4. DISEASES OF THE BLOOD AND BLOOD-FORMING ORGANS (ICD 9 CODES: 280-289)
Includes: anemia:
asiderotic
hypochromic-microcytic
sideropenic
Excludes: familial microcytic anemia (282.49)
Normocytic anemia due to blood loss
Excludes: acute posthemorrhagic anemia (285.1)
Paterson-Kelly syndrome
Plummer-Vinson syndrome
Sideropenic dysphagia
Anemia:
achlorhydric
chlorotic
idiopathic hypochromic
iron [Fe] deficiency NOS
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)
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)
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
Combined B12 and folate-deficiency anemia
Amino-acid-deficiency anemia
Scorbutic anemia
Anemia:
dimorphic
macrocytic
megaloblastic NOS
nutritional NOS
simple chronic
Acholuric (familial) jaundice
Congenital hemolytic anemia (spherocytic)
Congenital spherocytosis
Minkowski-Chauffard syndrome
Spherocytosis (familial)
Excludes: hemolytic anemia of newborn (773.0-773.5)
Elliptocytosis (congenital)
Ovalocytosis (congenital) (hereditary)
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
Anemia:
hemolytic nonspherocytic (hereditary), type II
hexokinase deficiency
pyruvate kinase [PK] deficiency
triosephosphate isomerase deficiency
Excludes: sickle-cell:
disease (282.60-282.69)
trait (282.5)
Thalassemia NOS
Microdrepanocytosis
Sickle-cell thalassemia NOS
Thalassemia Hb-S disease without 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)
Alpha thalassemia major
Hemoglobin H Constant Spring
Hemoglobin H disease
Hydrops fetalis due to alpha thalassemia
Severe alpha thalassemia
Triple gene defect alpha thalassemia
Excludes: alpha thalassemia trait or minor (282.46)
hydrops fetalis due to isoimmunization (773.3)
hydrops fetalis not due to immune hemolysis (778.0)
Beta thalassemia major
Cooley's anemia
Homozygous beta thalassemia
Severe beta thalassemia
Thalassemia intermedia
Thalassemia major
Excludes: beta thalassemia minor (282.46)
beta thalassemia trait (282.46)
delta-beta thalassemia (282.45)
hemoglobin E beta thalassemia (282.47)
sickle-cell beta thalassemia (282.41, 282.42)
Homozygous delta-beta thalassemia
Excludes: delta-beta thalassemia trait (282.46)
Alpha thalassemia minor
Alpha thalassemia silent carrier
Alpha thalassemia trait
Beta thalassemia minor
Beta thalassemia trait
Delta-beta thalassemia trait
Thalassemia trait NOS
Excludes: alpha thalassemia (282.43)
beta thalassemia (282.44)
delta-beta thalassemia (282.45)
hemoglobin E-beta thalassemia (282.47)
sickle-cell trait (282.5)
Excludes: beta thalassemia (282.44)
beta thalassemia minor (282.46)
beta thalassemia trait (282.46)
delta-beta thalassemia (282.45)
delta-beta thalassemia trait (282.46)
hemoglobin E disease (282.7)
other hemoglobinopathies (282.7)
sickle-cell beta thalassemia (282.41, 282.42)
Dominant thalassemia
Hemoglobin C thalassemia
Hereditary leptocytosis
Mediterranean anemia (with other hemoglobinopathy)
Mixed thalassemia
Thalassemia with other hemoglobinopathy
Excludes: hemoglobin C disease (282.7)
hemoglobin E disease (282.7)
other hemoglobinopathies (282.7)
sickle-cell anemias (282.60-282.69)
sickle-cell beta thalassemia (282.41-282.42)
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 (282.41-282.42)
Sickle-cell anemia
Excludes: sickle-cell thalassemia (282.41-282.42)
sickle-cell trait (282.5)
Sickle-cell anemia NOS
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)
Hb-S/Hb-C disease without 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)
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
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)
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 E-beta thalassemia (282.47)
hemoglobin M [Hb-M] disease (289.7)
high-oxygen-affinity hemoglobin (289.0)
other hemoglobinopathies with thalassemia (282.49)
Stomatocytosis
Hereditary hemolytic anemia NOS
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)
Use additional code to identify associated:
E. coli infection (041.41-041.49)
Pneumococcal pnemonia (481)
Shigella dysenteriae (004.0)
Hemolytic anemia:
mechanical
microangiopathic
toxic
Use additional E code to identify cause
Acute intravascular hemolysis
Hemoglobinuria:
from exertion
march
paroxysmal (cold) (nocturnal)
