Name

Polymorphic post-transplant lymphoproliferative disorders

ICD-O-3 Morphology

9971/3: Polymorphic Post Transplant Lymphoproliferative Disorder (PTLD)
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes, GI tract, lung and liver, CNS involvement (rare)

Grade

Not Applicable

Module Rule

None

Alternate Names

Early PTLD
Polymorphic B-cell PTLD
Polymorphic post-transplant lymphoproliferative disorder
Polymorphic PTLD
P-PTLD
PTLD

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.

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see codes 9970/1 or 9975/1.)

Note: Per the IARC/WHO Committee for ICD-O and included in the WHO Tumors of Hematopoietic Lymphoid Tissues, Revised 4th Ed, Volume 2, only 9971/1 is now assigned /1 behavior. This change has not been approved by the United States and Canada. Continue to collect PTLD as a 9971/3.

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.

Definitive Diagnostic Methods

FISH
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Clonally rearranged immunoglobulin genes
EBV positive
Mutated IGV

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 unc/unk beh of lymphoid, hematopoietic

Corresponding ICD-10-CM Codes (U.S. only)

D47.Z1 Post-transplant lymphoproliferative disorder (PTLD) (effective October 01, 2015)

Signs and Symptoms

Enlarged tonsils
Weight loss

Diagnostic Exams

CT (CAT) scan
Complete blood count (CBC)
MRI (magnetic resonance imaging)
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

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

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary