Q112 – 181: Chronic Graft vs. Host Disease (GVHD)

*Autologous Transplants
If this was an autologous HCT, continue with the Infection Prophylaxis section of the form starting. Chimerism testing and graft-versus-host disease sections should only be completed for allogeneic HCTs.

*Combined Follow Up and Chronic GVHD
In scenarios where an HCT was given after a cellular therapy and this form is now being completed based on the subsequent HCT date, chronic GVHD will always be reported on the Post-Infusion Follow-Up (2100) Form and is disabled on the Cellular Therapy Essential Data Follow- Up (4100) Form.

*GVHD Reporting Instruction Overview
Review the GVHD Reporting Instruction Overview for detailed GVHD reporting instructions and various GVHD reporting scenarios.

Question 112: Did chronic GVHD develop?

Indicate whether a new clinical diagnosis of chronic GVHD was documented during the reporting period.

The Unknown option should only be used when there is no information about the recipient’s GVHD status for the entire reporting period. This option should be used sparingly and only when no judgement can be made about the presence or absence of GVHD in the reporting period.

For detailed instructions on whether a new development of chronic GVHD should be captured, review the GVHD Reporting Instruction Overview.

Question 113: Date of chronic GVHD diagnosis

Report the date of clinical diagnosis of chronic GVHD. The clinical diagnosis date may not necessarily be the date the symptoms began (example: the recipient developed shortness of breath one month prior to the clinical diagnosis of pulmonary chronic GVHD). If the clinical diagnosis is documented, but the diagnosis date is unclear, obtain documentation from the primary physician confirming the clinical diagnosis date.

If the recipient developed more than one episode of chronic GVHD in the same reporting period, report the date of onset of the first episode of chronic GVHD.

For more information regarding reporting partial or unknown dates, see General Instructions, General Guidelines for Completing Forms.

!Persistent GVHD and Day 100 Reporting Period
Previously, reporting Yes for Did chronic GVHD persist since the date of last report was not an applicable option for the Day 100 reporting period. However, if there was a prior infusion, the recipient developed chronic GVHD in the last reporting period of the previous infusion and chronic GVHD persisted into the Day 100 reporting period of the current infusion, report Yes, chronic GVHD persisted since the date of last report.

Question 114: Did chronic GVHD persist?

Indicate whether chronic GVHD was clinically diagnosed during a previous reporting period and persisted, with active symptoms, into the present reporting period. Do not report quiescent or inactive chronic GVHD, or a prior history of GVHD. For detailed instructions on how to report a flare of chronic GVHD, review the GVHD Reporting Instruction Overview.

Indicate Unknown if there is no information about the recipient’s GVHD status for the entire reporting period. This option should be used sparingly and only when no judgment can be made about the presence or absence of GVHD in the reporting period.

Review the GVHD Reporting Instruction Overview for various GVHD reporting examples.

Question 115: Onset of chronic GVHD was

Indicate whether the onset of chronic GVHD was:

• Progressive: Acute GVHD present within two weeks prior to onset of chronic GVHD
• Interrupted: Prior acute GVHD resolved for greater than two weeks, then chronic GVHD developed
• De novo: Acute GVHD never developed

Question 116: Were signs of acute GVHD present at the time of chronic GVHD diagnosis (overlap syndrome)?

Chronic GVHD can be separated into two different categories; classical chronic GVHD and overlap syndrome. Overlap syndrome is a condition where there are features of both acute and chronic GVHD at the time of diagnosis. Indicate whether signs of acute GVHD were present at the time of diagnosis of chronic GVHD (overlap syndrome). Refer to GVHD Reporting Instruction Overview instructions on how to complete the acute and chronic GVHD sections for recipients with overlap syndrome.

Question 117 – 119: What was the scale used to determine the recipient’s functional status? (at time of chronic GVHD diagnosis)

The Karnofsky Scale is designed for recipients aged 16 years and older and is not appropriate for children under the age of 16. The Lansky Scale is designed for recipients one year old to less than 16 years old. If the recipient is less than one year old, leave these questions blank.

Indicate the score (10-100) that best represents the recipient’s activity status at diagnosis of chronic GVHD. The only valid scores are 10-100, zero is not a valid response for this scale, nor are values not ending in zero, such as “85.” The Karnofsky/Lansky scale can be found in Appendix L: Karnofsky/Lansky Performance Status.

For further information on reporting Karnofsky / Lansky Scores refer to the instructions for reporting performance scores in the Functional Status section below.

