Polymorphic PTLD

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

Code grade specified by pathologist. If no grade specified, code 9
Module Rule
Alternate Names
Polymorphic B-cell PTLD
Polymorphic post-transplant lymphoproliferative disorder
These are morphologically polymorphic lesions composed of immunoblasts, plasma cells and small and intermediate sized lymphoid cells that efface the architecture of lymph nodes or form destructive extranodal masses. This is the most common PTLD in children and follows primary EBV infection.
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.)

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
Genetic testing
Histologic confirmation
Genetics Data
Clonally rearranged immunoglobulin genes
EBV positive
Mutated IGV
Other RX
Stem cell transplant
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
Epidemiology and Mortality
Incidence: 1-3% of patients who undergo transplantation. For children, frequently follows primary EBV infection.