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INTERNATIONAL CLASSIFICATION OF DISEASES

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vertical line Table of Contents Alphabetical Index of Diseases and Injuries Explanation of Disease Classification

4. Diseases of the Blood and Blood-Forming Organs (280-289)




280	Iron deficiency anemias
Includes:	anemia:
asiderotic
hypochromic-microcytic
sideropenic
Excludes:	familial microcytic anemia (282.49)
280.0	Secondary to blood loss (chronic)
Normocytic anemia due to blood loss
Excludes:	acute posthemorrhagic anemia (285.1)
280.1	Secondary to inadequate dietary iron intake
280.8	Other specified iron deficiency anemias
Paterson-Kelly syndrome
Plummer-Vinson syndrome
Sideropenic dysphagia
280.9	Iron deficiency anemia, unspecified
Anemia:
achlorhydric
chlorotic
idiopathic hypochromic
iron [Fe] deficiency NOS
281	Other deficiency anemias
281.0	Pernicious anemia
Anemia:
Addison's
Biermer's
congenital pernicious
Congenital intrinsic factor [Castle's] deficiency
Excludes:	combined system disease without mention of anemia (266.2)
subacute degeneration of spinal cord without mention of anemia (266.2)
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-Gräsbeck
Excludes:	combined system disease without mention of anemia (266.2)
subacute degeneration of spinal cord without mention of anemia (266.2)
281.2	Folate-deficiency anemia
Congenital folate malabsorption
Folate or folic acid deficiency anemia:
NOS
dietary
drug-induced
Goat's milk anemia
Nutritional megaloblastic anemia (of infancy)
Use additional E code to identify drug
281.3	Other specified megaloblastic anemias, not elsewhere classified
Combined B12 and folate-deficiency anemia
281.4	Protein-deficiency anemia
Amino-acid-deficiency anemia
281.8	Anemia associated with other specified nutritional deficiency
Scorbutic anemia
281.9	Unspecified deficiency anemia
Anemia:
dimorphic
macrocytic
megaloblastic NOS
nutritional NOS
simple chronic
282	Hereditary hemolytic anemias
282.0	Hereditary spherocytosis
Acholuric (familial) jaundice
Congenital hemolytic anemia (spherocytic)
Congenital spherocytosis
Minkowski-Chauffard syndrome
Spherocytosis (familial)
Excludes:	hemolytic anemia of newborn (773.0-773.5)
282.1	Hereditary elliptocytosis
Elliptocytosis (congenital)
Ovalocytosis (congenital) (hereditary)
282.2	Anemias due to disorders of glutathione metabolism
Anemia:
6-phosphogluconic dehydrogenase deficiency
enzyme deficiency, drug-induced
erythrocytic glutathione deficiency
glucose-6-phosphate dehydrogenase [G-6-PD] deficiency
glutathione-reductase deficiency
hemolytic nonspherocytic (hereditary), type I
Disorder of pentose phosphate pathway
Favism
282.3	Other hemolytic anemias due to enzyme deficiency
Anemia:
hemolytic nonspherocytic (hereditary), type II
hexokinase deficiency
pyruvate kinase [PK] deficiency
triosephosphate isomerase deficiency
282.4	Thalassemias
Excludes:	sickle-cell:
disease (282.60-282.69)
trait (282.5)
282.41	Sickle-cell thalassemia without crisis
Sickle-cell thalassemia NOS
Thalassemia Hb-S disease without crisis
282.42	Sickle-cell thalassemia with crisis
Sickle-cell thalassemia with vaso-occlusive pain
Thalassemia Hb-S disease with crisis
Use additional code for type of crisis, such as:
Acute chest syndrome (517.3)
Splenic sequestration (289.52)
282.49	Other thalassemia
Cooley's anemia
Hb-Bart's disease
Hereditary leptocytosis
Mediterranean anemia (with other hemoglobinopathy)
Microdrepanocytosis
Thalassemia (alpha) (beta) (intermedia) (major) (minima) (minor) (mixed) (trait) (with
 other hemoglobinopathy)
Thalassemia NOS
282.5	Sickle-cell trait
Hb-AS genotype
Hemoglobin S [Hb-S] trait
Heterozygous:
hemoglobin S
Hb-S
Excludes:	that with other hemoglobinopathy (282.60-282.69)
that with thalassemia (282.49)
282.6	Sickle-cell disease
Sickle-cell anemia