Question 120: Platelets (at diagnosis of chronic GVHD)

Report the lowest platelet count recorded within 14 days (+ / -) of the diagnosis of chronic GVHD, whether or not the recipient has received a platelet transfusion. Indicate the units of measurement.

Question 121: Total serum bilirubin (at diagnosis of chronic GVHD)

Report the highest total serum bilirubin value (and units) within 14 days (+ / -) of the diagnosis of chronic GVHD. Indicate the units of measurement.

Question 122: Was chronic GVHD evaluated by biopsy (histology)? (at diagnosis)

Histological tests may be performed to confirm the clinical diagnosis of GVHD; however, the scoring of GVHD should be based on clinical evidence, not histological results.

Indicate if a biopsy was used to diagnose chronic GVHD. If a biopsy was not performed or it is unknown if performed at diagnosis, report No.

Questions 123 – 129: Specify result(s)

For each organ listed, indicate the test result documented on the pathology report as either Positive, Suggestive, Negative, Inconclusive / equivocal, or Not done.

Suggestive or Inconclusive / equivocal should be reported if in the final diagnosis or comments section of the pathology report, those words are used. Biopsy reports may use the term “consistent with GVHD” which could be either Positive or Suggestive, depending on the other comments in the report.

If the biopsy was performed on an “other site,” specify the site biopsied.

Questions 130 – 162: Specify organs involved and NIH scoring at diagnosis of chronic GVHD (check all that apply)

Report the organ involvement and NIH score of chronic GVHD for each organ / system listed at the time of diagnosis. For each involved organ, specify any features present at time of diagnosis. Refer to the Organ Scoring of Chronic GVHD Table in the GVHD Reporting Instruction Overview for organ scoring of chronic GVHD.

Signs or symptoms occurring at the time of diagnosis may be partially or entirely attributed to GVHD. Alternatively, reportable features may be observed at diagnosis but attributed entirely to non-GVHD causes. In any case, select the organ if any reportable signs / symptoms are documented during the reporting period regardless of whether those features are attributed to GVHD.

Features entirely explained by non-GVHD causes will be excluded when determining the overall severity of chronic GVHD but are still collected on the form. Spaces have been provided to document non-GVHD causes.

Specify all features observed at the time of diagnosis and report the score for each organ using the criteria from the Organ Scoring of Chronic GHVD Table located in the GVHD Reporting Instruction Overview. If any reported features are attributed entirely to non-GVHD causes, specify the non-GVHD cause(s) in the appropriate field. If a sign or symptom is caused by a combination of chronic GVHD and other causes, then the section on “non-GVHD causes” does not need to be completed. Further instruction has been provided GVHD Reporting Instruction Overview.

If a recipient has signs / symptoms of both acute and chronic GVHD during the reporting period, refer to the GVHD Reporting Instruction Overview for additional instructions.

Question 163: Maximum grade of chronic GVHD (according to best clinical judgment)

Report the maximum chronic GVHD involvement, based on the opinion of the clinician (i.e., clinical grade), since the date of the last report. The intent of this question is to capture the maximum grade based on the best clinical judgment. If both the global severity score and the score based on the clinician’s opinion is documented, report the clinician score. If the maximum clinical grade is not documented, request documentation from the recipient’s primary care provider.

Additional guidelines on reporting the maximum grade of chronic GVHD are outlined in the GVHD Reporting Instruction Overview.

Question 164: Date of maximum grade of chronic GVHD

Report the date of maximum chronic GVHD involvement during the current reporting period, based on clinical grade. If the recipient had multiple instances in which their GVHD reached the same maximum grade, report the earliest date.

Review the GVHD Reporting Instruction Overview for various GVHD reporting examples.

For more information regarding reporting partial or unknown dates, see General Instructions, General Guidelines for Completing Forms.

!Chronic GVHD Extent
This question is disabled and will be removed with a future revision.

Question 165: Specify if chronic GVHD was limited or extensive

Another grading system for chronic GVHD is divided into two categories: limited and extensive. Definitions are based on Sullivan KM, Blood 1981; 57:267.