due to other hemolysis
Marchiafava-Micheli syndrome
Use additional E code to identify cause
Acquired hemolytic anemia NOS
Chronic idiopathic hemolytic anemia
Aplasia, (pure) red cell:
congenital
of infants
primary
Blackfan-Diamond syndrome
Familial hypoplastic anemia
Fanconi's anemia
Pancytopenia with malformations
Excludes: pancytopenia (due to) (with):
aplastic anemia NOS (284.9)
bone marrow infiltration (284.2)
constitutional red blood cell aplasia (284.01)
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)
Excludes: aplastic anemia due to antineoplastic chemotherapy (284.89)
Excludes: aplastic anemia due to drugs (284.89)
Leukoerythroblastic anemia
Myelophthisic anemia
Code firstthe 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)
Red cell aplasia NOS
Aplastic anemia (due to):
chronic systemic disease
drugs
infection
radiation
toxic (paralytic)
Use additional E code to identify cause
Anemia:
aplastic (idiopathic) NOS
aregenerative
hypoplastic NOS
nonregenerative
Medullary hypoplasia
Excludes: refractory anemia (238.72)
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
Anemia due to acute blood loss
Excludes: anemia due to chronic blood loss (280.0)
blood loss anemia NOS (280.0)
Anemia in (due to) (with) chronic illness
Anemia in end-stage renal disease
Erythropoietin-resistant anemia (EPO resistant anemia)
Excludes: anemia due to antineoplastic chemotherapy (285.3)
aplastic anemia due to antineoplastic chemotherapy (284.89)
Anemia in other chronic illness
Anemia due to antineoplastic chemotherapy
Excludes: anemia due to drug NEC - code to type of anemia
anemia in neoplastic disease (285.22)
aplastic anemia due to antineoplastic chemotherapy (284.89)
Anemia:
dyserythropoietic (congenital)
dyshematopoietic (congenital)
von Jaksch's
Infantile pseudoleukemia
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)
Antihemophilic globulin [AHG] deficiency
Factor VIII (functional) deficiency
Hemophilia:
NOS
A
classical
familial
hereditary
Subhemophilia
Excludes: factor VIII deficiency with vascular defect (286.4)
Christmas disease
Deficiency:
factor IX (functional)
plasma thromboplastin component [PTC]
Hemophilia B
Hemophilia C
Plasma thromboplastin antecedent [PTA] deficiency
Rosenthal's disease
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
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)
Autoimmune hemophilia
Autoimmune inhibitors to clotting factors
Secondary hemophilia
Lupus anticoagulant (LAC) with hemorrhagic disorder
Systemic lupus erythematosus [SLE] inhibitor with hemorrhagic disorder
Excludes: antiphospholipid antibody, finding without diagnosis (795.79)
antiphospholipid antibody syndrome (289.81)
antiphospholipid antibody with hypercoagulable state (289.81)
lupus anticoagulant (LAC) finding without diagnosis (795.79)
lupus anticoagulant (LAC) with hypercoagulable state (289.81)
systemic lupus erythematosus [SLE] inhibitor finding without diagnosis (795.79)
systemic lupus erythematosus [SLE] inhibitor with hypercoagulable state (289.81)
286.59 Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors
Antithrombinemia
Antithromboplastinemia
Antithromboplastinogenemia
Increase in:
anti-II (prothrombin)
anti-VIIIa
anti-IXa
anti-Xla
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)
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
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)
Excludes: hemorrhagic thrombocythemia (238.79)
purpura fulminans (286.6)
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)
Thrombasthenia (hemorrhagic) (hereditary)
Thrombocytasthenia
Thrombocytopathy (dystrophic)
Thrombopathy (Bernard-Soulier)
Excludes: von Willebrand's disease (286.4)
Purpura:
NOS
senile
simplex
Excludes: thrombotic thrombocytopenic purpura (446.6)
transient thrombocytopenia of newborn (776.1)
Megakaryocytic hypoplasia
Idiopathic thrombocytopenic purpura
Tidal platelet dysgenesis
Congenital and hereditary thrombocytopenia
Thrombocytopenia with absent radii (TAR) syndrome
Excludes: Wiskott-Aldrich syndrome (279.12)
Use additional E code to identify cause
Excludes: heparin-induced thrombocytopenia (HIT) (289.84)
transient thrombocytopenia of newborn (776.1)
