3B10

ICD 11 Congenital Or Constitutional Haemorrhagic Condition

Definition of Congenital Or Constitutional Haemorrhagic Condition: A condition caused by determinants arising during the antenatal period or genetically inherited factors, leading to defects in clotting mechanisms or abnormalities causing structural flaws in the blood vessels. This disease is characterised by spontaneous bleeding or bruising.

Coded Elsewhere:

  • Congenital non-inherited haemorrhagic condition

Hereditary factor VIII deficiency

Definition of Hereditary factor VIII deficiency: A disease caused by a genetically inherited mutation leading to a deficiency in clotting due to lack of factor VIII. This disease is characterised by increasing haemorrhaging and bruising. Confirmation is by identification of mutations by genetic testing.

ICD 11 Code For Hereditary factor VIII deficiency

  3B10  Hereditary factor VIII deficiency

3B10.0 Haemophilia A

Definition of Haemophilia A: Haemophilia A is the most common form of haemophilia characterised by spontaneous or prolonged haemorrhages due to factor VIII deficiency. Depending on the extent of the factor VIII deficiency, it can be severe (biological activity of factor VIII below 1%), moderately severe (activity of factor VIII between 1% and 5%), or mild (activity of factor VIII between 5 and 40%).

Exclusions:

  • factor VIII deficiency with vascular defect (3B12)

3B10.1 Hereditary factor VIII deficiency with anti-factor VIII inhibitor

Definition of Hereditary factor VIII deficiency with anti-factor VIII inhibitor: A disease caused by a genetically inherited mutation leading to a deficiency in clotting due to lack of factor VIII. This disease also causes anti-factor VIII inhibitor antibodies to be produced when receiving transfusions. Anti-factor VIII inhibitor antibodies develop as the body recognises the factor VIII as foreign, therefore deeming factor VIII infusions ineffective. This disease is characterised by increasing haemorrhaging and bruising. Confirmation is by identification of mutations by genetic testing.

3B10.Y Other specified hereditary factor VIII deficiency

3B10.Z Hereditary factor VIII deficiency unspecified

Hereditary factor IX deficiency

Definition of Hereditary factor IX deficiency: A disease caused by a genetically inherited X-linked recessive trait leading to a defective gene located on the X chromosome. This disease is characterised by low levels of the protein factor IX in the body leading to increased haemorrhaging and bruising due to clotting abnormalities. Confirmation is by identification of recessive trait by genetic testing.

ICD 11 Code For Hereditary factor IX deficiency

  3B11  Hereditary factor IX deficiency

3B11.0 Haemophilia B

Definition of Haemophilia B: Haemophilia B is a form of haemophilia characterised by spontaneous or prolonged haemorrhages due to factor IX deficiency. Depending on the extent of the factor IX deficiency, it can be severe (biological activity of factor IX below 1%), moderately severe (activity of factor IX between 1% and 5%), or mild (activity of factor IX between 5 and 40%).

Inclusions:

  • PTC – [plasma thromboplastin component] deficiency

3B11.Y Other specified hereditary factor IX deficiency

3B11.Z Hereditary factor IX deficiency unspecified

Von Willebrand disease

Definition of Von Willebrand disease: A disease caused by inherited genetic mutations. This disease is characterised by quantitative, structural or function abnormalities of Von Willebrand factor leading to abnormalities in coagulation of the blood. This disease may present with prolonged bleeding, easy bruising or, bleeding gums. Confirmation is by identification of mutation through genetic testing.

ICD 11 Code For Von Willebrand disease

  3B12  Von Willebrand disease

Inclusions:

  • Factor VIII deficiency with vascular defect
  • Vascular haemophilia
  • Angiohaemophilia

Exclusions:

  • factor VIII deficiency with functional defect (3B10)
  • factor VIII deficiency NOS (3B10)
  • Acquired von Willebrand disease or syndrome (BlockL3‑3B2)

Haemophilia C

Definition of Haemophilia C: A disease caused by genetically inherited mutations. This disease is characterised by decreased levels of factor XI leading to abnormalities in coagulation of the blood. This disease may present with prolonged bleeding, easy bruising or, bleeding gums. Confirmation is by identification of mutation through genetic testing.

ICD 11 Code For Haemophilia C

  3B13  Haemophilia C

Other inherited coagulation factor deficiency with bleeding tendency

Definition of Other inherited coagulation factor deficiency with bleeding tendency: Any disease caused by genetically inherited mutations leading to lack of coagulation factors in the blood not elsewhere classified. These diseases are characterised by increased haemorrhaging and bruising as the blood cannot clot properly to control bleeding. Confirmation is identification of mutations by genetic testing.

ICD 11 Code For Other inherited coagulation factor deficiency with bleeding tendency

  3B14  Other inherited coagulation factor deficiency with bleeding tendency

3B14.0 Hereditary deficiency of factor I

Definition of Hereditary deficiency of factor I: Congenital deficiencies of fibrinogen are coagulation disorders characterised by bleeding symptoms ranging from mild to severe resulting from reduced quantity and/or quality of circulating fibrinogen. Afibrinogenaemia (complete absence of fibrinogen) and hypofibrinogenaemia (reduced plasma fibrinogen concentration) correspond to quantitative anomalies of fibrinogen while dysfibrinogenaemia corresponds to a functional anomaly of fibrinogen. Hypo- and dysfibrinogenaemia may be frequently combined (hypodysfibrinogenaemia).

3B14.1 Hereditary factor X deficiency

Definition of Hereditary factor X deficiency: Congenital factor X deficiency is an inherited bleeding disorder with a decreased antigen and/or activity of factor X (FX) and characterised by mild to severe bleeding symptoms.

3B14.2 Combined deficiency of vitamin K-dependent clotting factors

Definition of Combined deficiency of vitamin K-dependent clotting factors: Hereditary combined vitamin K-dependent clotting factors deficiency (VKCFD) is a congenital bleeding disorder resulting from variably decreased levels of coagulation factors II, VII, IX and X, as well as natural anticoagulants protein C, protein S and protein Z.

3B14.Z Other inherited coagulation factor deficiency with bleeding tendency unspecified

Inherited coagulation factor deficiency without bleeding tendency

Definition of Inherited coagulation factor deficiency without bleeding tendency: A disease caused by a genetically inherited mutation leading to decreased levels of coagulation factor. This disease is characterised by decreased levels of coagulation factor without leading to increased haemorrhaging. Confirmation is by identification of decreased levels of coagulation factor in a blood sample.

ICD 11 Code For Inherited coagulation factor deficiency without bleeding tendency

  3B15  Inherited coagulation factor deficiency without bleeding tendency

Congenital or constitutional haemorrhagic condition unspecified

ICD 11 Code For Congenital or constitutional haemorrhagic condition unspecified

  3B1Z  Congenital or constitutional haemorrhagic condition unspecified

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