capillary fragility (287.8) 286.5 Hemorrhagic disorder due to circulating anticoagulants Hyperheparinemia Increase in: anti-VIIIa anti-IXa anti-Xa anti-XIa antithrombin Systemic lupus erythematosus inhibitor Use additional E code, if desired, to identify any administered anticoagulant 286.6 Defibrination syndrome Afibrinogenemia, acquired Consumption coagulopathy Diffuse or disseminated intravascular coagulation Fibrinolytic hemorrhage, acquired Purpura: fibrinolytic fulminans Excludes: complicating abortion, pregnancy or the puerperium (634-638 with fourth digit .1, 639.1, 641.3, 666.3) disseminated intravascular coagulation in newborn (776.2) 286.7 Acquired coagulation factor deficiency Deficiency of coagulation factor due to: liver disease vitamin K deficiency Excludes: vitamin K deficiency of newborn (776.0) 286.9 Other and unspecified coagulation defects Defective coagulation NOS Deficiency, coagulation factor NOS Delay, coagulation Disorder: coagulation hemostasis Prolongation of time of: bleeding coagulation ICD-9/ch04.TXT - pg. 6 Excludes: complicating abortion, pregnancy or the puerperium (634-638 with fourth digit .1, 639.1, 641.3, 666.3) 287 Purpura and other hemorrhagic conditions Excludes: hemorrhagic thrombocythemia (238.7) purpura fulminans (286.6) 287.0 Allergic purpura Purpura: anaphylactoid Henoch (-Sch”nlein) nonthrombocytopenic: hemorrhagic idiopathic Vascular purpura Vasculitis, allergic 287.1 Qualitative platelet defects Thrombasthenia (hemorrhagic) (hereditary) Thrombocytopathy Excludes: von Willebrand's disease (286.4) 287.2 Other nonthrombocytopenic purpuras Purpura: NOS senile simplex 287.3 Primary thrombocytopenia Megakaryocytic hypoplasia Thrombocytopenia: Purpura, thrombocytopenic congenital congenital hereditary hereditary primary idiopathic 287.4 Secondary thrombocytopenia Thrombocytopenia (due to): dilutional drugs extracorporeal circulation of blood massive blood transfusion platelet alloimmunization Use additional E code, if desired, to identify cause 287.5 Thrombocytopenia, unspecified ICD-9/ch04.TXT - pg. 7 287.8 Other specified hemorrhagic conditions Capillary fragility (hereditary) Vascular pseudohemophilia 287.9 Unspecified hemorrhagic conditions 288 Diseases of white blood cells 288.0 Agranulocytosis Agranulocytic angina Infantile genetic agranulocytosis Kostmann's syndrome Neutropenia: NOS cyclic drug-induced periodic splenic (primary) toxic Neutropenic splenomegaly Use additional code, if desired, to identify drug 288.1 Functional disorders of neutrophils polymorphonuclears Chronic (childhood) granulomatous disease Congenital dysphagocytosis Progressive septic granulomatosis 288.2 Genetic anomalies of leucocytes Anomaly (granulation) (granulocyte) or syndrome: Alder's Chediak-Steinbrink May-Hegglin Pelger-Hu‰t Hereditary: hypersegmentation hyposegmentation leukomelanopathy 288.3 Eosinophilia Eosinophilia allergic hereditary Excludes: pulmonary eosinophilia (518.3) 288.8 Other ICD-9/ch04.TXT - pg. 8 Leukemoid reaction Lymphocytosis (symptomatic) lymphocytic Lymphopenia monocytic Monocytosis (symptomatic) myelocytic Plasmacytosis Leukocytosis Excludes: immunity disorders (279.-) 288.9 Unspecified 289 Other diseases of blood and blood-forming organs 289.0 Secondary polycythemia Polycythemia: Polycythemia, due to acquired erythropoietin due to: hypoxemic fall in plasma volume nephrogenous high altitude relative emotional stress Excludes: polycythemia neonatorum (776.4) polycythemia vera (238.4) 289.1 Chronic lymphadenitis Chronic: adenitis, any lymph node except mesenteric lymphadenitis, any lymph node except mesenteric Excludes: acute lymphadenitis (683) mesenteric (289.2) enlarged glands NOS (785.6) 289.2 Nonspecific mesenteric lymphadenitis Mesenteric lymphadenitis (acute) (chronic) 289.3 Lymphadenitis, unspecified, except mesenteric 289.4 Hypersplenism 289.5 Other diseases of spleen Atrophy of spleen Chronic congestive splenomegaly Cyst of spleen Fibrosis of spleen: Infarction of spleen NOS Rupture, nontraumatic of spleen Perisplenitis Excludes: splenomegaly NOS (789.2) 289.6 Familial polycythemia ICD-9/ch04.TXT - pg. 9 Familial: benign polycythemia erythrocytosis 289.7 Methemoglobinemia Congenital NADH-methemoglobin-reductase deficiency Hemoglobin M disease Methemoglobinemia: NOS acquired (with sulfhemoglobinemia) hereditary toxic Use additional E code, if desired, to identify cause 289.8 Other 289.9 Unspecified ICD-9/ch04.TXT - pg. 10