Acquired Haemolytic Anaemia – Definitions & ICD 11 Codes

Definition of Acquired Haemolytic Anaemia: A disease characterised by premature destruction of red blood cells arising after birth. This disease is further characterised by low levels of red blood cells in the body due to abnormal destruction of the cells. 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.

Acquired haemolytic anaemia immune

Definition of Acquired haemolytic anaemia immune: A condition characterised by antibodies that are directed against red blood cells in an autoimmune reaction leading to low levels of red blood cells. This condition may present with pallor, fatigue, shortness of breath. Confirmation is by identification of antibodies in a blood sample and positive Coombs test result.

ICD 11 Code For Acquired haemolytic anaemia immune

  3A20  Acquired haemolytic anaemia immune

3A20.0 Autoimmune haemolytic anaemia warm type

Definition of Autoimmune haemolytic anaemia warm type: Autoimmune haemolytic anaemia (AIHA) is an autoimmune disorder in which various types of auto-antibodies are directed against red blood cells causing their survival to be shortened and resulting in haemolytic anaemia. AIHA can be primary (idiopathic), secondary to infection or associated with diseases such as B-cell lymphomas, other systemic or organ-specific autoimmune diseases, Hodgkin’s disease, hepatitis or primary immunodeficiencies, or, in the case of drug-induced AIHA, caused by a reaction to drugs.

Exclusions:

  • Evans syndrome (3A20.5)
  • Haemolytic disease of fetus or newborn (KA84)
  • Paroxysmal cold haemoglobinuria (3A20.3)

3A20.1 Autoimmune haemolytic anaemia cold type

Definition of Autoimmune haemolytic anaemia cold type: Cold autoimmune haemolytic anaemia comprises two types of autoimmune haemolytic anaemia (AIHA) defined by the presence of cold autoantibodies (autoantibodies which are active at temperatures below 30°C): cold agglutinin disease (CAD), which is the more common, and paroxysmal cold haemoglobinuria (PCH). CAD is more common in people over the age of 55 years, while PCH typically presents in young children. CAD is caused by IgM autoantibodies while PCH is caused by an IgG immunoglobulin.

Exclusions:

  • Immune thrombocytopenic purpura (3B64.10)
  • Haemolytic disease of fetus or newborn (KA84)

3A20.2 Autoimmune haemolytic anaemia mixed type cold and warm

Definition of Autoimmune haemolytic anaemia mixed type cold and warm: Mixed autoimmune haemolytic anaemia is a type of autoimmune haemolytic anaemia (AIHA) defined by the presence of both warm and cold autoantibodies, which have a deleterious effect on red blood cells at either body temperature or at lower temperatures.

3A20.3 Paroxysmal cold haemoglobinuria

Definition of Paroxysmal cold haemoglobinuria: Paroxysmal cold hemoglobinuria is a very rare subtype of autoimmune haemolytic anaemia (AIHA), caused by the presence of cold-reacting autoantibodies in the blood and characterised by the sudden presence of hemoglobinuria, typically after exposure to cold temperatures. PCH is thought to account for at most 2-10% of cases of AIHA.

3A20.4 Alloimmune haemolytic anaemia

Definition of Alloimmune haemolytic anaemia: A disease caused by determinants such as a blood transfusion that lead to an immune response directed against the person’s own red blood cells. This disease is characterised by low levels of red blood cells in the body due to abnormal destruction of the red blood cells. 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.

Exclusions:

  • Haemolytic disease of fetus or newborn (KA84)

3A20.5 Evans syndrome

Definition of Evans syndrome: Evans syndrome is characterised by the association of autoimmune haemolytic anaemia with another haematological anomaly. The thrombocytopaenia may precede, occur concurrently with, or secondary to the autoimmune haemolytic anaemia.

