DISEASES OF THE BLOOD AND BLOOD-FORMING ORGANS (280-289)

Iron deficiency anemias
Other deficiency anemias
Hereditary hemolytic anemias
Acquired hemolytic anemias
Aplastic anemia
Other and unspecified anemias
Coagulation defects
Purpura and other hemorrhagic conditions
Diseases of white blood cells
Other diseases of blood and blood-forming organs

280 Iron deficiency anemias


Includes: anemia:
asiderotic
hypochromic-microcytic
sideropenic
Excludes: familial microcytic anemia (282.4)

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
Refractory megaloblastic 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

Cooley's anemia
Hereditary leptocytosis
Mediterranean anemia (with other hemoglobinopathy)
Microdrepanocytosis
Sickle-cell thalassemia
Thalassemia (alpha) (beta) (intermedia) (major) (minima) (minor) (mixed) (trait) (with other hemoglobinopathy)
Thalassemia-Hb-S disease
Excludes: sickle-cell:
anemia (282.60-282.69)
trait (282.5)

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 (282.4)

282.6 Sickle-cell anemia

Excludes: sickle-cell thalassemia (282.4)
sickle-cell trait (282.5)
282.60 Sickle-cell anemia, unspecified
282.61 Hb-S disease without mention of crisis
282.62 Hb-S disease with mention of crisis
Sickle-cell crisis NOS
282.63 Sickle-cell/Hb-C disease
Hb-S/Hb-C disease
282.69 Other
Disease:
Hb-S/Hb-D
Hb-S/Hb-E
sickle-cell/Hb-D
sickle-cell/Hb-E

282.7 Other hemoglobinopathies

Abnormal hemoglobin NOS
Congenital Heinz-body anemia
Disease:
Hb-Bart's
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 (287.3)
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


284.0 Constitutional aplastic anemia

Aplasia, (pure) red cell:
congenital
of infants
primary
Blackfan-Diamond syndrome
Familial hypoplastic anemia
Fanconi's anemia
Pancytopenia with malformations

284.8 Other specified aplastic anemias

Aplastic anemia (due to):
chronic systemic disease
drugs
infection
radiation
toxic (paralytic)
Pancytopenia (acquired)
Red cell aplasia (acquired) (adult) (pure) (with thymoma)
Use additional E code to identify cause

284.9 Aplastic anemia, unspecified

Anemia:
aplastic (idiopathic) NOS
aregenerative
hypoplastic NOS
nonregenerative
refractory
Medullary hypoplasia

285 Other and unspecified anemias


285.0 Sideroblastic anemia

Anemia:
hypochromic with iron loading
sideroachrestic
sideroblastic
acquired
congenital
hereditary
primary
refractory
secondary (drug-induced) (due to disease)
sex-linked hypochromic
vitamin B6-responsive
Pyridoxine-responsive (hypochromic) anemia
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 in chronic illness

285.21 Anemia in end-stage renal disease
285.22 Anemia in neoplastic disease
285.29 Anemia of other chronic illness

285.8 Other specified anemias

Anemia:
dyserythropoietic (congenital)
dyshematopoietic (congenital)
leukoerythroblastic
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 circulating anticoagulants

Antithrombinemia
Antithromboplastinemia
Antithromboplastino-genemia
Hyperheparinemia
Increase in:
anti-VIIIa
anti-IXa
anti-Xa
anti-XIa
antithrombin
Systemic lupus erythematosus [SLE] inhibitor
Use additional E code to identify cause, if drug-induced

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 (238.7)
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 (287.3)
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

Evans' syndrome
Megakaryocytic hypoplasia
Purpura, thrombocytopenic
congenital
hereditary
idiopathic
Thrombocytopenia:
congenital
hereditary
primary
Tidal platelet dysgenesis
Excludes: thrombotic thrombocytopenic purpura (446.6)
transient thrombocytopenia of newborn (776.1)

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 Agranulocytosis

Infantile genetic agranulocytosis
Kostmann's syndrome
Neutropenia:
NOS
cyclic
drug-induced
immune
periodic
toxic
Neutropenic splenomegaly
Use additional E code to identify drug or other cause
Excludes: transitory neonatal neutropenia (776.7)

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)

288.8 Other specified disease of white blood cells

Leukemoid reaction
lymphocytic
monocytic
myelocytic
Leukocytosis
Lymphocytopenia
Lymphocytosis (symptomatic)
Lymphopenia
Monocytosis (symptomatic)
Plasmacytosis
Excludes: 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 (288.0)

289.5 Other diseases of spleen

289.50 Disease of spleen, unspecified
289.51 Chronic congestive 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

Hypergammaglobulinemia
Myelofibrosis
Pseudocholinesterase deficiency

289.9 Unspecified diseases of blood and blood-forming organs

Blood dyscrasia NOS
Erythroid hyperplasia