ICD-10 Chapter III: Diseases of the blood and blood-forming organs, and certain disorders involving the immune mechanism

International Statistical Classification of Diseases and Related Health Problems 10th Revision
Chapter	Blocks	Title
I	A00–B99	Certain infectious and parasitic diseases
II	C00–D48	Neoplasms
III	D50–D89	Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
IV	E00–E90	Endocrine, nutritional and metabolic diseases
V	F00–F99	Mental and behavioural disorders
VI	G00–G99	Diseases of the nervous system
VII	H00–H59	Diseases of the eye and adnexa
VIII	H60–H95	Diseases of the ear and mastoid process
IX	I00–I99	Diseases of the circulatory system
X	J00–J99	Diseases of the respiratory system
XI	K00–K93	Diseases of the digestive system
XII	L00–L99	Diseases of the skin and subcutaneous tissue
XIII	M00–M99	Diseases of the musculoskeletal system and connective tissue
XIV	N00–N99	Diseases of the genitourinary system
XV	O00–O99	Pregnancy, childbirth and the puerperium
XVI	P00–P96	Certain conditions originating in the perinatal period
XVII	Q00–Q99	Congenital malformations, deformations and chromosomal abnormalities
XVIII	R00–R99	Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified
XIX	S00–T98	Injury, poisoning and certain other consequences of external causes
XX	V01–Y98	External causes of morbidity and mortality
XXI	Z00–Z99	Factors influencing health status and contact with health services
XXII	U00–U99	Codes for special purposes

The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO).^[1] This page contains ICD-10 Chapter III: 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

(D65–D69) Coagulation defects, purpura and other haemorrhagic conditions

(D65) Disseminated intravascular coagulation (defibrination syndrome)
- Afibrinogenaemia, acquired
- Consumption coagulopathy
- Diffuse or disseminated intravascular coagulation (DIC)
- Fibrinolytic haemorrhage, acquired
- Fibrinolytic purpura
- Purpura fulminans
(D66) Hereditary factor VIII deficiency
- Haemophilia A
(D67) Hereditary factor IX deficiency
- Christmas disease
- Haemophilia B
(D68) Other coagulation defects
- (D68.0) Von Willebrand's disease
- (D68.1) Hereditary factor XI deficiency
  - Haemophilia C
- (D68.2) Hereditary deficiency of other clotting factors
- (D68.3) Haemorrhagic disorder due to circulating anticoagulants
- (D68.4) Acquired coagulation factor deficiency
- (D68.8) Other specified coagulation defects
- (D68.9) Coagulation defect, unspecified
(D69) Purpura and other haemorrhagic conditions
- (D69.0) Allergic purpura
  - anaphylactoid purpura
  - Henoch-Schönlein purpura
- (D69.1) Qualitative platelet defects
  - Bernard-Soulier syndrome (giant platelet)
  - Glanzmann's disease
  - Grey platelet syndrome
  - Thromboasthenia (haemorrhagic)(hereditary)
  - Thrombocytopathy
- (D69.2) Other nonthrombocytopenic purpura
- (D69.3) Idiopathic thrombocytopenic purpura
  - Evans' syndrome
- (D69.4) Other primary thrombocytopenia
- (D69.5) Secondary thrombocytopenia
- (D69.6) Thrombocytopenia, unspecified
- (D69.8) Other specified haemorrhagic conditions
- (D69.9) Haemorrhagic condition, unspecified

(D70–D77) Other diseases of blood and blood-forming organs

(D70) Agranulocytosis
- Agranulocytic angina
- Infantile genetic agranulocytosis
- Kostmann's disease
- Neutropenia, NOS
(D71) Functional disorders of polymorphonuclear neutrophils
- Cell membrane receptor complex (CR3) defect
- Chronic (childhood) granulomatous disease
- Congenital dysphagocytosis
- Progressive septic granulomatosis
(D72) Other disorders of white blood cells
- (D72.0) Genetic anomalies of leukocytes
  - Alder anomaly
  - May-Hegglin anomaly
  - Pelger-Huët anomaly
- (D72.1) Eosinophilia
- (D72.8) Other specified disorders of white blood cells
  - Leukaemoid reaction: lymphocytic, monocytic, myelocytic
  - Leukocytosis
  - Lymphocytosis (symptomatic)
  - Lymphopenia
  - Monocytosis (symptomatic)
  - Plasmacytosis
- (D72.9) Disorder of white blood cells, unspecified
(D73) Diseases of spleen
- (D73.0) Hyposplenism
- (D73.1) Hypersplenism
- (D73.2) Chronic congestive splenomegaly
- (D73.3) Abscess of spleen
- (D73.4) Cyst of spleen
- (D73.5) Infarction of spleen
- (D73.8) Other diseases of spleen
- (D73.9) Disease of spleen, unspecified
(D74) Methaemoglobinaemia
- (D74.0) Congenital methaemoglobinaemia
  - Congenital NADH-methaemoglobin reductase deficiency
  - Haemoglobin-M (Hb-M) disease
  - Methaemoglobinaemia, hereditary
- (D74.8) Other methaemoglobinaemias
  - Acquired methaemoglobinaemia (with sulfhaemoglobinaemia)
  - Toxic methaemoglobinaemia
- (D74.9) Methaemoglobinaemia, unspecified
(D75) Other diseases of blood and blood-forming organs
- (D75.0) Familial erythrocytosis
- (D75.1) Secondary polycythaemia
- (D75.2) Essential thrombocytosis
- (D75.8) Other specified diseases of blood and blood-forming organs
  - Basophilia
- (D75.9) Disease of blood and blood-forming organs, unspecified
(D76) Certain diseases involving lymphoreticular tissue and reticulohistiocytic system
- (D76.0) Langerhans' cell histiocytosis, not elsewhere classified
  - Eosinophilic granuloma
  - Hand-Schüller-Christian disease
  - Histiocytosis X (chronic)
- (D76.1) Haemophagocytic lymphohistiocytosis
  - Familial haemophagocytic reticulosis
- (D76.2) Haemophagocytic syndrome, infection-associated
- (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 elsewhere