Excludes:	sickle-cell thalassemia (282.41-282.42)
sickle-cell trait (282.5)
282.60	Sickle-cell disease, unspecified
Sickle-cell anemia NOS
282.61	Hb-SS disease without crisis
282.62	Hb-SS disease with crisis
Hb-SS disease with vaso-occlusive pain
Sickle-cell crisis NOS
Use additional code for type of crisis, such as:
Acute chest syndrome (517.3)
Splenic sequestration (289.52)
282.63	Sickle-cell/Hb-C disease without crisis
Hb-S/Hb-C disease without crisis
282.64	Sickle-cell/HB-C disease with crisis
Hb-S/Hb-C disease with crisis
Sickle-cell/Hb-C disease with vaso-occlusive pain
Use additional code for types of crisis, such as:
Acute chest syndrome (517.3)
Splenic sequestration (289.52)
282.68	Other sickle-cell disease without crisis
Hb-S/Hb-D disease without crisis
Hb-S/Hb-E disease without crisis
Sickle-cell/Hb-D disease without crisis
Sickle-cell/Hb-E disease without crisis
282.69	Other sickle-cell disease with crisis
Hb-S/Hb-D disease with crisis
Hb-S/Hb-E disease with crisis
Sickle-cell/Hb-D disease with crisis
Sickle-cell/Hb-E disease with crisis
Other sickle-cell disease with vaso-occlusive pain
Use additional code for type of crisis, such as:
Acute chest syndrome (517.3)
Splenic sequestration (289.52)
282.7	Other hemoglobinopathies
Abnormal hemoglobin NOS
Congenital Heinz-body anemia
Disease:
hemoglobin C [Hb-C]
hemoglobin D [Hb-D]
hemoglobin E [Hb-E]
hemoglobin Zurich [Hb-Zurich]
Hemoglobinopathy NOS
Hereditary persistence of fetal hemoglobin [HPFH]
Unstable hemoglobin hemolytic disease
Excludes:	familial polycythemia (289.6)
hemoglobin M [Hb-M] disease (289.7)
high-oxygen-affinity hemoglobin (289.0)
282.8	Other specified hereditary hemolytic anemias
Stomatocytosis
282.9	Hereditary hemolytic anemia, 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)
drug-induced
warm type (secondary) (symptomatic)
Use additional E code to identify cause,  if drug-induced
Excludes:	Evans' syndrome (287.32)
hemolytic disease of newborn (773.0-773.5)
283.1	Non-autoimmune hemolytic anemias
283.10	Non-autoimmune hemolytic anemia, unspecified
283.11	Hemolytic-uremic syndrome
283.19	Other non-autoimmune hemolytic anemias
Hemolytic anemia:
mechanical
microangiopathic
toxic
Use additional E code to identify cause
283.2	Hemoglobinuria due to hemolysis from external causes
Acute intravascular hemolysis
Hemoglobinuria:
from exertion
march
paroxysmal (cold) (nocturnal)
due to other hemolysis
Marchiafava-Micheli syndrome
Use additional E code to identify cause
283.9	Acquired hemolytic anemia, unspecified
Acquired hemolytic anemia NOS
Chronic idiopathic hemolytic anemia
284	Aplastic anemia and other bone marrow failure syndromes
284.0	Constitutional aplastic anemia
284.01	Constitutional red blood cell aplasia
Aplasia, (pure) red cell:
congenital
of infants
primary
Blackfan-Diamond syndrome
Familial hypoplastic anemia
284.09	Other constitutional aplastic anemia
Fanconi's anemia
Pancytopenia with malformations
284.1	Pancytopenia
Excludes:	pancytopenia (due to) (with):
aplastic anemia NOS (284.9)
bone marrow infiltration (284.2)
constitutional red blood cell aplasia (284.01)
drug induced (284.89)
hairy cell leukemia (202.4)
human immunodeficiency virus disease (042)
leukoerythroblastic anemia (284.2)
malformations (284.09)
myelodysplastic syndromes (238.72-238.75)
myeloproliferative disease (238.79)
other constitutional aplastic anemia (284.09)
284.2	Myelophthisis
Leukoerythroblastic anemia
Myelophthisic anemia
Code first the underlying disorder, such as:
malignant neoplasm of breast (174.0-174.9, 175.0-175.9)
tuberculosis (015.0-015.9)
Excludes:	idiopathic myelofibrosis (238.76)
myelofibrosis NOS (289.83)
myelofibrosis with myeloid metaplasia (238.76)
primary myelofibrosis (238.76)
secondary myelofibrosis (289.83)
284.8	Other specified aplastic anemias
284.81	Red cell aplasia (acquired) (adult) (with thymoma)