3A20.Y Other specified acquired haemolytic anaemia immune

Acquired haemolytic anaemia non-immune

Definition of Acquired haemolytic anaemia non-immune: A disease caused by determinants such as infection, toxic chemicals, drugs and trauma arising after birth. This disease is characterised by haemolysis of red blood cells. This disease may present with pallor, fatigue, or shortness of breath. Confirmation is by identification of decreased red blood cell count in a blood sample and negative Coombs test result.

ICD 11 Code For Acquired haemolytic anaemia non-immune

  3A21  Acquired haemolytic anaemia non-immune

3A21.0 Paroxysmal nocturnal haemoglobinuria

Definition of Paroxysmal nocturnal haemoglobinuria: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder characterised by corpuscular haemolytic anaemia, bone marrow failure and frequent thrombotic events.

Exclusions:

  • haemoglobinuria NOS (MF94)
  • Aplastic anaemia with paroxysmal nocturnal haemoglobulinuria (3A70.1)

3A21.1 Microangiopathic haemolytic anaemia

Definition of Microangiopathic haemolytic anaemia: This is a microangiopathic subgroup of haemolytic anaemia (loss of red blood cells through destruction) caused by factors in the small blood vessels. It is identified by the finding of anaemia and schistocytes on microscopy of the blood film.

3A21.2 Haemolytic uraemic syndrome

Definition of Haemolytic uraemic syndrome: A disease caused by acquired haematopoietic stem cell mutation defect of the PIGA gene in the X chromosome leading to the premature break down of red blood cells. This disease is characterised by haemolytic anaemia, thrombosis and. This disease may present with haemoglobinuria (blood in the urine will be most noticeable in the morning, and clear as the day progresses), pallor, fatigue, or shortness of breath. Confirmation is by identification of genetic mutation in the PIGA gene through genetic testing.

Exclusions:

  • Hereditary haemolytic uraemic syndrome (3A10)

3A21.Y Other specified acquired haemolytic anaemia non-immune

Acquired haemolytic anaemia unspecified

ICD 11 Code For Acquired haemolytic anaemia unspecified

  3A2Z  Acquired haemolytic anaemia unspecified

Haemolytic anaemias unspecified

ICD 11 Code For Haemolytic anaemias unspecified

  3A4Z  Haemolytic anaemias unspecified

Thalassaemias

Definition of Thalassaemias: A disease caused by genetically inherited autosomal recessive mutations leading to abnormal production of haemoglobin. This disease is characterised by destruction of red blood cells leading to anaemia and abnormal production of haemoglobin. This disease may present with pallor, jaundice, iron overload, fatigue, or shortness of breath. Confirmation is by identification of mutations through genetic testing.

ICD 11 Code For Thalassaemias

  3A50  Thalassaemias

3A50.0 Alpha thalassaemia

Definition of Alpha thalassaemia: Alpha-thalassemia is an inherited haemoglobinopathy characterised by impaired synthesis of alpha-globin chains leading to a variable clinical picture depending on the number of affected alleles, and encompassing the alpha thalassaemia trait, haemoglobin H disease (HbH) and Bart’s hydrops fetalis.

Exclusions:

  • Hydrops fetalis due to haemolytic disease (KA85)

3A50.00 Mild alpha thalassaemia diseases

Definition of Mild alpha thalassaemia diseases: A disease caused by genetically inherited factors affecting the alpha chain of the haemoglobin molecule. This disease is characterised by structural abnormalities of the haemoglobin molecule. This disease may present with mild anaemia: pallor, fatigue, shortness of breath. Confirmation is by identification of changes to the alpha chain by genetic testing.

3A50.01 Thalassaemic alpha-chain variants

3A50.02 Haemoglobin H disease (– α/– – included)

Definition of Haemoglobin H disease (– α/– – included): Haemoglobin H (HbH) disease is a moderate to severe form of alpha-thalassemia characterised by pronounced microcytic hypochromic haemolytic anaemia.

3A50.03 Homozygous or compound heterozygous alpha thalassaemia

Definition of Homozygous or compound heterozygous alpha thalassaemia: Hb Bart’s hydrops fetalis is the most severe form of alpha-thalassemia and is almost always lethal. It is characterised by fetal onset of generalised oedema, pleural and pericardial effusions, and severe hypochromic anaemia.