(D80–D89) Certain disorders involving the immune mechanism

(D80) Immunodeficiency with predominantly antibody defects
- (D80.0) Hereditary hypogammaglobulinaemia
  - Autosomal recessive agammaglobulinaemia (Swiss type)
  - X-linked agammaglobulinaemia (Bruton) (with growth hormone deficiency)
- (D80.1) Nonfamilial hypogammaglobulinaemia
  - Agammaglobulinaemia with immunoglobulin-bearing B-lymphocytes
  - Common variable agammaglobulinaemia (CVAgamma)
  - Hypogammaglobulinaemia 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 hyperimmunoglobulinaemia
- (D80.7) Transient hypogammaglobulinaemia of infancy
- (D80.8) Other immunodeficiencies with predominantly antibody defects
  - Kappa light chain deficiency
- (D80.9) Immunodeficiency with predominantly antibody defects, unspecified
(D81) Combined immunodeficiencies
- (D81.0) Severe combined immunodeficiency (SCID) with reticular dysgenesis
- (D81.1) Severe combined immunodeficiency (SCID) with low T-cell and B-cell numbers
- (D81.2) Severe combined immunodeficiency (SCID) with low or normal B-cell numbers
- (D81.3) Adenosine deaminase deficiency (ADA)
- (D81.4) Nezelof's syndrome
- (D81.5) Purine nucleoside phosphorylase deficiency (PNP)
- (D81.6) Major histocompatibility complex class I deficiency
  - Bare lymphocyte syndrome
- (D81.7) Major histocompatibility complex class II deficiency
- (D81.8) Other combined immunodeficiencies
  - Biotin-dependent carboxylase deficiency
- (D81.9) Combined immunodeficiency, unspecified
  - Severe combined immunodeficiency disorder (SCID) NOS
(D82) Immunodeficiency associated with other major defects
- (D82.0) Wiskott-Aldrich syndrome
  - Immunodeficiency with thrombocytopenia and eczema
- (D82.1) Di George's syndrome
- (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 syndrome (IgE)
(D83) Common variable immunodeficiency
(D84) Other immunodeficiencies
- (D84.0) Lymphocyte function antigen-1 (LFA-1) defect
- (D84.1) Defects in the complement system
  - C1 esterase inhibitor deficiency (C1-INH)
- (D84.8) Other specified immunodeficiencies
- (D84.9) Immunodeficiency, unspecified
(D86) 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 and combined sites
(D89) Other disorders involving the immune mechanism, not elsewhere classified
- (D89.0) Polyclonal hypergammaglobulinaemia
  - Benign hypergammaglobulinaemic purpura
  - Polyclonal gammopathy NOS
- (D89.1) Cryoglobulinaemia
- (D89.2) Hypergammaglobulinaemia, unspecified
- (D89.8) Other specified disorders involving the immune mechanism, not elsewhere classified
- (D89.9) Disorder involving the immune mechanism, unspecified

Excludes

Autoimmune disease (systemic) NOS (M35.9)
Certain conditions originating in the perinatal period (P00-P96)
Complications of pregnancy, childbirth and the puerperium (O00-O9A)
Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
Endocrine, nutritional, and metabolic diseases (E00-E88)
Human immunodeficiency virus [HIV] disease (B20)
Injury, poisoning and certain other consequences of external causes (S00-T88)
Neoplasms (C00-D49)
Symptoms, signs, and abnormal clinical and laboratory findings, NEC (R00-R94)

References

↑ WHO | International Classification of Diseases (ICD)

Diseases of red blood cells (D50–69,74, 280–287)

Red
blood cells

↑

Polycythemia	Polycythemia vera

↓

Anemia

Nutritional

Micro-: Iron-deficiency anemia
- Plummer–Vinson syndrome

Macro-: Megaloblastic anemia
- Pernicious anemia

Hemolytic
(mostly normo-)