Report the extent of chronic GVHD since the date of last report. Report Limited if chronic GVHD includes only localized skin involvement and/or liver dysfunction. Report Extensive if any of the following symptoms are attributed to chronic GVHD:

• Generalized skin involvement and / or liver dysfunction
• Liver histology showing chronic aggressive hepatitis, bridging necrosis, or cirrhosis
• Involvement of the eye: Schirmer’s test with < 5 mm wetting, or
• Involvement of minor salivary glands or oral mucosa demonstrated on labial biopsy (labial biopsy not required), or
• Involvement of any other target organ

The intent of this question is to capture if chronic GVHD was limited or extensive throughout the entire reporting period and is not dependent on the maximum grade and date of chronic GVHD. If the criteria to report extensive was met at any time in the reporting period, report Extensive.

Questions 166 – 167: Select other indicators, clinical features, or complications related to chronic GVHD (check all that apply)

Select other indicators, clinical features, or complications related to chronic GVHD (check all that apply)

• Ascites (serositis): Accumulation of fluid in the peritoneal cavity
• Pericardial effusion: Accumulation of fluid in the pericardial cavity
• Pleural effusion(s): Buildup of fluid between the chest and the tissues which line the lungs
• Nephrotic Syndrome: Kidney disorder that causes the body to excrete too much protein in the urine
• Myasthenia gravis: Weakness of muscles caused by antibodies to acetylcholine receptors
• Peripheral neuropathy: Nerve damage, usually in the hands and feet
• Polymyositis: Inflammation causing muscle weakness on both sides of the body
• Weight loss >5% without GI symptoms
• Eosinophilia: Elevation in eosinophils in the peripheral blood (> 500 cells / µL)
• Platelets: Decrease in platelets in the blood (< 100,000 / µL)
• Other indicator: If selected, specify the other indicator
  

If there were not any other indicators, clinical features, or complications related to chronic GVHD occurring in the current reporting period, select None.

Question 191: Corticosteroids (topical GI) (check all that apply)

Select all topical corticosteroids (beclomethasone and / or budesonide) used to treat GI GVHD. If topical corticosteroids to treat GI GVHD were not given, select None.

Topical therapies used for skin or lung GVHD are not captured in this question. Also, do not report systemic corticosteroids such as prednisone or dexamethasone. Systemic therapies are captured below.

*Reporting Multiple Systemic Therapies
Complete Select systemic treatment used to treat chronic GVHD through Specify other agent for each reported systemic therapy by adding an additional instance in the FormsNet3^SM application.

Questions 169 – 181: Select systemic treatment used to treat chronic GVHD

Select all systemic agents used to treat chronic GVHD during the reporting period, including any prophylactic medications continued after the diagnosis of chronic GVHD. If systemic therapy was not given for treatment of chronic GVHD, select None. Review the Chronic GVHD treatment reporting scenarios below for examples.

If systemic therapy was given to treat chronic GVHD during the reporting period, specify the drugs given and indicate if the treatment was continued from prophylaxis. If the drug was continued from prophylaxis or acute GVHD treatment, select Yes. If the drug was started in a prior reporting period and continued into the current reporting period, select Previously reported. The Previously reported option is not applicable for the Day 100 reporting period.

If the drug was not continued from prophylaxis / acute GVHD treatment and was not started in the prior reporting period and continued into the current reporting period, select No and report the therapy start date. When reporting the date started, report the first day the drug was given on or after the GVHD diagnosis date (reported Date of chronic GVHD diagnosis). If treatment is started and subsequently escalated during the same reporting period, report the earliest date treatment was actually given during the reporting period. Additionally, report the earliest start date if a drug is started multiple times during the same reporting period.

Report the total dose administered during the reporting period if a dose is required.

Refer to the acute GVHD treatment questions above for a description of most agents listed. Agents not described under acute GVHD are described below under Additional Agents. “Systemic” refers to drugs given by mouth, intramuscularly (IM), or intravenously (IV). “Topical” refers to drugs applied to the surface of skin or mouth, eye drops, or inhalation therapy. An exception to this would be the drug budesonide; it is a drug given by mouth for treatment of lower gut GVHD, but it is considered a “topical” drug since it is not absorbed.

Chronic GVHD Treatment Reporting Scenarios:

A. During the one-year reporting period, a recipient on cyclosporine for GVHD prophylaxis was diagnosed with chronic skin GVHD (5/1/2016). This was initially treated with topical steroids in addition to continuing their cyclosporine at the current dose. The chronic skin GVHD worsened shortly thereafter. On 5/15/2016, prednisone was started, and the dose of cyclosporine was increased. Symptoms persisted into the two-year reporting period but improved shortly thereafter. Upon resolution of symptoms, prednisone and cyclosporine doses were tapered.

