Fibrinolytic Defects – Definitions & ICD 11 Codes
Definition of Fibrinolytic Defects: A disease caused by determinants arising during the antenatal period, after birth or genetically inherited factors, affecting the fibrinolysis system which prevents blood clots from growing and becoming problematic. This disease is characterised by defects in the fibrinolysis system leading to coagulation of the blood. This disease may present with thrombosis.
Inherited fibrinolytic defects
Definition of Inherited fibrinolytic defects: A disease caused by genetically inherited mutations affecting the fibrinolysis system which prevents blood clots from growing and becoming problematic. This disease is characterised by defects in the fibrinolysis system leading to coagulation of the blood. This disease may present with thrombosis.
ICD 11 Code For Inherited fibrinolytic defects
3B50 Inherited fibrinolytic defects
Coded Elsewhere:
- Hypoplasminogenaemia (DA0D.3)
3B50.0 Congenital alpha-2 antiplasmin deficiency
3B50.1 Congenital plasminogen activator inhibitor type 1 deficiency
Definition of Congenital plasminogen activator inhibitor type 1 deficiency: Congenital plasminogen activator inhibitor type 1(PAI-1) deficiency is a disorder that causes premature lysis of haemostatic clots and a moderate bleeding syndrome. Spontaneous bleeding is rarely observed, whereas moderate haemorrhages of the knees, elbows, nose and gingiva are usually triggered by mild trauma. However, menstrual bleeding may be severe and a prolonged bleeding after surgery is common. The PAI-1 deficiency may be qualitative or quantitative, total or partial.
3B50.Y Other specified inherited fibrinolytic defects
3B50.Z Inherited fibrinolytic defects unspecified
Acquired fibrinolytic defects
Definition of Acquired fibrinolytic defects: A disease caused by determinates arising after birth, affecting the fibrinolysis system which prevents blood clots from growing and becoming problematic. This disease is characterised by defects in the Fibrinolysis system leading to coagulation of the blood. This disease may present with thrombosis.
ICD 11 Code For Acquired fibrinolytic defects
3B51 Acquired fibrinolytic defects
Non-thrombocytopenic purpura
Definition of Non-thrombocytopenic purpura: A descriptive term for purpura caused by determinants other than low platelet count. This should be used for coding only when a more precise diagnosis is not available.
ICD 11 Code For Non-thrombocytopenic purpura
3B60 Non-thrombocytopenic purpura
Exclusions:
- Antineutrophil cytoplasmic antibody-associated vasculitis (4A44.A)
- Antiphospholipid syndrome (4A45)
- Drug-associated immune complex vasculitis (4A85.03)
- Immune complex small vessel vasculitis (4A44.9)
- Leukocytoclastic vasculitis (4A44.B)
- Purpura or bruising due to vascular fragility (EE40.32)
- Thrombotic thrombocytopenic purpura (3B64.14)
- Traumatic purpura (EF31)
3B60.0 Hereditary vascular purpura
3B60.1 Acquired vascular purpura
Definition of Acquired vascular purpura: Purpura resulting from vascular factors rather than from abnormalities in the blood such as dysproteinaemias and disorders of platelets and coagulation.
Exclusions:
- Antineutrophil cytoplasmic antibody-associated vasculitis (4A44.A)
- Antiphospholipid syndrome (4A45)
- Capillaritis (EF40.0)
- Drug-associated immune complex vasculitis (4A85.03)
- IgA vasculitis (4A44.92)
- Leukocytoclastic vasculitis (4A44.B)
- Purpura or bruising due to vascular fragility (EE40.32)
- Thrombotic thrombocytopenic purpura (3B64.14)
- Traumatic purpura (EF31)
Thrombophilia
Definition of Thrombophilia: A disease caused by determinants arising after birth or genetically inherited factors leading to abnormalities in blood. This disease is characterised by abnormality of blood coagulation that increases the risk of thrombosis, clots in blood vessels. This disease may present with deep vein thrombosis or pulmonary embolism. Confirmation is identification of abnormal blood coagulation in a blood sample.