Hereditary	enzymopathy: G6PD glycolysis PK TI HK hemoglobinopathy: Thalassemia alpha beta delta Sickle-cell disease/trait HPFH membrane: Hereditary spherocytosis Minkowski–Chauffard syndrome Hereditary elliptocytosis Southeast Asian ovalocytosis Hereditary stomatocytosis

Acquired	Autoimmune WAHA CAD PCH membrane PNH MAHA TM HUS Drug-induced autoimmune Drug-induced nonautoimmune Hemolytic disease of the newborn

Aplastic
(mostly normo-)

Blood tests

MCV
MCHC
- Normochromic
- Hypochromic

Other

Diseases of clotting (D50–69,74, 280–287)

Coagulation/
coagulopathy

↑

Hyper- coagulability	primary: Antithrombin III deficiency Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden Prothrombin G20210A Sticky platelet syndrome acquired:Thrombocytosis essential DIC Purpura fulminans autoimmune Antiphospholipid

↓

Hypo-
coagulability

Thrombocytopenia	Thrombocytopenic purpura: ITP Evans syndrome TM TTP Upshaw Schulman syndrome Heparin-induced thrombocytopenia May–Hegglin anomaly

Platelet function	adhesion Bernard–Soulier syndrome aggregation Glanzmann's thrombasthenia platelet storage pool deficiency Hermansky–Pudlak syndrome Gray platelet syndrome

Clotting factor	Hemophilia A/VIII B/IX C/XI von Willebrand disease Hypoprothrombinemia/II XIII Dysfibrinogenemia Congenital afibrinogenemia

Lymphatic disease: Congenital lymphatic organ disorders (Q89.0/Q89.2, 759.0/759.2)

Thymus	Thymic hypoplasia DiGeorge syndrome Ectopic thymus

Spleen	Congenital asplenia/hyposplenism Asplenia with cardiovascular anomalies Accessory spleen Polysplenia

Histiocytosis (D76.0, 277.89)

WHO-I/Langerhans cell histiocytosis/ X-type histiocytosis	Letterer–Siwe disease Hand–Schüller–Christian disease Eosinophilic granuloma Congenital self-healing reticulohistiocytosis

WHO-II/non-Langerhans cell histiocytosis/ Non-X histiocytosis	Juvenile xanthogranuloma Hemophagocytic lymphohistiocytosis Erdheim-Chester disease Niemann-Pick disease Sea-blue histiocyte Benign cephalic histiocytosis Generalized eruptive histiocytoma Xanthoma disseminatum Progressive nodular histiocytosis Papular xanthoma Hereditary progressive mucinous histiocytosis Reticulohistiocytosis (Multicentric reticulohistiocytosis, Reticulohistiocytoma) Indeterminate cell histiocytosis

WHO-III/malignant histiocytosis	Histiocytic sarcoma Langerhans cell sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma

Ungrouped	Rosai–Dorfman disease

Immune disorders: Lymphoid and complement immunodeficiency (D80–D85, 279.0–4)

Primary

Antibody/humoral (B)

Hypogammaglobulinemia	X-linked agammaglobulinemia Transient hypogammaglobulinemia of infancy

Dysgammaglobulinemia	IgA deficiency IgG deficiency IgM deficiency Hyper IgM syndrome (1 2 3 4 5) Wiskott-Aldrich syndrome Hyper-IgE syndrome

Other	Common variable immunodeficiency ICF syndrome

T cell deficiency (T)

thymic hypoplasia: hypoparathyroid (Di George's syndrome)
euparathyroid (Nezelof syndrome
Ataxia telangiectasia)

peripheral: Purine nucleoside phosphorylase deficiency

Hyper IgM syndrome (1)

Severe combined (B+T)

x-linked: X-SCID
autosomal: Adenosine deaminase deficiency
Omenn syndrome
ZAP70 deficiency
Bare lymphocyte syndrome

Acquired

AIDS

Leukopenia:
Lymphocytopenia

Idiopathic CD4+ lymphocytopenia

Complement deficiency

Sarcoidosis (D86, 135)

Skin Lupus pernio Neurosarcoidosis Löfgren syndrome Heerfordt's syndrome

Immunoproliferative immunoglobulin disorders (D89, 273)

PCDs/PP	Plasmacytoma Multiple myeloma (Plasma cell leukemia) MGUS IgM (Macroglobulinemia/Waldenström's macroglobulinemia) heavy chain (Heavy chain disease) light chain (Primary amyloidosis)

Other hypergammaglobulinemia	Cryoglobulinemia

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ICD-10 Chapter III: 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–D53) Nutritional anemias

(D55–D59) Haemolytic anaemias

(D60–D64) Aplastic and other anaemias

(D65–D69) Coagulation defects, purpura and other haemorrhagic conditions

(D70–D77) Other diseases of blood and blood-forming organs

(D80–D89) Certain disorders involving the immune mechanism

Excludes

See also

References