2D00, 2D01, 2D02, 2D03, 2D04, 2D05, 2D06, 2D07, 2D0Y, 2D0Z

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

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