Question 1: Date of diagnosis of primary disease for infusion
Use the following guidelines if the recipient was diagnosed with a non-malignant disease:
- Newborn screening: If the diagnosis was made using a newborn screening, report the date of birth as the diagnosis date.
- Genetic testing: If the recipient was not diagnosed with a newborn screening, report the date of genetic testing that confirmed the diagnosis.
- Other Definitive Assessment: If genetic testing was not completed, report the date of the other definitive assessment (i.e., electrophoresis, flow cytometry, etc.), that confirmed the diagnosis.
- Diagnosis by exclusion: If the diagnosis was made by exclusion (i.e., all assessments returned normal and the diagnosis made clinically), report the date of the clinical diagnosis as documented by the physician.
- Diagnosis at an outside center: If the diagnosis was completed at an outside center (the confirmatory test such as genetic testing, another definitive test, or clinical diagnosis) and the HCT / CT center performs their own confirmatory testing, report the date of the initial confirmatory test as the diagnosis date.
- If the exact date is not known or documentation is limited, report an estimated date.
Enter the date the sample was collected for examination. If the diagnosis was determined at an outside center, and no documentation of a pathological or laboratory assessment is available, the dictated date of diagnosis within a physician note may be reported. Do not report the date symptoms first appeared.
If the exact diagnosis date is not known, use the process described in General Instructions, Guidelines for Completing Forms.
Questions 566 – 568: Specify the hemoglobinopathy classification
Indicate the hemoglobinopathy classification of the primary disease for infusion.
- Sickle cell disease: A group of disorders that adversely affect the body’s production of hemoglobin, the component in red blood cells that delivers oxygen throughout the body. Individuals with these disorders possess atypical hemoglobin molecules, called hemoglobin “S,” which can distort the red blood cell morphology into a sickle, or crescent, shape.
- Transfusion dependent thalassemia: Previously described as “beta thalassemia major” on CIBMTR forms. Transfusion-dependent thalassemia is a blood disorder that reduces the production of hemoglobin in the body and is defined as requiring eight or more transfusion events per year for two years or more for treatment of symptomatic anemia.
If Transfusion dependent thalassemia is selected, specify if the disease is Transfusion dependent beta thalassemia (known as “beta thalassemia major” on prior CIBMTR forms) or Other transfusion dependent thalassemia.
If the recipient is diagnosed with a hemoglobinopathy other than sickle cell disease or transfusion dependent thalassemia, select Other hemoglobinopathy and specify the type.
Questions 569 – 571: Was tricuspid regurgitant jet velocity (TRJV) measured by echocardiography?
Tricuspid regurgitant jet velocity (TRJV) measurements are used in determining the pulmonary artery pressure for patients with hemolytic disorders. An elevated TRJV is an indication of pulmonary hypertension, a condition common in adults with hemolytic diseases. TRJV can be determined by echocardiography (ECHO); this information is typically documented in the echocardiogram report.
Report whether the TRJV was measured by echocardiography prior to the start of the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy). Seek physician clarification, as needed, if the results are unclear.
If the TRJV was measured by echocardiography, select Yes and indicate if the TRJV value is known. If Known, specify the TRJV value documented in the laboratory report. If the TRJV was measured multiple times prior to the start of the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy) by an echocardiography, report the most recent value.
If the TRJV was not measured or it is not known if measured by echocardiography, report No or Unknown, respectively.
Questions 572 – 574: Was liver iron content (LIC) tested within 6 months prior to infusion?
Transfusion support for hemolytic diseases can often lead to iron build up or accumulation in the liver and other target organs. Liver iron content (LIC) is commonly used to measure total iron storage for recipients at risk of hemosiderosis. LIC is a more sensitive method of testing for measuring the level of iron in the liver. Common methods include, but are not limited to, liver biopsy, T2*MRI, and FerriScan.
Specify if the liver iron content was assessed within six months prior to the start of the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy). If Yes, report the value, unit of measure documented in the laboratory report, and the method used to estimate the LIC. If the method of assessment is not listed, select Other.
Report No if liver iron content was not assessed or it is not known if assessed within six months prior to the start of the preparative regimen / infusion.
Questions 575 – 576: Is the recipient red blood cell transfusion dependent? (requiring transfusion to maintain HGB 9 – 10 g/dL)
Indicate if the recipient is red blood cell transfusion dependent at any time prior to the start of the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy). In this context, “red blood cell transfusion dependent” is defined as requiring transfusions to maintain hemoglobin 9 – 10 d / dL.
If the recipient the recipient was red blood cell transfusion dependent at any time prior to the start of the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy), report Yes and specify the year of the first transfusion (since diagnosis).
If the recipient was never red blood cell transfusion dependent at any time prior to the start of the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy) or it is unknown, report No.
Question 577: Was iron chelation therapy given at any time since diagnosis?
Iron chelation therapy is commonly used for recipients to prevent or reduce iron overload. Iron chelation therapy is the removal of excess iron from the body using drugs such as Deferrioxamine (Desferal) or Deferasirox (Jadenu, Exjade).
Specify if iron chelation therapy was given at any time since diagnosis. If iron chelation was not given or it is unknown whether iron chelation therapy was given, select No or Unknown, respectively.
Question 578: Did iron chelation therapy meet the following criteria: initiated within 18 months of the first transfusion and administered for at least 5 days / week (either oral or parenteral iron chelation medication)
Indicate if the iron chelation therapy was initiated within 18 months of the first transfusion and administered for at least five days a week (either oral or parenteral iron chelation medication).