3A50.0Y Other specified alpha thalassaemia

3A50.0Z Alpha thalassaemia unspecified

3A50.1 Alpha thalassaemia related syndromes

Definition of Alpha thalassaemia related syndromes: Alpha-thalassemia-related diseases refers to a group of diseases characterised by alpha-thalassemia and an associated disorder. Three conditions are included in this group: alpha-thalassemia-intellectual deficit syndrome, X-linked (or ATR-X syndrome), alpha-thalassemia-intellectual deficit syndrome and alpha-thalassemia-myelodysplastic disease or ATMDS.

3A50.2 Beta thalassaemia

Definition of Beta thalassaemia: Beta-thalassemia (BT) is a haemoglobinopathy characterised by deficiency (Beta+) or absence (Beta0) of synthesis of the beta globin chains of haemoglobin (Hb). Three main types of BT have been described: minor, intermedia and major with clinical presentation ranging from asymptomatic forms to microcytic anaemia and splenomegaly due to defective erythropoiesis and haemolysis.

3A50.3 Delta delta-beta or gamma-delta-beta thalassaemia

Definition of Delta delta-beta or gamma-delta-beta thalassaemia: Delta-beta-thalassemia is a form of beta-thalassemia characterised by decreased or absent synthesis of the delta- and beta-globin chains with a compensatory increase in expression of fetal gamma-chain synthesis.

3A50.4 Hereditary persistence of fetal haemoglobin

Definition of Hereditary persistence of fetal haemoglobin: Hereditary persistence of fetal haemoglobin (HPFH) associated with beta-thalassaemia is a haemoglobinopathy characterised by high haemoglobin (Hb)F levels and an increased number of fetal-Hb-containing-cells. The association of HPFH with beta-thalassaemia mitigates the clinical manifestations which vary from a normal state to beta-thalassaemia intermedia.

3A50.Y Other specified thalassaemias

3A50.Z Thalassaemias unspecified

Sickle cell disorders or other haemoglobinopathies

Definition of Sickle cell disorders or other haemoglobinopathies: Any disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may present with fatigue, shortness of breath, dizziness, headaches, pallor of skin or mucous membranes, and jaundice. This disorder is confirmed by identification of HbS mutation by genetic testing.

ICD 11 Code For Sickle cell disorders or other haemoglobinopathies

  3A51  Sickle cell disorders or other haemoglobinopathies

Coded Elsewhere:

  • Osteonecrosis due to haemoglobinopathy (FB81.4)
  • Other sickle-cell disorders with retinopathy (9B71.Y)

3A51.0 Sickle cell trait

Definition of Sickle cell trait: A disease caused by genetic inheritance of one abnormal allele of the haemoglobin gene. This disease does not display the severe symptoms of sickle cell disease that occurs in homozygous individuals. Confirmation is by identification of mutation through genetic testing.

3A51.1 Sickle cell disease without crisis

Definition of Sickle cell disease without crisis: A disorder caused by a HbS mutation in the haemoglobin gene. This disorder is characterised by abnormal rigid sickle-shaped red blood cells decreasing its ability to carry oxygen. This disorder may present with fatigue, shortness of breath, dizziness, headaches, pallor of skin or mucous membranes, and jaundice. This disorder is confirmed by identification of HbS mutation by genetic testing.

3A51.2 Sickle cell disease with crisis

Definition of Sickle cell disease with crisis: Sickle cell crisis occurs when the sickle cells block blood flow, thus decreasing oxygen delivery to the tissues. This results in intense to severe pain in the extremities, lower back, abdomen, and chest. A crisis can be brought on by illness, stress, dehydration, exposure to temperature changes or high altitudes.