ICD 11 Code For Thrombophilia
3B61 Thrombophilia
3B61.0 Hereditary thrombophilia
Definition of Hereditary thrombophilia: A disease caused by determinants arising after birth or genetically inherited factors leading to abnormalities in blood. This disease is characterised by abnormality of blood coagulation that increases the risk of thrombosis, clots in blood vessels. This disease may present with deep vein thrombosis or pulmonary embolism. Confirmation is identification of abnormal blood coagulation in a blood sample.
Inclusions:
- antithrombin deficiency
3B61.00 Hyperhomocysteinaemia
Definition of Hyperhomocysteinaemia: A disease caused by deficiencies of vitamin B6, folic acid, or vitamin B12. Genetic defects in 5-MTHF reductase can consequently lead to hyperhomocysteinaemia. This disease is characterised by abnormally high level of homocysteine in the blood. This disease may present with cardiovascular disease, thrombosis, schizophrenia and osteoporosis. Confirmation is by identification of deficiency in a blood sample.
3B61.0Y Other specified hereditary thrombophilia
3B61.1 Acquired thrombophilia
Definition of Acquired thrombophilia: A disease caused by determinants arising after birth. This disease is characterised by abnormality of blood coagulation that increases the risk of thrombosis, clots in blood vessels. This disease may present with deep vein thrombosis or pulmonary embolism. Confirmation is identification of abnormal blood coagulation in a blood sample.
Coded Elsewhere:
- Antiphospholipid syndrome (4A45)
3B61.Y Other specified thrombophilia
3B61.Z Thrombophilia unspecified
Qualitative platelet defects
Definition of Qualitative platelet defects: A disease caused by determinants arising after birth, during the antenatal period or genetically inherited factors. This disease is characterised by abnormalities in coagulation of the blood due to defective platelets. This condition may present with easy bruising, prolonged bleeding or, bleeding gums. Confirmation is by identification of decreased platelets in a blood sample.
ICD 11 Code For Qualitative platelet defects
3B62 Qualitative platelet defects
Inclusions:
- Thrombocytopathy
Exclusions:
- Von Willebrand disease (3B12)
3B62.0 Inherited qualitative platelet defects
Definition of Inherited qualitative platelet defects: A disease caused by genetically inherited mutations leading to abnormalities in platelets. This disease is characterised by abnormal platelet formation or function. Confirmation is by identification of mutations by genetic testing.
Coded Elsewhere:
- Dense granule disease (3B62.3)
- Alpha-delta dense granule deficiency (3B62.4)
3B62.00 Alpha-granule diseases
Definition of Alpha-granule diseases: A condition caused by determinants arising after birth, in the antenatal period. This condition is characterised by defects in the alpha granules in platelets leading to abnormalities in coagulation mechanisms. This condition may present with prolonged bleeding, epistaxis, menorrhagia, easy bruising, anaemia, fatigue or shortness of breath. Confirmation is by identification of platelet defects in a blood sample.
3B62.01 Inherited giant platelet disorder
Definition of Inherited giant platelet disorder: A disease caused by genetically inherited mutations. This disease is characterised by abnormally large platelets, low platelet count and a bleeding tendency. Confirmation is by identification of mutations through genetic testing.
Coded Elsewhere:
- MYH9 macrothrombocytopenia syndromes (3B64.01)
3B62.0Y Other specified inherited qualitative platelet defects
3B62.0Z Inherited qualitative platelet defects unspecified
3B62.1 Bleeding diathesis due to thromboxane synthesis deficiency
Definition of Bleeding diathesis due to thromboxane synthesis deficiency: A disease caused by thromboxane synthesis deficiency. This disease is characterised by low levels of eicosanoids (lipids), abnormalities in coagulation leading to haemorrhaging. Confirmation is by identification of low levels of eicosanoids in a blood sample.
3B62.2 Isolated thrombocytopenia
3B62.3 Dense granule disease
Definition of Dense granule disease: A condition caused by determinants arising after birth, in the antenatal period. This condition is characterised by defects in the dense granules in platelets leading to abnormalities in coagulation mechanisms. This condition may present with prolonged bleeding, epistaxis, menorrhagia, easy bruising, anaemia, fatigue or shortness of breath. Confirmation is by identification of platelet defects in a blood sample.
