Post Transplant Lymphoproliferative Disorder-PTLD

Description

Post Transplant Lymphoproliferative Disorder (PTLD) is a lymphoid proliferation arising in a recipient of a solid organ transplant, allogeneic bone marrow transplantation, or an umbilical cord blood transfusion. The patient must have a history of a solid organ transplant or an allogeneic bone marrow transplant. Both polymorphic and monomorphic PTLD are actually caused by post-transplant immunosuppression. Most cases of PTLD occur within a year of transplantation; however, they can occur any time after the transplant. Monomorphic PTLD may have histology indistinguishable from that of various malignant hematopoietic neoplasms, particularly lymphomas such as Diffuse Large B-cell Lymphoma.

Rationale

The presence of PTLD, either polymorphic or monomorphic, has clinical significance and prognostic value, especially in the Pediatric and Adolescence and Young Adult (AYA) populations.

Notes

**Note 1:** **Effective years** * This SSDI is effective for diagnosis years 2025+ * For cases diagnosed 2018-2024, this SSDI must be blank **Note 2:** **Definition of PTLD** * Post Transplant Lymphoproliferative Disorder (PTLD) is a lymphoid proliferation arising in a recipient of a solid organ transplant, allogeneic bone marrow transplantation, or an umbilical cord blood transfusion. * The development of PTLD is clinically significant and a prognostic indicator * See the Hematopoietic database for more information **Note 3:** **Types of PTLD** * **Polymorphic PTLD**: This is a PTLD by itself, there is no accompanying lymphoma, plasmacytoma or other type of Hematopoietic neoplasm. This type of PTLD is NOT collected in this data item. * **Monomorphic PTLD (Code 1)**: This is the most common PTLD and is associated with a malignant hematopoietic neoplasm. Most common, but not limited to, diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma * **(Classic Hodgkin lymphoma-PTLD type (Code 2)**: Under the microscope, Reed-Sternberg cells, which are associated with Hodgkin lymphoma are present * **PTLD, NOS (Code 4)**: Used when only PTLD (NOS) is documented and there is no mention of monomorphic or Hodgkin like type

Coding Guidelines

**See the Hematopoietic manual (Rules M14 and PH1. Hematopoietic Project - SEER Registrars (cancer.gov))** **1.** Code 0 when there is no mention of PTLD on the pathology report or medical record **2.** Code 1 when the pathology report describes monomorphic PTLD with a lymphoma **3.** Code 2 when the pathology report describes Classic Hodgkin lymphoma-PTLD type **4.** Code 4 when the pathology report describes a Burkitt like PTLD or doesn’t specify what type of PTLD ***Examples (Courtesy of Ask a SEER Registrar)*** **1.** Staining supports the diagnosis of PTLD, monomorphic type, EBV+ diffuse large b-cell lymphoma (non-germinal center) *(Code histology 9680/3, PTLD data item-1)* **2.** Monomorphic post-transplant lymphoproliferative disorder (Plasma cell myeloma), EBV negative. Monoclonal kappa plasmacytosis (estimated 20% by CD138 IHC). *(Code histology, 9732/3, PTLD data item: 1)* **3.** Per Physician, Stage IIA (bulk) PTLD - Hodgkin-like morphology, Intermediate risk. *(Code histology 9650/3, PTLD data item: 2)* **4.** High grade b cell lymphoma/Burkitt like post-transplant lymphoproliferative disorder. *(Code histology 9687/3, PTLD data item-4)* **5.** Non-Hodgkin malignant lymphoma with plasmablastic features. Represents a post-transplant/immunodeficiency-related malignant lymphoma. *(Code histology 9590/3, PTLD data item: 4)* **6.** Colonoscopy w/bx showed diffuse large B-cell lymphoma w/PTLD. *(Code histology 9680/3, PTLD data item 4)* **7.** Right inguinal lymph node biopsy that shows a CD20+ polymorphic PTLD *(Not collected in this schema. Effective 2025+, this would be abstracted as 9971/3 and collected in the HemeRetic schema)*

Default

8

NAACCR Item

NAACCR #1172

Metadata

SSDI 2025–
Code Description
0 PTLD not documented on the pathology report or in the medical record
1 Monomorphic PTLD * PTLD WITH a specified histology (lymphoma, plasmacytoma, plasma cell myeloma)
2 Classic Hodgkin lymphoma-PTLD type PTLD, Hodgkin like
4 PTLD not specified as monomorphic or Hodgkin lymphoma-PTLD type • WITH a specified histology (lymphoma, plasmacytoma, plasma cell myeloma) • Includes Burkitt type PTLD
8 Not applicable: Information not collected for this case (If this item is required by your standard setter, use of code 8 will result in an edit error)
<BLANK> Diagnosis year prior to 2025