Pure Red Cell Aplasia – Definitions & ICD 11 Codes

Definition of Pure Red Cell Aplasia: A condition caused by determinates arising during the antenatal period, after birth or genetically inherited factors, leading to a change in the formation of erythrocytes. This condition is characterised by maturation arrest occurs in the formation of erythrocytes. This condition may present with severe anaemia. Confirmation is by identification of abnormally formed erythrocytes in a blood sample.

Congenital pure red cell aplasia

Definition of Congenital pure red cell aplasia: A condition caused by determinants arising during the antenatal period, leading to a change in the formation of erythrocytes. This condition is characterised by maturation arrest occurs in the formation of erythrocytes. This condition may present with severe anaemia. Confirmation is by identification of decreased red blood cell count in a blood sample.

ICD 11 Code For Congenital pure red cell aplasia

  3A60  Congenital pure red cell aplasia

3A60.0 Congenital non-inherited pure red cell aplasia

Definition of Congenital non-inherited pure red cell aplasia: A condition caused by determinates arising during the antenatal period, leading to a change in the formation of erythrocytes. This condition is characterised by maturation arrest occurs in the formation of erythrocytes. This condition may present with severe anaemia. Confirmation is by identification of decreased levels of red blood cells in a blood sample.

3A60.1 Hereditary pure red cell aplasia

Definition of Hereditary pure red cell aplasia: A condition caused by determinates arising during the antenatal period, leading to a change in the formation of erythrocytes. This condition is characterised by maturation arrest occurs in the formation of erythrocytes. This condition may present with severe anaemia. Confirmation is by identification of decreased red blood cell count in a blood sample.

3A60.Z Congenital pure red cell aplasia unspecified

Acquired pure red cell aplasia

Definition of Acquired pure red cell aplasia: A condition characterised by the near absence of red blood cell precursors in bone marrow, often associated with thymomas and autoimmune disorders

ICD 11 Code For Acquired pure red cell aplasia

  3A61  Acquired pure red cell aplasia

Exclusions:

  • Aplastic anaemia with paroxysmal nocturnal haemoglobulinuria (3A70.1)

3A61.0 Acute acquired pure red cell aplasia

Definition of Acute acquired pure red cell aplasia: This refers to transient (acute) and acquired type of anaemia affecting the precursors to red blood cells but not to white blood cells. In PRCA, the bone marrow ceases to produce red blood cells.

3A61.1 Chronic acquired pure red cell aplasia

Definition of Chronic acquired pure red cell aplasia: This refers to a chronic and acquired type of anaemia affecting the precursors to red blood cells but not to white blood cells. In PRCA, the bone marrow ceases to produce red blood cells.

3A61.Y Other specified acquired pure red cell aplasia

3A61.Z Acquired pure red cell aplasia unspecified

Pure red cell aplasia unspecified

ICD 11 Code For Pure red cell aplasia unspecified

  3A6Z  Pure red cell aplasia unspecified

Aplastic anaemia

Definition of Aplastic anaemia: A disease caused by determinants arising after birth, in the antenatal period or genetically inherited factors leading to the inability of stem cells to generate new mature cells. This disease is characterised by low levels of red blood cells, white blood cells, and platelets. This disease may present with pallor, fatigue, dizziness, increased risk of infection or increased bruising or bleeding.

ICD 11 Code For Aplastic anaemia

  3A70  Aplastic anaemia

Inclusions:

  • Medullary hypoplasia
  • Panmyelophthisis

3A70.0 Congenital aplastic anaemia

Definition of Congenital aplastic anaemia: A disease caused by determinants in the antenatal period leading to the inability of stem cells to generate new mature cells. This disease is characterised by low levels of red blood cells, white blood cells, platelets. This disease may present with pallor, fatigue, dizziness, increased risk of infection or increased bruising or bleeding.

Inclusions:

  • familial hypoplastic anaemia
  • Constitutional medullar aplasia

Exclusions:

Coded Elsewhere:

  • Congenital hypoplastic anaemia (KA8C)
  • Noonan syndrome (LD2F.15)

3A70.1 Acquired aplastic anaemias

Definition of Acquired aplastic anaemias: A condition occurring secondary to other disorders or via an auto-immune response directed to the bone marrow arising after birth. This disease is characterised by an almost complete absence of hematopoietic stem cells resulting in low levels of red and white blood cells and platelets. This condition may present with fatigue, chronic infections, dizziness, weakness, headaches, and episodes of bleeding, usually in the skin and mucous membranes.

Inclusions:

  • Acquired medullar aplasia

Coded Elsewhere:

  • Paroxysmal nocturnal haemoglobinuria (3A21.0)
  • Myelofibrosis with myeloid metaplasia (2A20.2)

3A70.10 Drug-induced aplastic anaemia

Definition of Drug-induced aplastic anaemia: A disease caused by drug intake. This disease is characterised by inability of stem cells to generate new mature cells leading to low levels of red blood cells, white blood cells, platelets. This disease may present with pallor, fatigue, dizziness, increased risk of infection or increased bruising/bleeding.

