ICD 11

Myeloid And Lymphoid Neoplasms With Eosinophilia And Abnormalities Of PDGFRA PDGFRB or FGFR1 – Definitions & ICD 11 Codes


Definition of Myeloid And Lymphoid Neoplasms With Eosinophilia And Abnormalities Of PDGFRA PDGFRB or FGFR1: A group of rare myeloid and lymphoid neoplasms characterised by rearrangement of the PDGFRA, PDGFRB, or FGFR1 genes, resulting in the formation of fusion transcripts and aberrant tyrosine kinase activity. Eosinophilia is a characteristic finding but it is not always present.

Myeloid neoplasm associated with PDGFRA rearrangement

ICD 11 Codes For Myeloid neoplasm associated with PDGFRA rearrangement

  2A50  Myeloid neoplasm associated with PDGFRA rearrangement


Myeloid neoplasm associated with PDGFRB rearrangement

Definition of Myeloid neoplasm associated with PDGFRB rearrangement: Myeloid neoplasms characterised by the rearrangement of the PDGFRB gene. Patients usually present with a picture resembling chronic myelomonocytic leukaemia and, less often atypical chronic myeloid leukaemia or chronic eosinophilic leukaemia.

ICD 11 Codes For Myeloid neoplasm associated with PDGFRB rearrangement

  2A51  Myeloid neoplasm associated with PDGFRB rearrangement

Myeloid or lymphoid neoplasms with FGFR1 abnormalities

Definition of Myeloid or lymphoid neoplasms with FGFR1 abnormalities: Hematologic neoplasms characterised by the rearrangement of the FGFR1 gene, resulting in translocations with an 8p11 breakpoint. Patients may present with a myeloproliferative neoplasm, acute myeloid leukaemia, lymphoblastic lymphoma/leukaemia of T or B-cell lineage, or acute leukaemia of mixed phenotype.

ICD 11 Codes For Myeloid or lymphoid neoplasms with FGFR1 abnormalities

  2A52  Myeloid or lymphoid neoplasms with FGFR1 abnormalities

Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA PDGFRB or FGFR1 unspecified

ICD 11 Codes For Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA PDGFRB or FGFR1 unspecified

  2A5Z  Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA PDGFRB or FGFR1 unspecified

Acute myeloid leukaemias and related precursor neoplasms

Definition of Acute myeloid leukaemias and related precursor neoplasms: Acute myeloid leukaemia is characterised by clonal expansion of myeloid blasts in the peripheral blood and bone marrow. Clinical manifestations are fever, pallor, anaemia, hemorrhages and recurrent infections.

ICD 11 Codes For Acute myeloid leukaemias and related precursor neoplasms

  2A60  Acute myeloid leukaemias and related precursor neoplasms

2A60.0 Acute myeloid leukaemia with recurrent genetic abnormalities

2A60.1 Acute myeloid leukaemia with myelodysplasia-related changes

Definition of Acute myeloid leukaemia with myelodysplasia-related changes: An acute myeloid leukaemia with at least 20% blasts in the bone marrow or blood, and either a previous history of myelodysplastic syndrome, multilineage dysplasia or typical myelodysplastic syndrome-related cytogenetic abnormalities. There is no history of prior cytotoxic therapy for an unrelated disorder, and there is absence of the genetic abnormalities that are present in acute myeloid leukaemia with recurrent genetic abnormalities.

2A60.2 Therapy-related myeloid neoplasms

Inclusions:

2A60.20 Therapy related acute myeloid leukaemia or myelodysplastic syndrome

2A60.2Y Other specified therapy-related myeloid neoplasms

2A60.2Z Therapy-related myeloid neoplasms unspecified

2A60.3 Acute myeloid leukaemia not elsewhere classified by criteria of other types

Definition of Acute myeloid leukaemia not elsewhere classified by criteria of other types: Acute myeloid leukaemias specified by morphological criteria should only be classified as such, if recurrent genetic abnormalities, prior history of a myelodysplastic syndrome or myelodysplastic/myeloproliferative neoplasm, or history of cytotoxic chemotherapy and/or radiotherapy are absent.

Exclusions:

  • Acute myeloid leukaemia with recurrent genetic abnormalities (2A60.0)
  • Therapy-related myeloid neoplasms (2A60.2)
  • Acute myeloid leukaemia with myelodysplasia-related changes (2A60.1)

2A60.30 Acute myeloid leukaemia with minimal differentiation

Definition of Acute myeloid leukaemia with minimal differentiation: An acute myeloid leukaemia (AML) in which the blasts do not show evidence of myeloid differentiation by morphology and conventional cytochemistry.

