Polymorphic PTLD

Name
Polymorphic PTLD
ICD-O-3 Morphology
9971/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
See Abstractor Notes

Help me code for diagnosis year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
M14 Module 1: PH1
Alternate Names
Polymorphic B-cell PTLD
Polymorphic post-transplant lymphoproliferative disorder
P-PTLD
PTLD
Post-transplant lymphoproliferative disorder
Definition
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, allogeneic bone marrow transplantation, or an umbilical cord blood transfusion.

There are two types of lymphoproliferative disorders: polymorphic and monomorphic.

Monomorphic PTLD is a PTLD that has an accompanying B-cell lymphoma, T-cell lymphoma, classical Hodgkin lymphoma, or a plasmacytoma. When there is both a PTLD and a lymphoma or plasmacytoma, the histology is coded to the lymphoma or plasmacytoma. The patient receives treatment for the lymphoma or plasmacytoma. See Rule M14 and PH1 in the Hematopoietic manual.

Polymorphic PTLD does not have an accompanying lymphoma or plasmacytoma. Polymorphic PTLD commonly involves lymph nodes, GI tract, lungs and liver. CNS involvement is rare; however in solid organ recipiants, the CNS may be the only site of involvement or may be associated with multi-organ involvement. Ceasing immunosupressing drugs commonly lead to a regression or complete absence of the PTLD..

The frequency of polymorphic Post-transplant lymphoproliferative disorder (P-PTLD) in children frequently follows primary EBV infection. Ceasing immunosuppresing drugs leads to a regression in a variable proportion of cases. Others progress and require treatment for lymphoma.

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.

Treatment for the polymorphic PTLD is the decrease or cessation of immunosuppressant drugs.
Definitive Diagnostic Methods
FISH
Histologic confirmation
Genetics Data
Clonally rearranged immunoglobulin genes
EBV positive
Mutated IGV
Immunophenotyping
EBER+
Treatments
Chemotherapy
Immunotherapy
Other RX
Radiation
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 (effective October 1, 2015 U.S. only)
D47.Z1 Post-transplant lymphoproliferative disorder (PTLD)
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None