3A70.11 Aplastic anaemia due to other external agents

3A70.12 Idiopathic aplastic anaemia

3A70.1Y Other specified acquired aplastic anaemias

3A70.1Z Acquired aplastic anaemias unspecified

3A70.Z Aplastic anaemia unspecified

Anaemia due to chronic disease

Definition of Anaemia due to chronic disease: A disease caused by chronic diseases such as chronic infection. This disease is characterised by inflammatory responses targeted at red blood cells leading to low levels of red blood cells in the body. This disease may present with pallor, fatigue, or shortness of breath. Confirmation is by identification of low levels of red blood cells in a blood sample.

ICD 11 Code For Anaemia due to chronic disease

  3A71  Anaemia due to chronic disease

Coding Note:

  • Code also the causing condition

3A71.0 Anaemia in neoplastic disease

Definition of Anaemia in neoplastic disease: A disease caused by chronic neoplastic diseases. This disease is characterised by inflammatory responses targeted at red blood cell leading to low levels of red blood cells in the body. This disease may present with pallor, fatigue, or shortness of breath. Confirmation is by identification of low levels of red blood cells in a blood sample

Coding Note:

  • Code also the causing condition

3A71.1 Anaemia in chronic infectious diseases

Definition of Anaemia in chronic infectious diseases: A disease caused by chronic infectious diseases leading to decreased levels of red blood cells in the blood. This disease is characterised by a low red blood cell count in the body. This disease may present with pallor, fatigue, or shortness of breath. Confirmation is by identification of low red blood cell count in a blood sample.

Coding Note:

  • Code also the causing condition

3A71.2 Anaemia in chronic kidney disease

Definition of Anaemia in chronic kidney disease: A disease caused by chronic kidney disease. This disease is characterised by a low red blood cell count in the blood. This disease may present with pallor, fatigue, or shortness of breath. Confirmation is by identification of a low red blood cell count in a blood sample.

Coding Note:

  • Code also the causing condition

3A71.Y Anaemia due to other specified chronic disease

Coding Note:

  • Code also the causing condition

3A71.Z Anaemia due to chronic disease unspecified

Coding Note:

  • Code also the causing condition

Sideroblastic anaemia

Definition of Sideroblastic anaemia: Sideroblastic anaemias are a group of disorders in which haemoglobin is insufficiently synthesized, because of defective use of iron (although plasmatic iron levels may be normal or elevated). They are said to be sideroblastic because of the presence of ringed sideroblasts in the blood due to accumulated ferritin in mitochondria. Anaemias may be microcytic hypochrome (in thalassemia and hereditary sideroblastic anaemias), or macrocytic (in idiopathic acquired sideroblastic anaemias).

ICD 11 Code For Sideroblastic anaemia

  3A72  Sideroblastic anaemia

3A72.0 Congenital sideroblastic anaemias

Definition of Congenital sideroblastic anaemias: A disease caused by determinants arising in the antenatal period leading to the production of ringed sideroblasts; abnormal nucleated erythroblasts. This disease is characterised by the inability of to incorporate haemoglobin, which red blood cells need to transport oxygen efficiently. This disease may present with pallor, fatigue, dizziness, and enlarged spleen and liver, heart disease, liver damage, or kidney failure.

3A72.00 Hereditary sideroblastic anaemias

Inclusions:

  • Sex-linked hypochromic sideroblastic anaemia

3A72.01 Hereditary syndromic sideroblastic anaemia

Coded Elsewhere:

  • Thiamine-responsive megaloblastic anaemia syndrome (5C63.Y)

3A72.0Y Other specified congenital sideroblastic anaemias

3A72.0Z Congenital sideroblastic anaemias unspecified

3A72.1 Acquired sideroblastic anaemias

Definition of Acquired sideroblastic anaemias: A disease caused by determinants arising after birth such as myelodysplastic syndromes, antimicrobials, pyridoxine deficiency, lead poisoning, or copper deficiency. Zinc can indirectly cause sideroblastic anaemia by decreasing absorption and increasing excretion of copper. This disease is characterised by the inability of to incorporate haemoglobin, which red blood cells need to transport oxygen efficiently. This disease may present with pallor, fatigue, dizziness, and enlarged spleen and liver, heart disease, liver damage, or kidney failure.

Coded Elsewhere:

  • Refractory anaemia with ring sideroblasts (2A33)

3A72.Z Sideroblastic anaemia unspecified

Congenital dyserythropoietic anaemia

Definition of Congenital dyserythropoietic anaemia: Congenital dyserythropoietic anaemias (CDA) result from diverse erythropoietic disorders; they lead to the defective production of red blood cells (RBC) and often mild haemolysis that attests to a qualitative defect of these RBC released into the circulation. Three forms of CDA have been characterised: types I, II and III. The shared symptoms include anaemia of variable severity, intermittent jaundice, splenomegaly and hepatomegaly.

ICD 11 Code For Congenital dyserythropoietic anaemia

  3A73  Congenital dyserythropoietic anaemia

Exclusions:

  • Blackfan-Diamond syndrome (3A60.1)
  • Di Guglielmo disease (2A60.35)

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