2A60.31 Acute myeloid leukaemia without maturation

Definition of Acute myeloid leukaemia without maturation: An acute myeloid leukaemia (AML) characterised by blasts without evidence of maturation to more mature neutrophils.

2A60.32 Acute myeloid leukaemia with maturation

Definition of Acute myeloid leukaemia with maturation: An acute myeloid leukaemia (AML) characterised by blasts with evidence of maturation to more mature neutrophils.

2A60.33 Acute myelomonocytic leukaemia

Definition of Acute myelomonocytic leukaemia: An acute leukaemia characterised by the proliferation of both neutrophil and monocyte precursors.

2A60.34 Acute monoblastic or monocytic leukaemia

Definition of Acute monoblastic or monocytic leukaemia: Acute monoblastic leukaemia and acute monocytic leukaemia are myeloid leukaemias in which 80% or more of the leukaemic cells are of monocytic lineage including monoblasts, promonocytes and monocytes; a minor neutrophil component, <20%, may be present.

2A60.35 Acute erythroid leukaemia

Inclusions:

  • Erythroleukaemia

2A60.36 Acute megakaryoblastic leukaemia

Definition of Acute megakaryoblastic leukaemia: An acute myeloid leukaemia in which at least 50% of the blasts are of megakaryocytic lineage.

Inclusions:

2A60.37 Acute basophilic leukaemia

Definition of Acute basophilic leukaemia: An acute myeloid leukaemia in which the immature cells differentiate towards basophils. This is a rare leukaemia.

2A60.38 Acute panmyelosis with myelofibrosis

Definition of Acute panmyelosis with myelofibrosis: An acute myeloid leukaemia characterised by bone marrow fibrosis without preexisting primary myelofibrosis.

Inclusions:

  • Acute myelofibrosis

Exclusions:

  • Cases that meet criteria for AML with myelodysplasia related changes (2A60.1)

2A60.39 Myeloid sarcoma

Definition of Myeloid sarcoma: Myeloid sarcoma is a rare solid tumour of the myelogenous cells occurring in an extramedullary site.

Inclusions:

  • Chloroma
  • Granulocytic sarcoma

2A60.3Y Other specified acute myeloid leukaemia not elsewhere classified by criteria of other types

2A60.3Z Acute myeloid leukaemia unspecified

2A60.4 Myeloid proliferation associated with Down syndrome

Definition of Myeloid proliferation associated with Down syndrome: Myeloid neoplasms occurring in individuals with Down syndrome. There is an increased risk of acute leukaemias in both children and adults with Down syndrome. In particular, the incidence of acute myeloid leukaemia in Down syndrome children of less than five years of age is particularly high, it is usually an acute megakaryoblastic leukaemia, and is associated with GATA1 gene mutation. This group of disorders also includes the entity transient abnormal myelopoiesis which occurs in neonates and is associated with GATA1 gene mutation.

2A60.40 Transient abnormal myelopoiesis

Definition of Transient abnormal myelopoiesis: A myeloid proliferation occurring in newborns with Down syndrome. It is clinically and morphologically indistinguishable from acute myeloid leukaemia and is associated with GATA1 mutations. The blasts display morphologic and immunophenotypic features of megakaryocytic lineage. In the majority of patients the myeloid proliferation undergoes spontaneous remission.

2A60.41 Myeloid leukaemia associated with Down syndrome

Definition of Myeloid leukaemia associated with Down syndrome: Leukaemia of children with Down syndrome. Encompasses both MDS and AML

2A60.4Y Other specified myeloid proliferation associated with Down syndrome

2A60.4Z Myeloid proliferation associated with Down syndrome unspecified

2A60.5 Blastic plasmacytoid dendritic cell neoplasm

Definition of Blastic plasmacytoid dendritic cell neoplasm: An aggressive immature hematologic neoplasm formerly known as blastic NK cell lymphoma, composed of cells with a lymphoblast-like morphology. Recent evidence suggests derivation from a plasmacytoid dendritic cell precursor. Patients present with cutaneous tumours and bone marrow involvement.

Inclusions:

  • Blastic NK-cell lymphoma

2A60.Y Other specified acute myeloid leukaemias and related precursor neoplasms

2A60.Z Acute myeloid leukaemias and related precursor neoplasms unspecified

Acute leukaemias of ambiguous lineage

Definition of Acute leukaemias of ambiguous lineage: An acute leukaemia in which the blasts lack sufficient evidence to classify as myeloid or lymphoid or they have morphologic and/or immunophenotypic characteristics of both myeloid and lymphoid cells.

ICD 11 Codes For Acute leukaemias of ambiguous lineage

  2A61  Acute leukaemias of ambiguous lineage

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