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Name
ICD-O-3 Morphology
9971/3: Polymorphic Post Transplant Lymphoproliferative Disorder (PTLD)
Effective
2010 and later
Reportable
2010-2020, 2025 and later; see Abstractor Notes
Primary Site(s)
See Module 7
Most common sites of involvement: lymph nodes, GI tract, lung and liver, CNS involvement (rare)
See abstractor notes
See abstractor notes
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Post-transplant lymphoproliferative disorder, NOS is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)
(This code is effective for cases diagnosed 2010-2020 and 2025+. Cases diagnosed prior to 2010 are not reportable.
For 2021-2024, Polymorphic PTLD (PTLD by itself, no accompanying lymphoma, plasmacytoma, multiple myeloma) is coded as 9971/1 and is only reportable for CNS sites (C700-C729, C751-C753)
Per the IARC/WHO Committee for ICD-O and included in the WHO Tumors of Hematopoietic and Lymphoid Tissues, Revised 4th Ed, Volume 2, PTLD by itself is a /1 starting in ICD-O-3.2. For the US and Canada, 9971/1 will be reportable for years 2021-2024 for CNS sites only.
Starting 1/1/25, PTLD (by itself [polymorphic]), will be reportable for any site and is to be coded as 9971/3.
Note that ICD-O-3.2 has this as 9971/1; however, the US will be collecting this as 9971/3 starting 1/1/25.
Polymorphic LPDs mainly involve lymph nodes, although other lymphoid organs (e.g. tonsils) and a variety of extranodal sites (e.g. lung, gastrointestinal tract, liver, and CNS) can be involved.
PTLD can arise in immunodeficiency settings but are mostly seen following transplantation. Transplants can be hematopoietic stem cell or solid-organ transplants.
There are two types of PTLD:
1. Monomorphic - PTLD with an accompanying B-cell lymphoma, T-cell lymphoma, classical Hodgkin lymphoma, or plasmacytoma. When both a PTLD and lymphoma or plasmacytoma occur, the histology is coded to the lymphoma or plasmacytoma. Treatment is given for the lymphoma or plasmacytoma. See Rule M14 and PH1 in the Hematopoietic manual.
2. Polymorphic: PTLD with no accompanying lymphoma or plasmacytoma. In solid organ recipients, the CNS may be the only site of involvement or may be associated with multi-organ involvement.
Treatment for the polymorphic PTLD is the decrease or cessation of immunosuppressant drugs. This commonly leads to a regression or complete absence of the PTLD.
(This code is effective for cases diagnosed 2010-2020 and 2025+. Cases diagnosed prior to 2010 are not reportable.
For 2021-2024, Polymorphic PTLD (PTLD by itself, no accompanying lymphoma, plasmacytoma, multiple myeloma) is coded as 9971/1 and is only reportable for CNS sites (C700-C729, C751-C753)
Per the IARC/WHO Committee for ICD-O and included in the WHO Tumors of Hematopoietic and Lymphoid Tissues, Revised 4th Ed, Volume 2, PTLD by itself is a /1 starting in ICD-O-3.2. For the US and Canada, 9971/1 will be reportable for years 2021-2024 for CNS sites only.
Starting 1/1/25, PTLD (by itself [polymorphic]), will be reportable for any site and is to be coded as 9971/3.
Note that ICD-O-3.2 has this as 9971/1; however, the US will be collecting this as 9971/3 starting 1/1/25.
Polymorphic LPDs mainly involve lymph nodes, although other lymphoid organs (e.g. tonsils) and a variety of extranodal sites (e.g. lung, gastrointestinal tract, liver, and CNS) can be involved.
PTLD can arise in immunodeficiency settings but are mostly seen following transplantation. Transplants can be hematopoietic stem cell or solid-organ transplants.
There are two types of PTLD:
1. Monomorphic - PTLD with an accompanying B-cell lymphoma, T-cell lymphoma, classical Hodgkin lymphoma, or plasmacytoma. When both a PTLD and lymphoma or plasmacytoma occur, the histology is coded to the lymphoma or plasmacytoma. Treatment is given for the lymphoma or plasmacytoma. See Rule M14 and PH1 in the Hematopoietic manual.
2. Polymorphic: PTLD with no accompanying lymphoma or plasmacytoma. In solid organ recipients, the CNS may be the only site of involvement or may be associated with multi-organ involvement.
Treatment for the polymorphic PTLD is the decrease or cessation of immunosuppressant drugs. This commonly leads to a regression or complete absence of the PTLD.
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Module Rule
None
Alternate Names
Polymorphic post-transplant lymphoproliferative disorder (Polymorphic PTLD) (see 9971/1 prior to 2025)
Polymorphic lymphoproliferative disorders arising in immune deficiency/dysregulation (see 9971/1 prior to 2025)
Definition
Polymorphic lymphoproliferative disorders (LPDs) arise in patients with immune deficiency or dysregulation and are composed of a heterogeneous lymphoid cell infiltrate with variable numbers of B cells exhibiting a full spectrum of B-cell differentiation that efface the architecture of involved tissues. Large B cells, including HRS-like cells, may be present. Polymorphic LPDs are monoclonal or oligoclonal, usually EBV-driven B-cell proliferations that have no or only a few genetic alterations. (WHO 5th edition)
Definitive Diagnostic Methods
Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping
Genetics Data
Monoclonal or oligoclonal IG rearrangements
TR gene rearrangements
Immunophenotyping
CD20+ (expression/positive)
CD30+ (expression/positive)
EBV+ (expression/positive)
PAX5+ (expression/positive)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
Other therapy
Radiation therapy
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
There are no known same primaries
Corresponding ICD-10 Codes (Cause of Death codes only)
D47.7 Other specified neoplasms of uncertain or unknown behavior of lymphoid, hematopoietic, and related tissue
Corresponding ICD-10-CM Codes (U.S. only)
D47.Z1 Post-transplant lymphoproliferative disorder (PTLD) (effective October 01, 2015)
Signs and Symptoms
Diagnostic Exams
Biopsy
Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Immunophenotyping
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Incidence: 1-3% of patients who undergo transplantation. For children, frequently follows primary EBV infection.
Sources
WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Lymphoid proliferations and lymphomas associated with immune deficiency and dysregulation
Pages: Part B: 561-564
Section: Lymphoid proliferations and lymphomas associated with immune deficiency and dysregulation
Pages: Part B: 561-564
International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
