Hodgkin lymphoma (HL or Hodgkin disease) is a cancer of the immune system that is marked by the presence of a type of cell called the Reed-Sternberg cell. The two major types of Hodgkin lymphoma are classical Hodgkin lymphoma (90-95% of cases) and nodular lymphocyte-predominant Hodgkin lymphoma (5-10% of cases).
Classical Hodgkin lymphoma can be further subdivided into four histologic subtypes: nodular sclerosis (NS), mixed cellularity (MC), lymphocyte deplete (LD), and lymphocyte rich (LR). Symptoms include the painless enlargement of lymph nodes, spleen, or other immune tissue. Generalized pruritus is also common and may precede the diagnosis by months. The most common sites of involvement include cervical, supraclavicular, and mediastinal lymph nodes. Central nervous system involvement may occur in rare cases. Other symptoms include fever, weight loss, fatigue, and/or night sweats.
Non-Hodgkin lymphoma (NHL) is a large group of cancers derived from lymphocytes (white blood cells). Non-Hodgkin lymphomas can occur at any age and are often marked by enlarged lymph nodes, fever, night sweats and weight loss. There are many different types of non-Hodgkin lymphoma. These types can be divided into aggressive (fast-growing), intermediate, or indolent (slow-growing) and can develop from either B-cells or T-cells. See Table 10.
Lymphomas that occur after bone marrow or stem cell transplantation are usually B-cell non-Hodgkin lymphomas and are collectively known as post-transplant lymphoproliferative disorders (PTLD).
Hodgkin Lymphoma (HL) and non-Hodgkin Lymphoma (NHL) are WHO disease classification subtypes of lymphoma. HL and NHL can transform into other disease subtypes. NHL can transform into other NHL subtypes, or into HL subtypes, but HL will rarely transform into NHL. Additionally, HL and NHL can occur at the same time and most likely classified as “B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin lymphoma”.
In order to complete the correct Disease Classification questions for a recipient who has a history of both HL and NHL, it is important to determine which disease is active prior to the start of the preparative regimen. A physician must make this determination.
The following two scenarios are examples of the data reporting practice for recipients with a combination of HL and NHL.
Scenario 1: A recipient is being transplanted for active NHL, but has a history of HL that is in remission at the start of the preparative regimen. Report the active NHL on the Disease Classification questions, and report HL as a prior malignancy on the Pre-TED Form (Form 2400).
Scenario 2: A recipient is being transplanted for both active NHL and active HL. Report this as NHL using “Other B-cell Lymphoma” and specify in question 269. Complete the Disease Classification questions for NHL. This only applies when the NHL and HL have been diagnosed at different times (i.e., two primaries).
Question 379-380: Specify the lymphoma histology (at infusion)
Indicate the histology for which the recipient is receiving a transplant or cellular therapy. If the histology is “Other B-cell lymphoma” or “Other T-cell / NK-cell lymphoma,” specify the histology in question 380.
Go to question 381 if either of the following histologies were reported in question 380:
- Diffuse, large B-cell lymphoma – Activated B-cell type (non-GCB)
- Diffuse, large B-cell lymphoma – Germinal center B-cell type
Otherwise, go to question 382.
Question 381: Assignment of DLBCL subtype:
DLBCL subtypes may be identified using different techniques including immunohistochemistry (IHC) and gene expression profiling. IHC involves staining a tissue sample and determining the presence of cell surface markers via microscopy. Gene expression profiling utilized molecular techniques.
Report the method used to determine the DLBCL subtype. Indicate “Unknown” if the method cannot be determined from the available source documentation.
Question 382: Is the lymphoma histology reported at transplant a transformation from CLL?
In some cases, CLL may evolve to a more aggressive diffuse large B-cell lymphoma (DLBCL). This is commonly referred to as Richter’s syndrome or Richter’s transformation. In a sub-set of CLL cases, the transformation may be to Hodgkin lymphoma (HL).
If the histology reported at infusion (question 379) is a transformation from CLL, indicate “Yes,” and go to question 383.
If the histology reported at infusion is not a transformation from CLL, indicate “No” and go to question 384.
Question 383: Was any 17p abnormality detected?
Report “Yes” if an abnormality was ever detected (by any method) on the short arm of chromosome 17 since the date of diagnosis of CLL. This includes any 17p abnormality detected after transformation to lymphoma and go to question 385. Report “No” if a 17p abnormality was not detected and go to question 388.
Question 384: Is the lymphoma histology reported at transplant a transformation from a different lymphoma histology (not CLL)?
