D47 Other neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue

Codes in D47

1. D47.0
   Mast cell neoplasms of uncertain behavior

   Header

   Excludes1 3

   Excludes1

   • congenital cutaneous mastocytosis (Q82.2) Q82.2
   • histiocytic neoplasms of uncertain behavior (D47.Z9) D47.Z9
   • malignant mast cell neoplasm (C96.2-) C96.2
2. D47.01
   Cutaneous mastocytosis

   BillableCC

   Inclusion terms 5, Excludes1 3

   Inclusion terms

   • Diffuse cutaneous mastocytosis
   • Maculopapular cutaneous mastocytosis
   • Solitary mastocytoma
   • Telangiectasia macularis eruptiva perstans
   • Urticaria pigmentosa

   Excludes1

   • congenital (diffuse) (maculopapular) cutaneous mastocytosis (Q82.2) Q82.2
   • congenital urticaria pigmentosa (Q82.2) Q82.2
   • extracutaneous mastocytoma (D47.09) D47.09
3. D47.02
   Systemic mastocytosis

   BillableCC

   Inclusion terms 4, Excludes1 2, Code also 10

   Inclusion terms

   • Indolent systemic mastocytosis
   • Isolated bone marrow mastocytosis
   • Smoldering systemic mastocytosis
   • Systemic mastocytosis, with an associated hematological non-mast cell lineage disease (SM-AHNMD)

   Excludes1

   • aggressive systemic mastocytosis (C96.21) C96.21
   • mast cell leukemia (C94.3-) C94.3

   Code also

   • , if applicable, any associated hematological non-mast cell lineage disease, such as:
   • acute myeloid leukemia (C92.6-, C92.A-) C92.6 C92.A
   • chronic myelomonocytic leukemia (C93.1-) C93.1
   • essential thrombocytosis (D47.3) D47.3
   • hypereosinophilic syndrome (D72.1) D72.1
   • myelodysplastic syndrome (D46.9) D46.9
   • myeloproliferative syndrome (D47.1) D47.1
   • non-Hodgkin lymphoma (C82-C85) C82 C85
   • plasma cell myeloma (C90.0-) C90.0
   • polycythemia vera (D45) D45
4. D47.09
   Other mast cell neoplasms of uncertain behavior

   BillableCC

   Inclusion terms 4

   Inclusion terms

   • Extracutaneous mastocytoma
   • Mast cell tumor NOS
   • Mastocytoma NOS
   • Mastocytosis NOS
5. D47.1
   Chronic myeloproliferative disease

   BillableCC21

   Inclusion terms 2, Excludes1 6

   Inclusion terms

   • Chronic neutrophilic leukemia
   • Myeloproliferative disease, unspecified

   Excludes1

   • atypical chronic myeloid leukemia BCR/ABL-negative (C92.2-) C92.2
   • chronic myeloid leukemia BCR/ABL-positive (C92.1-) C92.1
   • myelofibrosis NOS (D75.81) D75.81
   • myelophthisic anemia (D61.82) D61.82
   • myelophthisis (D61.82) D61.82
   • secondary myelofibrosis NOS (D75.81) D75.81
6. D47.2
   Monoclonal gammopathy

   Billable

   Inclusion terms 1

   Inclusion terms

   • Monoclonal gammopathy of undetermined significance [MGUS]
7. D47.3
   Essential (hemorrhagic) thrombocythemia

   Billable23

   Inclusion terms 3, Excludes2 4

   Inclusion terms

   • Essential thrombocytosis
   • Idiopathic hemorrhagic thrombocythemia
   • Primary thrombocytosis

   Excludes2

   • reactive thrombocytosis (D75.838) D75.838
   • secondary thrombocytosis (D75.838) D75.838
   • thrombocythemia NOS (D75.839) D75.839
   • thrombocytosis NOS (D75.839) D75.839
8. D47.4
   Osteomyelofibrosis

   Billable19

   Inclusion terms 4, Excludes1 1

   Inclusion terms

   • Chronic idiopathic myelofibrosis
   • Myelofibrosis (idiopathic) (with myeloid metaplasia)
   • Myelosclerosis (megakaryocytic) with myeloid metaplasia
   • Secondary myelofibrosis in myeloproliferative disease

   Excludes1

   • acute myelofibrosis (C94.4-) C94.4
9. D47.Z
   Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue

   Header
10. D47.Z1
    Post-transplant lymphoproliferative disorder (PTLD)

    BillableCC21

    Code first 1

    Code first

    • complications of transplanted organs and tissue (T86.-) T86
11. D47.Z2
    Castleman disease

    BillableCC21

    Excludes2 1, Code also 1

    Excludes2

    • Kaposi's sarcoma (C46.-) C46

    Code also

    • , if applicable, human herpesvirus 8 infection (B10.89) B10.89
12. D47.Z9
    Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue

    BillableCC21

    Inclusion terms 1

    Inclusion terms

    • Histiocytic tumors of uncertain behavior
13. D47.9
    Neoplasm of uncertain behavior of lymphoid, hematopoietic and related tissue, unspecified

    BillableCC21

    Inclusion terms 1

    Inclusion terms

    • Lymphoproliferative disease NOS

CC/MCC: as a secondary diagnosis, unless the principal diagnosis is on the code’s PDX exclusion list (MS-DRG v44 Appendix C; list on the code card).

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