Red cell aplasia NOS
284.89	Other specified aplastic anemias
Aplastic anemia (due to):
chronic systemic disease
drugs
infection
radiation
toxic (paralytic)
Use additional E code to identify cause
284.9	Aplastic anemia, unspecified
Anemia:
aplastic (idiopathic) NOS
aregenerative
hypoplastic NOS
nonregenerative
Medullary hypoplasia
Excludes:	refractory anemia (238.72)
285	Other and unspecified anemias
285.0	Sideroblastic anemia
Anemia:
hypochromic with iron loading
sideroachrestic
sideroblastic:
acquired
congenital
hereditary
primary
secondary (drug-induced) (due to disease)
sex-linked hypochromic
vitamin B6-responsive
Pyridoxine-responsive (hypochromic) anemia
Excludes:	refractory sideroblastic anemia (238.72)
Use additional E code to identify cause, if drug-induced
285.1	Acute posthemorrhagic anemia
Anemia due to acute blood loss
Excludes:	anemia due to chronic blood loss (280.0)
blood loss anemia NOS (280.0)
285.2	Anemia of chronic disease
Anemia in chronic illness
285.21	Anemia in chronic kidney disease
Anemia in end-stage renal disease
Erythropoietin-resistant anemia (EPO resistant anemia)
285.22	Anemia in neoplastic disease
285.29	Anemia of other chronic disease
Anemia in other chronic illness
285.8	Other specified anemias
Anemia:
dyserythropoietic (congenital)
dyshematopoietic (congenital)
von Jaksch's
Infantile pseudoleukemia
285.9	Anemia, unspecified
Anemia:
NOS
essential
normocytic, not due to blood loss
profound
progressive
secondary
Oligocythemia
Excludes:	anemia (due to):
blood loss:
acute (285.1)
chronic or unspecified (280.0)
iron deficiency (280.0-280.9)
286	Coagulation defects
286.0	Congenital factor VIII disorder
Antihemophilic globulin [AHG] deficiency
Factor VIII (functional) deficiency
Hemophilia:
NOS
A
classical
familial
hereditary
Subhemophilia
Excludes:	factor VIII deficiency with vascular defect (286.4)
286.1	Congenital factor IX disorder
Christmas disease
Deficiency:
factor IX (functional)
plasma thromboplastin component [PTC]
Hemophilia B
286.2	Congenital factor XI deficiency
Hemophilia C
Plasma thromboplastin antecedent [PTA] deficiency
Rosenthal's disease
286.3	Congenital deficiency of other clotting factors
Congenital afibrinogenemia
Deficiency:
AC globulin
factor:
I [fibrinogen]
II [prothrombin]
V [labile]
VII [stable]
X [Stuart-Prower]
XII [Hageman]
XIII [fibrin stabilizing]
Laki-Lorand factor
proaccelerin
Disease:
Owren's
Stuart-Prower
Dysfibrinogenemia (congenital)
Dysprothrombinemia (constitutional)
Hypoproconvertinemia
Hypoprothrombinemia (hereditary)
Parahemophilia
286.4	von Willebrand's disease
Angiohemophilia (A) (B)
Constitutional thrombopathy
Factor VIII deficiency with vascular defect
Pseudohemophilia type B
Vascular hemophilia
von Willebrand's (-Jürgens') disease
Excludes:	factor VIII deficiency:
NOS (286.0)
with functional defect (286.0)
hereditary capillary fragility (287.8)
286.5	Hemorrhagic disorder due to intrinsic circulating anticoagulants
Antithrombinemia
Antithromboplastinemia
Antithromboplastino-genemia
Hyperheparinemia
Increase in:
anti-VIIIa
anti-IXa
anti-Xa
anti-XIa
antithrombin
Secondary hemophilia
Systemic lupus erythematosus [SLE] inhibitor
286.6	Defibrination syndrome
Afibrinogenemia, acquired
Consumption coagulopathy
Diffuse or disseminated intravascular coagulation [DIC syndrome]
Fibrinolytic hemorrhage, acquired
Hemorrhagic fibrinogenolysis
Pathologic fibrinolysis
Purpura:
fibrinolytic
fulminans
Excludes:	that complicating:
abortion (634-638 with .1, 639.1)
pregnancy or the puerperium (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
Hypoprothrombinemia, acquired
Excludes:	vitamin K deficiency of newborn (776.0)
Use additional E-code to identify cause, if drug-induced
286.9	Other and unspecified coagulation defects
Defective coagulation NOS
Deficiency, coagulation factor NOS