If iron chelation therapy met the criteria listed above, report Yes, iron chelation therapy given as specified.
If iron chelation therapy was given but does not meet the specified criteria, report No, iron chelation therapy given, but not meeting criteria listed.
If iron chelation therapy was given but administration details are unavailable, report Iron chelation therapy given, but details of administration unknown.
Questions 579 – 580: Specify reason criteria not met
If iron chelation therapy was given but does not meet the criteria specified above, indicate why the criteria was not met. If the reason is not listed, report Other and specify the reason criteria were not met.
Questions 581 – 582: Year iron chelation therapy started
Indicate if the year iron chelation therapy was started is known. If Known, specify the year when iron chelation therapy began.
If the exact date is not known, use the process described in General Instructions, Guidelines for Completing Forms.
Questions 583 – 584: Did the recipient have hepatomegaly?
Indicate if the recipient had hepatomegaly (i.e. abnormal enlargement of the liver) at the last evaluation prior to the start of the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy). Hepatomegaly is often documented during the physician’s physical assessment of the recipient and represents an abnormal finding. If Yes, report the liver measurement (in centimeters below the right costal margin).
If hepatomegaly was not present or is not known if it was present at the last evaluation prior to the start of the preparative regimen, select No.
Questions 585 – 587: Was a liver biopsy performed at any time since diagnosis?
Evaluation of liver tissue may be necessary to determine the extent of the recipient’s disease. Indicate if a liver biopsy was performed at any time since diagnosis.
If Yes, report if the assessment date was known and if Known, the date of the liver biopsy. If multiple liver biopsies were performed since diagnosis, report the date of the most recent biopsy.
If the date of the liver biopsy is partially known, use the process described for reporting partial or unknown dates in General Instructions, Guidelines for Completing Forms; check the box Date estimated.
If a liver biopsy was not performed or it is unknown if a biopsy was performed at any time since diagnosis, report No.
Question 588: Was there evidence of liver cirrhosis?
Indicate if the liver biopsy performed on the date reported above showed evidence / characteristics of liver cirrhosis. If the results are unclear, seek physician clarification.
Select Unknown if no information is available to determine if the biopsy showed characteristics of liver cirrhosis.
Questions 589 – 590: Was there evidence of liver fibrosis?
Indicate if the liver biopsy performed on the date reported above showed evidence / characteristics of liver fibrosis. If the results are unclear, seek physician clarification. If Yes, specify the type of fibrosis observed.
- Bridging: Bands of fibrous tissue and collagen which span portal spaces and/or centrilobular spaces creating a “bridge-like” appearance
- Periportal: Fibrous expansion of portal fields with fibrosis extending along the terminal portal veins
- Other: Select this option if the type of fibrosis present is not listed.
Select Unknown if no information is available to determine if the biopsy showed characteristics of liver fibrosis.
Question 591: Was there evidence of chronic hepatitis?
Indicate if the liver biopsy performed on the date reported above showed evidence / characteristics of chronic hepatitis. If the results are unclear, seek physician clarification.
Select Unknown if no information is available to determine if the biopsy showed characteristics of chronic hepatitis or if the results were inconclusive.
Question 592: Is there evidence of abnormal cardiac iron deposition based on an MRI of the heart at time of infusion? (within 60 days of infusion)
A cardiac MRI may be performed to assess cardiac iron deposition. This information is typically listed within the MRI interpretation of the report.
Indicate if cardiac MRI shows evidence of abnormal cardiac iron deposition at the time of infusion (within 60 days of infusion). If multiple MRIs were performed, report the results based on the most recent scan prior to the start of the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy).
h4.Question 593: Did the recipient have a splenectomy?
Indicate if the recipient had a splenectomy prior to the start of the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy). If unknown, select No.
Questions 594 – 599: Laboratory studies at last evaluation prior to start of preparative regimen (check all that apply)
The questions are intended to determine the clinical status of the recipient prior to the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy). Select all lab values assessed at the last evaluation prior to the start of the preparative regimen / lymphodepleting therapy (or infusion if no preparative regimen / lymphodepleting therapy).
- Serum iron: A serum iron test is used to determine how much iron is in the serum. If the serum iron level is lower than normal, it indicates the body’s iron stores are low (iron deficiency). If the serum iron level is higher than normal, it could indicate hemochromatosis, a condition that causes the body to store too much iron. If the serum iron was assessed at the last evaluation, report the value and unit of measurement as documented on the lab report.
- TIBC (total iron binding capacity): Total iron binding capacity (TIBC) is a test used to gauge the total amount of iron in the blood. If the TIBC was assessed at the last evaluation, report the value and unit of measurement as documented on the lab report.
- Direct bilirubin: Also known as conjugated bilirubin. If the direct bilirubin was assessed at the last evaluation, report the value and unit of measurement as documented on the lab report.
- Total serum bilirubin: Bilirubin is a pigment that is formed from the breakdown of hemoglobin in red blood cells. Serum bilirubin is a test of liver function that reflects the ability of the liver to take up, process, and secrete bilirubin. Total bilirubin includes the direct (conjugated) and indirect (unconjugated) bilirubin values. If the lab reports direct and indirect separately, add the two together to report the total serum bilirubin. If the total serum bilirubin was assessed at the last evaluation, report the value, unit of measurement, and the upper limit of normal as documented on the laboratory report.
If none of the lab values listed above were assessed, select None.
Section Updates:
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