Coded Elsewhere:
- Hermansky-Pudlak syndrome (EC23.20)
- Chédiak-Higashi syndrome (EC23.20)
3B62.4 Alpha-delta dense granule deficiency
Definition of Alpha-delta dense granule deficiency: A condition caused by determinants arising after birth, in the antenatal period. This condition is characterised by defects in the alpha delta dense granules in platelets, leading to abnormalities in coagulation mechanisms. This condition may present with prolonged bleeding, epistaxis, menorrhagia, easy bruising, anaemia, fatigue or shortness of breath. Confirmation is by identification of low levels of alpha delta dense granules in a blood sample.
3B62.5 Haemophagocytic syndrome associated with infection
Definition of Haemophagocytic syndrome associated with infection: This is an uncommon hematologic disorder that, typically, clinically manifests as fever, hepatosplenomegaly, lymphadenopathy, jaundice and rash, with laboratory findings of histiocytosis, and the pathologic finding of haemophagocytosis, infection-associated.
3B62.Y Other specified qualitative platelet defects
3B62.Z Qualitative platelet defects unspecified
Thrombocytosis
Definition of Thrombocytosis: A disease caused by essential thrombocytosis or other myelo-proliferative disorders such as chronic myelogenous leukaemia, polycythaemia, myelofibrosis. This disease can also have secondary causes such as inflammation, surgery, hyposplenism, Splenectomy, asplenia, iron deficiency anaemia or haemorrhage. This disease is characterised by elevated platelet count in the blood. Confirmation is by identification of increased platelet count in a blood sample.
ICD 11 Code For Thrombocytosis
3B63 Thrombocytosis
3B63.0 Congenital thrombocytosis
Definition of Congenital thrombocytosis: Familial thrombocytosis is a type of thrombocytosis, a sustained elevation of platelet numbers, which affects the platelet/megakaryocyte lineage and may create a tendency for thrombosis and haemorrhage but does not cause myeloproliferation.
Inclusions:
- Hereditary thrombocytosis
Exclusions:
- Essential thrombocythemia (3B63.1)
3B63.1 Acquired thrombocytosis
Definition of Acquired thrombocytosis: A chronic myeloproliferative neoplasm that involves primarily the megakaryocytic lineage. It is characterised by sustained thrombocytosis in the blood, increased numbers of large, mature megakaryocytes in the bone marrow, and episodes of thrombosis and/or haemorrhage. Progression to a post -essential thrombocythaemia myelofibrosis stage or transformation to acute myeloid leukaemia is rarely observed.
Inclusions:
- Idiopathic haemorrhagic thrombocythaemia
3B63.10 Secondary thrombocytosis
Coding Note:
- Code also the causing condition
3B63.1Y Other specified acquired thrombocytosis
3B63.1Z Acquired thrombocytosis unspecified
3B63.Y Other specified thrombocytosis
3B63.Z Thrombocytosis unspecified
Thrombocytopenia
Definition of Thrombocytopenia: This disease is characterised by decreased levels of platelets within the blood. This disease may present with increased bruising or haemorrhaging. Confirmation is by identification of decreased platelet count in a blood sample.
ICD 11 Code For Thrombocytopenia
3B64 Thrombocytopenia
Coded Elsewhere:
- Isolated thrombocytopenia (3B62.2)
3B64.0 Congenital thrombocytopenia
Definition of Congenital thrombocytopenia: A disease caused by determinants arising during the antenatal period leading to low platelet count. This disease is characterised by decreased levels of platelets within the blood. This disease may present with increased bruising or haemorrhaging. Confirmation is by identification of a decreased platelet count in a blood sample.
3B64.00 Congenital non-inherited thrombocytopenia
Coded Elsewhere:
- Transient neonatal thrombocytopaenia (KA89)
3B64.01 Hereditary thrombocytopenia
Definition of Hereditary thrombocytopenia: A disease caused by a genetically inherited mutation leading to decreased platelet count. This disease is characterised by decreased levels of platelets within the blood. This disease may present with increased bruising or haemorrhaging. Confirmation is by identification of decreased platelet count in a blood sample.