Delay, coagulation
Disorder:
coagulation
hemostasis
Excludes:	abnormal coagulation profile (790.92)
hemorrhagic disease of newborn (776.0)
that complicating:
abortion (634-638 with .1, 639.1)
pregnancy or the puerperium (641.3, 666.3)
287	Purpura and other hemorrhagic conditions
Excludes:	hemorrhagic thrombocythemia (238.79)
purpura fulminans (286.6)
287.0	Allergic purpura
Peliosis rheumatica
Purpura:
anaphylactoid
autoimmune
Henoch's
nonthrombocytopenic:
hemorrhagic
idiopathic
rheumatica
Schönlein-Henoch
vascular
Vasculitis, allergic
Excludes:	hemorrhagic purpura (287.39)
purpura annularis telangiectodes (709.1)
287.1	Qualitative platelet defects
Thrombasthenia (hemorrhagic) (hereditary)
Thrombocytasthenia
Thrombocytopathy (dystrophic)
Thrombopathy (Bernard-Soulier)
Excludes:	von Willebrand's disease (286.4)
287.2	Other nonthrombocytopenic purpuras
Purpura:
NOS
senile
simplex
287.3	Primary thrombocytopenia
Excludes:	thrombotic thrombocytopenic purpura (446.6)
transient thrombocytopenia of newborn (776.1)
287.30	Primary thrombocytopenia unspecified
Megakaryocytic hypoplasia
287.31	Immune thrombocytopenic purpura
Idiopathic thrombocytopenic purpura
Tidal platelet dysgenesis
287.32	Evans' syndrome
287.33	Congenital and hereditary thrombocytopenic purpura
Congenital and hereditary thrombocytopenia
Thrombocytopenia with absent radii (TAR) syndrome
Excludes:	Wiskott-Aldrich syndrome (279.12)
287.39	Other primary thrombocytopenia
287.4	Secondary thrombocytopenia
Posttransfusion purpura
Thrombocytopenia (due to):
dilutional
drugs
extracorporeal circulation of blood
massive blood transfusion
platelet alloimmunization
Use additional E code to identify cause
Excludes:	transient thrombocytopenia of newborn (776.1)
287.5	Thrombocytopenia, unspecified
287.8	Other specified hemorrhagic conditions
Capillary fragility (hereditary)
Vascular pseudohemophilia
287.9	Unspecified hemorrhagic conditions
Hemorrhagic diathesis (familial)
288	Diseases of white blood cells
Excludes:	leukemia (204.0-208.9)
288.0	Neutropenia
Decreased Absolute Neutrophil Count (ANC)
Use additional code for any associated:
fever (780.6)
mucositis (478.11, 528.00-528.09, 538, 616.81)
Excludes:	neutropenic splenomegaly (289.53)
transitory neonatal neutropenia (776.7)
288.00	Neutropenia, unspecified
288.01	Congenital neutropenia
Congenital agranulocytosis
Infantile genetic agranulocytosis
Kostmann's syndrome
288.02	Cyclic neutropenia
Cyclic hematopoiesis
Periodic neutropenia
288.03	Drug induced neutropenia
Use additional E code to identify drug
288.04	Neutropenia due to infection
288.09	Other neutropenia
Agranulocytosis
Neutropenia:
immune
toxic
288.1	Functional disorders of polymorphonuclear neutrophils
Chronic (childhood) granulomatous disease
Congenital dysphagocytosis
Job's syndrome
Lipochrome histiocytosis (familial)
Progressive septic granulomatosis
288.2	Genetic anomalies of leukocytes
Anomaly (granulation) (granulocyte) or syndrome:
Alder's (-Reilly)
Chédiak-Steinbrinck (-Higashi)
Jordan's
May-Hegglin
Pelger-Huet
Hereditary:
hypersegmentation
hyposegmentation
leukomelanopathy
288.3	Eosinophilia
Eosinophilia
allergic
hereditary
idiopathic
secondary
Eosinophilic leukocytosis
Excludes:	Löffler's syndrome (518.3)
pulmonary eosinophilia (518.3)
288.4	Hemophagocytic syndromes
Familial hemophagocytic lymphohistiocytosis
Familial hemophagocytic reticulosis
Hemophagocytic syndrome, infection-associated
Histiocytic syndromes
Macrophage activation syndrome
288.5	Decreased white blood cell count
Excludes:	neutropenia (288.01-288.09)
288.50	Leukocytopenia, unspecified
Decreased leukocytes, unspecified
Decreased white blood cell count, unspecified
Leukopenia NOS
288.51	Lymphocytopenia 
Decreased lymphocytes
288.59	Other decreased white blood cell count
Basophilic leukopenia
Eosinophilic leukopenia
Monocytopenia
Plasmacytopenia
