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Name
Polymorphic transplant lymphoproliferative disorder, NOS (PTLD, NOS)
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)
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
(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.
Please 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.
PTLD is a lymphoid proliferation arising in a recipient of a solid organ transplant, allogenic bone marrow transplantation, or an umbilical cord blood transfusion. The patient must have a history of a solid organ transplant or an allogenic bone marrow transplant. The polymorphic PTLD is actually caused by the post-transplant T-cell immunosuppressant drugs. Most cases of polymorphic PTLD occur within a year of transplantation; however, they can occur anytime after the transplant.
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.
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.
Please 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.
PTLD is a lymphoid proliferation arising in a recipient of a solid organ transplant, allogenic bone marrow transplantation, or an umbilical cord blood transfusion. The patient must have a history of a solid organ transplant or an allogenic bone marrow transplant. The polymorphic PTLD is actually caused by the post-transplant T-cell immunosuppressant drugs. Most cases of polymorphic PTLD occur within a year of transplantation; however, they can occur anytime after the transplant.
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.
Grade
Not Applicable
Module Rule
None
Definition
Polymorphic post-transplant lymphoproliferative disorders (P-PTLDs) are composed of a heterogeneous population of immunoblasts, plasma cells, and small and intermediate-sized lymphoid cells that efface the architecture of lymph nodes or form destructive extranodal masses and do not fulfill the criteria for any of the recognized types of lymphoma described in immunocomponent hosts.
Definitive Diagnostic Methods
FISH
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Immunophenotyping
EBER+
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-9 Codes
238.77 Post-transplant lymphoproliferative disorder (PTLD)
Corresponding ICD-10 Codes
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
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Immunodeficiency-associated lymphoproliferative disorders
Pages: 453-462
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Immunodeficiency-associated lymphoproliferative disorders
Pages: 453-462
International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2
National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
