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 601 – 603: Specify inborn error of immunity classification
CIBMTR captures some classifications of the disorders of immune system using on the International Union of Immunological Societies (IUIS) 2022. Indicate the disorder of the immune system’s disease classification at diagnosis. If the subtype is ‘Leaky SCID’ without one of the listed mutations, report the phenotype (i.e., T-B-NK+, etc.). If the subtype is not listed, report as Other SCID (with known genetic mutation) or Other immunodeficiencies and specify the reported disease.
If Other SCID (with known genetic mutation) is selected, specify any known mutations in addition to the subtype.
If a certain disease becomes a common indication for infusion, the CIBMTR will add the disease as a separate category.
Questions 604 – 605: Did the recipient have an active or recent infection with a viral pathogen within 60 days of infusion?
Viral infections are caused by exposure to a new virus or reactivation of a dormant virus already present in the body. The most common viral infections are due to HSV (Herpes Simplex Virus), and CMV (Cytomegalovirus).
Report Yes if the recipient had an active or recent infection with a viral pathogen within 60 days of infusion and specify the viral pathogens causing the infection. Check all that apply.
If the recipient did not have an active or recent infection with a viral pathogen or it is unknown, report No.
Question 606: Has the recipient ever been infected with PCP/PJP
PCP Pneumocystis is a common fungal infection commonly affecting the lungs. Indicate if the recipient has ever been infected with PCP/PJP.
Question 607: Does the recipient have GVHD due to maternal cell engraftment pre-infusion? (SCID only)
Recipients with SCID often have presence of maternal T lymphocytes (T cells) in the circulation. This is a complication that results from maternal-fetal transfusion and the failure in SCID patients to recognize and to reject foreign cells, allowing maternal T cells to engraft. This is also known as maternal engraftment. This engraftment can induce graft-versus-host disease (GVHD).
Report Yes if the recipient has a history of or current manifestations of GVHD due to maternal cell engraftment at the last evaluation prior to the preparative regimen and continue to signature line.
If the recipient does not have GVHD due to maternal cell engraftment pre-infusion, report No and submit the form.
Question 608: Was the recipient identified based on an abnormal newborn screen (SCID only)
SCID may be identified via a blood screen which measures a T-cell marker to identify the risk for SCID. This blood test is available as part of newborn screening. Infants are often diagnosed at birth or in utero.
Specify if the recipient was diagnosed with SCID based on abnormal newborn screen. If it is unknown whether the recipient was diagnosed via an abnormal newborn screen, report Unknown. This option should be used sparingly and only when no information exists regarding SCID screening as an infant.
Section Updates:
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