Transformation may occur when a slow-growing lymphoma with an indolent clinical history changes to a more aggressive lymphoma histologically and clinically. An example of a common transformation would include follicular lymphoma evolving to a diffuse large B-cell lymphoma (DLBCL).
If a histologic transformation occurred after or concurrently with diagnosis, indicate “Yes” and go to question 386. If a histologic transformation did not occur, indicate “No” and go to question 388.
Question 385-386: Specify the original lymphoma histology (prior to transformation)
Report the histology of the recipient’s primary disease at diagnosis. If the histology is “Other B-cell lymphoma” or “Other T-cell / NK-cell lymphoma,” specify the histology in question 386.
Question 387: Date of original lymphoma diagnosis
Report the date of diagnosis for the histology specified in questions 385-386. If the exact pathological diagnosis date is not known, use the process described in General Instructions, General Guidelines for Completing Forms.
Question 388: Was a PET (or combination PET / CT) scan performed? (at last evaluation prior to the start of the preparative regimen / infusion)
Report “Yes” and go to question 389 if a PET scan was performed within three months prior to the start of the preparative regimen / infusion and meets the following criteria:
- Was performed within three months prior to the start of the preparative regimen / infusion and
- Was performed after the last pre-infusion line of therapy started
Combination PET / CT may also be reported, but a CT scan alone should not be captured here. Centers may report a PET scan performed during the most recent line of therapy so long as it is the most recent scan and was done within noted period. Report “No” and go to question 394 if a PET scan was not performed within this period.
Question 389: Was the PET (or PET / CT) scan positive for lymphoma involvement at any disease site?
Report “Yes” if the most recent PET scan prior to the start of the preparative regimen / infusion detected the recipient’s primary disease. Otherwise, report “No.”
Question 390-391: Date of PET scan
Questions 390-391 refer to the PET scan used to answer question 389. If the date of this PET scan is known, report “Known” and specify the date in question 391. If the date is only partially known (e.g., the month and year are known, but not the day) report “Known”, and use the process described in General Instructions, General Guidelines for Completing Forms to complete question 391. If the date cannot be determined / estimated, report “Unknown” and go to question 393.
Question 392-393: Deauville (five-point) score of the PET (or PET/CT) scan
Questions 392-393 refer to the PET scan used to answer question 388. Report whether the five-point PET score is known. This information is typically documented in the PET report. Consult the appropriate transplant physician if the results are unclear. If “Known,” report the score in question 393. Otherwise, report “Unknown” for question 392 and go to question 394. If the PET scan result is only documented as an ‘X’, report this as “Unknown” for question 392.
If multiple scores are documented, report the highest.
Question 394: What was the disease status?
The recipient’s pre-HCT disease status may be evaluated by a PET scan, CT scan, or both. If possible, complete question 394 using the metabolic (PET) criteria provided in the Lymphoma Response Criteria section of the manual. If it is not possible to use metabolic criteria to report the recipient’s disease (e.g., insufficient PET scan(s), non-PET-avid disease), use the radiographic criteria instead.
Indicate the disease status at the last evaluation prior to the start of the preparative regimen. When determining the disease status, compare the restaging assessments immediately prior to the preparative regimen to the assessments at baseline. “Baseline” is defined as the disease at diagnosis or at relapse/progression. When a transformation has occurred (e.g., follicular lymphoma (FL) transformed to DLBCL), count the response number (CR1, REL2, etc.) beginning with the transformed lymphoma (in this case the DLBCL). Do not include the responses to the lymphoma sub-type prior to the transformation.
Question 395: Total number of lines of therapy received (between diagnosis and HCT / infusion)
A single line of therapy refers to any agents administered during the same time period with the same intent (induction, consolidation, etc.). If a recipient’s disease status changes resulting in a change to treatment, this should be considered a new line of therapy. Additionally, if therapy is changed because a favorable disease response was not achieved, this should be considered a new line of therapy.
Indicate how many lines of therapy the recipient received prior to the start of the preparative regimen / infusion.
Question 396: Date assessed:
Enter the date of the most recent assessment of disease status prior to the start of the preparative regimen. Report the date imaging took place for the radiographic assessment (CT, MRI, PET, or PET/CT). Report the date the sample was collected for pathological evaluation (e.g., bone marrow biopsy). If no radiographic or pathologic assessment was performed within one month prior to transplant, report the most recent office visit in which the physician evaluated the recipient’s disease status.
If the exact date is not known, use the process for reporting partial or unknown dates as described in General Instructions, General Guidelines for Completing Forms.
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