Inclusions:

  • Hb-SS disease with crisis

3A51.3 Compound heterozygous sickling disorders without crisis

Definition of Compound heterozygous sickling disorders without crisis: A disease caused by genetic inheritance of two heterozygous recessive alleles of the haemoglobin gene leading to abnormal formation of haemoglobin molecule. This disease is characterised by rigid, sickle shaped red blood cells. Confirmation is by identification of mutations through genetic testing.

3A51.4 Compound heterozygous sickling disorders with crisis

Definition of Compound heterozygous sickling disorders with crisis: Compound heterozygous sickling disorders with crisis may present with acute chest syndrome, splenic sequestration, haemolytic crisis, and pain.

3A51.5 Haemoglobin C disease

Definition of Haemoglobin C disease: A disease caused by the bi-parental gene that encodes for haemoglobin C. This disease is characterised by abnormal structure of one of the globin chains of the haemoglobin molecule. This disease may present with mild haemolytic anaemia, increased risk for gallstones, enlarged spleen, episodes of joint pain, and increased risk of infection. This disease is confirmed by identification of the haemoglobin C gene by genetic testing.

Exclusions:

  • Hereditary persistence of fetal haemoglobin (3A50.4)

3A51.6 Haemoglobin D disease

Definition of Haemoglobin D disease: Haemoglobin D (Hb D) disease is characterised by mild haemolytic anaemia and mild to moderate splenomegaly. Prevalence is unknown. Heterozygous forms of Hb D are clinically silent. Molecular testing can be useful to distinguish Hb D homozygosity from cases of heterozygous Hb D in association with beta-(0) thalassaemia.

3A51.7 High affinity haemoglobin

Definition of High affinity haemoglobin: A disease caused by determinants arising after birth, in the antenatal period or by genetically inherited factors leading to high oxygen affinity haemoglobin. This disease is characterised by abnormalities the globin chains that alter the affinity of the haemoglobin molecule for oxygen, affecting the normal loading of oxygen in the lungs and delivery of oxygen to the tissues.

3A51.8 Low affinity haemoglobin

Definition of Low affinity haemoglobin: A disease caused by determinants arising after birth, in the antenatal period or by genetically inherited factors leading to low oxygen affinity haemoglobin. This disease is characterised by abnormalities the globin chains that alter the affinity of the haemoglobin molecule for oxygen, affecting the normal loading of oxygen in the lungs and delivery of oxygen to the tissues. This disease may present with fatigue, muscle weakness, loss of appetite, weight loss, diarrhoea, nausea, fast heartbeat or numbness in extremities.

3A51.9 Haemoglobin O disease

Definition of Haemoglobin O disease: A disease caused by the bi-parental inheritance of the gene that encodes for haemoglobin O. This disease is characterised by abnormal structure of one of the globin chains of the haemoglobin molecule. This disease may present with mild haemolytic anaemia, increased risk for gallstones, enlarged spleen, episodes of joint pain, and increased risk of infection. This disease is confirmed by identification of the haemoglobin O gene by genetic testing.

3A51.A Haemoglobin E disease

Definition of Haemoglobin E disease: Haemoglobin E disease is characterised by the synthesis of an abnormal haemoglobin called haemoglobin E (HbE), instead of the normal haemoglobin A (HbA). Subjects heterozygous for HbE (AE) have an asymptomatic condition with no clinical relevance, except for the risk of transmitting E/beta thalassemia if the other parent carries beta thalassemia. The severity of these E/beta thalassemia forms is very variable, the clinical picture ranging from that of beta thalassemia minor through to thalassemia intermedia to thalassemia major. Subjects homozygous for HbE (EE) are asymptomatic.

3A51.B Haemoglobin C/beta thalassaemia compound heterozygosity

Definition of Haemoglobin C/beta thalassaemia compound heterozygosity: Haemoglobin C/beta thalassaemia is a condition resulting from coinheritance of haemoglobin C and beta thalassaemia, both beta globin genes being mutated.

3A51.Y Other specified sickle cell disorders or other haemoglobinopathies

3A51.Z Sickle cell disorders or other haemoglobinopathies unspecified

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