Coded Elsewhere:
- Thrombocytopaenia – absent radius (LD2F.1Y)
- Familial platelet syndrome with predisposition to acute myelogenous leukaemia (3B62.3)
- Congenital thrombotic thrombocytopenic purpura due to ADAMTS-13 deficiency (3B64.14)
- Macrothrombocytopenia with mitral valve insufficiency (3B62.01)
3B64.0Z Congenital thrombocytopenia unspecified
3B64.1 Acquired thrombocytopenia
Definition of Acquired thrombocytopenia: A disease caused by determinants arising after birth, leading to low platelet count. This disease is characterised by low levels of platelets within the blood. This disease may present with increased bruising or haemorrhaging. Confirmation is by identification of decreased platelet count in a blood sample.
3B64.10 Immune thrombocytopenic purpura
Definition of Immune thrombocytopenic purpura: Immune thrombocytopenic purpura (or immune thrombocytopenia; ITP) is an autoimmune coagulation disorder characterised by isolated thrombocytopenia (a platelet count <100,000/microL), in the absence of any underlying disorder that may be associated with thrombocytopenia.
Coded Elsewhere:
- Evans syndrome (3A20.5)
3B64.11 Secondary thrombocytopenic purpura
Definition of Secondary thrombocytopenic purpura: This disease is characterised by a relative decrease in levels of platelets within the blood. This disease may present with increased bruising or haemorrhaging. Confirmation is by identification of decreased platelets in a blood sample.
Coding Note:
- Code also the causing condition
3B64.12 Drug-induced thrombocytopenic purpura
Definition of Drug-induced thrombocytopenic purpura: Thrombocytopenic purpura attributable to drug toxicity (e.g. cytotoxic chemotherapeutic or immunosuppressive agents) or to an idiosyncratic drug-associated allergic thrombocytopenia (e.g. quinine, thiazides).
3B64.13 Alloimmune thrombocytopenia
Definition of Alloimmune thrombocytopenia: A disease caused by determinants such as a blood transfusion that lead to an immune response to the foreign antigens. This disease is characterised by low levels of platelets in the body due to an immune reactive response to the foreign platelet antigens. This disease may present with increased bruising or haemorrhaging. Confirmation is by identification of decreased platelet count and presence of autoantibodies in a blood sample.
3B64.14 Thrombotic thrombocytopenic purpura
Definition of Thrombotic thrombocytopenic purpura: This condition is idiopathic. This condition is characterised by abnormality of blood coagulation causing extensive microscopic clots to form in the small blood vessels throughout the body resulting in low platelet count. This condition may present with seizures, hemiplegia, paresthesias, visual disturbance, and aphasia or anaemia. Confirmation is by identification of thromboses in a blood sample.
3B64.1Y Other specified acquired thrombocytopenia
3B64.Z Thrombocytopenia unspecified
Thrombotic microangiopathy not elsewhere classified
Definition of Thrombotic microangiopathy not elsewhere classified: Thrombotic microangiopathies are microvascular occlusive disorders characterised by systemic or intrarenal aggregation of platelets, thrombocytopenia, and mechanical injury to erythrocytes. Thrombotic thrombocytopenic purpura (TTP) and haemolytic–uremic syndrome (HUS) represent a spectrum of thrombotic microangiopathies. In TTP, systemic microvascular aggregation of platelets causes ischemia in the brain and other organs. In HUS, platelet–fibrin thrombi predominantly occlude the renal circulation.
ICD 11 Code For Thrombotic microangiopathy not elsewhere classified
3B65 Thrombotic microangiopathy not elsewhere classified
Coded Elsewhere:
- Thrombotic thrombocytopenic purpura (3B64.14)
- Haemolytic uraemic syndrome (3A21.2)
- Methylcobalamin deficiency type cbl G (5C50.B)
- Hereditary haemolytic uraemic syndrome (3A10.Y)
Other specified coagulation defects purpura or other haemorrhagic or related conditions
ICD 11 Code For Other specified coagulation defects purpura or other haemorrhagic or related conditions
3B6Y Other specified coagulation defects purpura or other haemorrhagic or related conditions
Coagulation defects purpura or other haemorrhagic or related conditions unspecified
ICD 11 Code For Coagulation defects purpura or other haemorrhagic or related conditions unspecified
3B6Z Coagulation defects purpura or other haemorrhagic or related conditions unspecified
