Q562 – 564: Severe Aplastic Anemia

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 562 – 564: Specify the aplastic anemia classification

Indicate the aplastic anemia classification of the primary disease for infusion.

If the primary disease for infusion is any of the classifications listed below, specify the severity as either Severe / Very Severe or Not Severe at the time of diagnosis using the criteria also provided below:

• Acquired AA, not otherwise specified
• Acquired AA secondary to chemotherapy
• Acquired AA secondary to hepatitis (any form of hepatitis)
• Acquired AA secondary to immunotherapy or immune effector cell therapy
• Acquired AA secondary to toxin / other drug

Severe / Very Severe or Not Severe Criteria

• Severe / Very Severe
  • Requires both of the following¹:
    • Bone marrow cellularity < 25% (or 25% to 50% if < 30% of residual cells are hematopoietic)
      and
    • At least two of the following:
      • Peripheral blood absolute neutrophil count (ANC) < 500 / µL (<0.5 x 10⁹/L)
      • Peripheral blood platelet count < 20,000 / µL
      • Peripheral blood reticulocyte count < 20,000 / µL

• Not severe
  • Does not meet the criteria for Severe / Very Severe

¹ Olson, T. S. (2019, July 18). Aplastic anemia: pathogenesis, clinical manifestations, and diagnosis. UpToDate. https://www-uptodate-com/contents/aplastic-anemia-pathogenesis-clinical-manifestations-and-diagnosis?search=aplastic+anemia&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H683870569.

Select* Acquired AA secondary to chemotherapy* only when a recipient develops acquired AA after receiving chemotherapy. See example below:

• Example 1: A female recipient in her 50’s was diagnosed with Stage IV breast cancer and received dose-dense chemotherapy with doxorubicin and cyclophosphamide every other week, followed by weekly Taxol. After receiving four cycles, the recipient became neutropenic requiring red blood cell and platelet transfusions. The chemotherapy was discontinued but the blood counts remained low. After three months of persistent neutropenia, the decision was made to proceed with transplant and the recipient was diagnosed with acquired AA secondary to chemotherapy.

If the classification is not listed, select Other acquired cytopenic syndrome and specify the other acquired cytopenic syndrome.

Section Updates:

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