Posttransfusion purpura from whole blood (fresh) or blood products
PTP
Thrombocytopenia (due to):
dilutional
drugs
extracorporeal circulation of blood
massive blood transfusion
platelet alloimmunization
secondary NOS
Capillary fragility (hereditary)
Vascular pseudohemophilia
Hemorrhagic diathesis (familial)
Excludes: leukemia (204.0-208.9)
Decreased Absolute Neutrophil Count (ANC)
Use additional code for any associated:
fever (780.61)
mucositis (478.11, 528.00-528.09, 538, 616.81)
Excludes: neutropenic splenomegaly (289.53)
transitory neonatal neutropenia (776.7)
Congenital agranulocytosis
Infantile genetic agranulocytosis
Kostmann's syndrome
Cyclic hematopoiesis
Periodic neutropenia
Use additional E code to identify drug
Agranulocytosis
Neutropenia:
immune
toxic
Chronic (childhood) granulomatous disease
Congenital dysphagocytosis
Job's syndrome
Lipochrome histiocytosis (familial)
Progressive septic granulomatosis
Anomaly (granulation) (granulocyte) or syndrome:
Alder's (-Reilly)
Chédiak-Steinbrinck (-Higashi)
Jordan's
May-Hegglin
Pelger-Huet
Hereditary:
hypersegmentation
hyposegmentation
leukomelanopathy
Eosinophilia
allergic
hereditary
idiopathic
secondary
Eosinophilic leukocytosis
Excludes: Löffler's syndrome (518.3)
pulmonary eosinophilia (518.3)
Familial hemophagocytic lymphohistiocytosis
Familial hemophagocytic reticulosis
Hemophagocytic syndrome, infection-associated
Histiocytic syndromes
Macrophage activation syndrome
Excludes: neutropenia (288.01-288.09)
Decreased leukocytes, unspecified
Decreased white blood cell count, unspecified
Leukopenia NOS
Decreased lymphocytes
Basophilic leukopenia
Eosinophilic leukopenia
Monocytopenia
Plasmacytopenia
Excludes: eosinophilia (288.3)
Elevated leukocytes, unspecified
Elevated white blood cell count, unspecified
Elevated lymphocytes
Basophilic leukemoid reaction
Lymphocytic leukemoid reaction
Monocytic leukemoid reaction
Myelocytic leukemoid reaction
Neutrophilic leukemoid reaction
Excludes: infectious mononucleosis (075)
Bandemia without diagnosis of specific infection
Excludes: confirmed infection - code to infection
leukemia (204.00-208.9)
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)
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)
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)
Mesenteric lymphadenitis (acute) (chronic)
"Big spleen" syndrome
Dyssplenism
Hypersplenia
Excludes: primary splenic neutropenia (289.53)
Code firstsickle-cell disease in crisis (282.42, 282.62, 282.64, 282.69)
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)
Familial:
benign polycythemia
erythrocytosis
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
Activated protein C resistance
Antiphospholipid antibody syndrome
Antithrombin III deficiency
Factor V Leiden mutation
Lupus anticoagulant with hypercoagulable state
Protein C deficiency
Protein S deficiency
Prothrombin gene mutation
Systemic lupus erythematosus [SLE] inhibitor with hypercoagulable state
Excludes: anti-phospholipid antibody, finding without diagnosis (795.79)
anti-phospholipid antibody with hemorrhagic disorder (286.53)
lupus anticoagulant (LAC) finding without diagnosis (795.79)
lupus anticoagulant (LAC) with hemorrhagic disorder (286.53)
secondary activated protein C resistance (289.82)
secondary antiphospholipid antibody syndrome (289.82)
secondary lupus anticoagulant with hypercoagulable state (289.82)
secondary systemic lupus erythematosus [SLE] inhibitor with hypercoagulable state (289.82)
systemic lupus erythematosus [SLE] inhibitor finding without diagnosis (795.79)
systemic lupus erythematosus [SLE] inhibitor with hypercoagulable state (286.53)
Excludes: heparin-induced thrombocytopenia (HIT) (289.84)
Myelofibrosis NOS
Secondary myelofibrosis
Code first the underlying disorder, such as:
malignant neoplasm of breast (174.0-174.9, 175.0-175.9)
Use additional code for associated therapy-related myelodysplastic syndrome, if applicable (238.72, 238.73)
Use additional external cause code if due to anti-neoplastic chemotherapy (E933.1)
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)
Hypergammaglobulinemia
Pseudocholinesterase deficiency
Blood dyscrasia NOS
Erythroid hyperplasia
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