One Year Post-HCT Data Form

Corticosteroids: Report No to indicate no topical GI corticosteroids were given. Topical steroids applied to the skin should not be reported here.
Select systemic treatment used to treat chronic GVHD: Select Cyclosporine to indicate systemic therapy was escalated to treat chronic GVHD.
Specify if the treatment was continued from prophylaxis / acute GVHD treatment: Report Yes to indicate cyclosporine was continued from prophylaxis / aGVHD treatment

Complete a second instance of the chronic GVHD treatment questions to capture the Corticosteroids, report No for Specify if the treatment was continued from prophylaxis / acute GVHD treatment and the start date as 5/15/2016.

Two Year Post-HCT Data Form

Corticosteroids: Report No to indicate no topical GI corticosteroids were given. Topical steroids applied to the skin should not be reported here.
Select systemic treatment used to treat chronic GVHD: Report two instances for Cyclosporine and Corticosteroids as treatment was continued into the two year reporting period and report Previously reported for Specify if the treatment was continued from prophylaxis / acute GVHD treatment.

B. During the one-year reporting period, a recipient on sirolimus for GVHD prophylaxis was diagnosed with chronic mouth and gut GVHD (7/1/2016). This was initially treated with topical steroids (oral dexamethasone and budesonide) in addition to continuing sirolimus at the current dose. Prednisone was started 7/30/2016 due to minimal improvement. The chronic mouth and gut GVHD resolved and dexamethasone, budesonide, as well as prednisone were discontinued. Sirolimus was continued. Later in the one-year reporting period, a severe flare of chronic gut GVHD occurred (10/15/2016). This was first treated by restarting prednisone on the date of diagnosis; however, no response was observed. Ruxolitinib was started on 10/20/2016 and symptoms resolved.

One Year Post-HCT Data Form

Corticosteroids: Report Yes to indicate topical GI corticosteroids were given. Budesonide should be reported here.
Select systemic treatment used to treat chronic GVHD: Report Sirolimus to indicate systemic therapy was given to treat chronic GVHD.
Specify if the treatment was continued from prophylaxis / acute GHVD treatment: Report Yes to indicate sirolimus was continued from prophylaxis / aGVHD treatment. Note, topical steroids, including dexamethasone and budesonide, should not be considered when completing the systemic chronic GVHD therapy questions.

Complete a second instance of chronic GVHD treatment questions to capture Ruxolitinib, report No for Specify if the treatment was continued from prophylaxis / acute GVHD treatment, and the start date as 10/20/2016.

Complete a third instance of chronic GVHD treatment questions to capture Corticosteroids, report No for Specify if the treatment was continued from prophylaxis / acute GVHD treatment, with a start date of 7/30/2016. Report the earliest start date if a medication is started multiple times during the reporting period.

C. During the six-month reporting period, a recipient off all immunosuppression was diagnosed with chronic mouth GVHD (9/15/2016). This was initially treated with topical steroids (oral dexamethasone). Cyclosporine was started on 9/20/2016 due to minimal response. Symptoms resolved by the one-year date of contact (10/1/2016) at which time dexamethasone was discontinued. The recipient remained on cyclosporine. During the one-year reporting period, a flare of chronic mouth GVHD occurred on 11/15/2016 while attempting to taper cyclosporine. This was treated by increasing the dose of cyclosporine on the date of diagnosis of the flare.

Six Month Post-HCT Data Form

Corticosteroids: Report No to indicate the recipient was initially treated with topical steroids.
Select systemic treatment used to treat chronic GVHD: Report Cyclosporine to indicate systemic therapy was given to treat chronic GVHD.
Specify if the treatment was continued from prophylaxis / acute GHVD treatment: Report No and specify 9/20/2016 as the treatment start date. This is the date cyclosporine was started as treatment for chronic GVHD. Note, topical steroids, including dexamethasone, budesonide, etc. should not be considered when completing systemic chronic GVHD treatment questions.

One Year Post-HCT Data Form

Corticosteroids: Report No to indicate the recipient was initially treated with topical steroids.
Select systemic treatment used to treat chronic GVHD: Report Cyclosporine to indicate systemic therapy was given to treat chronic GVHD.
Specify if the treatment was continued from prophylaxis / acute GHVD treatment: Report No and specify 11/15/2016 as the treatment start date.

Section Updates:

Question Number	Date of Change	Add/Remove/Modify	Description	Reasoning (If applicable)
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