288.6	Elevated white blood cell count
Excludes:	eosinophilia (288.3)
288.60	Leukocytosis, unspecified
Elevated leukocytes, unspecified
Elevated white blood cell count, unspecified
288.61	Lymphocytosis (symptomatic)
Elevated lymphocytes
288.62	Leukemoid reaction
Basophilic leukemoid reaction
Lymphocytic leukemoid reaction
Monocytic leukemoid reaction
Myelocytic leukemoid reaction
Neutrophilic leukemoid reaction
288.63	Monocytosis (symptomatic)
Excludes:	infectious mononucleosis (075)
288.64	Plasmacytosis
288.65	Basophilia
288.66	Bandemia
Bandemia without diagnosis of specific infection
Excludes:	confirmed infection - code to infection
leukemia  (204.00-208.9)
288.69	Other elevated white blood cell count
288.8	Other specified disease of white blood cells
Excludes:	decreased white blood cell counts (288.50-288.59)
elevated white blood cell counts (288.60-288.69)
immunity disorders (279.0-279.9)
288.9	Unspecified disease of white blood cells
289	Other diseases of blood and blood-forming organs
289.0	Polycythemia, secondary
High-oxygen-affinity hemoglobin
Polycythemia:
acquired
benign
due to:
fall in plasma volume
high altitude
emotional
erythropoietin
hypoxemic
nephrogenous
relative
spurious
stress
Excludes:	polycythemia:
neonatal (776.4)
primary (238.4)
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
"Big spleen" syndrome
Dyssplenism
Hypersplenia
Excludes:	primary splenic neutropenia (289.53)
289.5	Other diseases of spleen
289.50	Disease of spleen, unspecified
289.51	Chronic congestive splenomegaly
289.52	Splenic sequestration
Code first sickle-cell disease in crisis (282.42, 282.62, 282.64, 282.69)
289.53	Neutropenic splenomegaly
289.59	Other
Lien migrans
Perisplenitis
Splenic:
abscess
atrophy
cyst
fibrosis
infarction
rupture, nontraumatic
Splenitis
Wandering spleen
Excludes:	bilharzial splenic fibrosis (120.0-120.9)
hepatolienal fibrosis (571.5)
splenomegaly NOS (789.2)
289.6	Familial polycythemia
Familial:
benign polycythemia
erythrocytosis
289.7	Methemoglobinemia
Congenital NADH [DPNH]-methemoglobin-reductase deficiency
Hemoglobin M [Hb-M] disease
Methemoglobinemia:
NOS
acquired (with sulfhemoglobinemia)
hereditary
toxic
Stokvis' disease
Sulfhemoglobinemia
Use additional E code to identify cause
289.8	Other specified diseases of blood and blood-forming organs
289.81	Primary hypercoagulable state
Activated protein C resistance
Antithrombin III deficiency
Factor V Leiden mutation
Lupus anticoagulant
Protein C deficiency
Protein S deficiency
Prothrombin gene mutation
289.82	Secondary hypercoagulable state
289.83	Myelofibrosis
Myelofibrosis NOS
Secondary myelofibrosis
Code first the underlying disorder, such as:
malignant neoplasm of breast (174.0-174.9, 175.0-175.9)
Excludes:	idiopathic myelofibrosis (238.76)
leukoerythroblastic anemia (284.2)
myelofibrosis with myeloid metaplasia (238.76)
myelophthisic anemia (284.2)
myelophthisis (284.2)
primary myelofibrosis (238.76)
289.89	Other specified diseases of blood and blood-forming organs
Hypergammaglobulinemia
Pseudocholinesterase deficiency
289.9	Unspecified diseases of blood and blood-forming organs
Blood dyscrasia NOS
Erythroid hyperplasia













TABLE OF CONTENTS

Preface

Acknowledgments

Introduction

The ICD-9-CM Coordination and Maintenance Committee

Characteristics of the ICD-9-CM

The Disease Classification

Alphabetical Index of Diseases

Table of Drugs and Chemicals

Index To External Causes of Injury (E Code)

Classification of Procedures

Index to Procedures

Appendix A:
Morphology of Neoplasms


Appendix C:
Classification of Drugs by American Hospital Formulary Services List Number and their ICD-9-CM Equivalents


Appendix D:
Classification of Industrial Accidents According to Agency


Appendix E:
List of Three-Digit Categories






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