CLL, or chronic lymphocytic leukemia, is characterized by ≥ 5 × 109/L monoclonal lymphocytes with a CLL phenotype (usually co-expressed CD5 and CD23). The term SLL, or small lymphocytic lymphoma is used for non-leukemic cases with the tissue morphology and immunophenotype of CLL.
Hairy cell leukemia is characterized by the presence of abnormal B-lymphocytes in the bone marrow, peripheral blood, and spleen.
PLL, or prolymphocytic leukemia, is a type of CLL and is characterized by increased presence of immature prolymphocytes in the bone marrow and peripheral blood.
Question 372-373: Specify the other leukemia classification
Indicate the other leukemia disease classification at diagnosis. If the subtype is not listed, report as “other leukemia” and specify the reported disease in question 372.
Question 374: Was any 17p abnormality detected?
Cytogenetics is the study of chromosomes. Cytogenetic assessment involves testing blood or bone marrow for the presence of a known chromosomal abnormality that reflects the recipient’s disease. Testing methods you may see include conventional chromosome analysis (karyotyping) or fluorescence in situ hybridization (FISH). For more information about cytogenetic testing and terminology, see Appendix C.
Indicate if cytogenetic studies detected any 17p abnormality at any time prior to the start of the preparative regimen.
If “yes” and the disease classification is CLL, continue with question 375. If “yes” and the disease classification is PLL, continue with question 377.
If cytogenetic studies did not detect any 17p abnormality at any time prior to the start of the preparative regimen, select “no” and continue with question 378.
Question 375: Did a histologic transformation to diffuse large B-cell lymphoma (Richter syndrome) occur at any time after CLL diagnosis?
Histologic transformation may occur after CLL diagnosis. Indicate if CLL transformed into diffuse large B-cell lymphoma (known as Richter’s transformation or Richter’s syndrome). If CLL transformed, select “yes” and continue with question 379. If CLL did not transform, select “no” and continue with question 377.
Question 376: What was the disease status? (Atypical CML)
Indicate the disease status for atypical CML at the last evaluation prior the start of the preparative regimen (or infusion of no preparative regimen was given). If no treatment was given prior to HCT, submit the form. Otherwise, continue with question 377.
The patient received treatment for atypical CML but never achieved complete remission at any time. PIF is not limited by the number of unsuccessful treatments; this disease status only applies to recipients who have never been in complete remission.
All of the following criteria are met and maintained for four or more weeks:
- Marrow with normal maturation of all cellular components
- ≤ 5% blasts in the marrow
- No signs or symptoms of the disease
If the timeframe between achieving CR and the start date of the HCT (i.e., day 0) is less than four weeks, and the recipient is believed to be in CR, report the status at transplantation as CR.
Important: if within four weeks following transplant the recipient’s status is determined to not be CR, an Error Correction Form must be submitted to change the pre-HCT status.
Include recipients with persistent cytogenetic abnormalities who otherwise meet all the criteria of CR.
Report that the recipient is in CR at the time of transplant no matter how many courses of therapy it may have taken to achieve that CR.
The number of this complete remission can be determined by using the following guidelines:
- 1st CR: no prior relapse
- 2nd CR: one prior relapse
- 3rd or higher: two or more prior relapses
Recurrence of disease after CR. Relapse is defined as:
- > 5% blasts in the marrow
- Extramedullary disease
- Reappearance of cytogenetic abnormalities and/or molecular markers associated with the diagnosis at levels that, as determined by a physician, represent relapse.
The number of this relapse can be determined by using the following guidelines:
- 1st relapse: one prior CR
- 2nd relapse: two prior CRs
- 3rd or higher: three or more CRs
The recipient was diagnosed with atypical CML and never treated.
Question 377: What was the disease status? (CLL, PLL, Hairy cell leukemia, Other leukemia)
Indicate the disease status for CLL / SLL, PLL, hairy cell leukemia, or other leukemia at the last evaluation prior the start of the preparative regimen (or infusion if no preparative regimen was given) and continue with question 234.
The recipient was diagnosed with hairy cell leukemia and never treated.
Disappearance of all evidence of disease.
Requires all of the following:
- Neutrophils ≥ 1.5 × 109
- Hemoglobin ≥ 11.0 g/dL (without transfusion)
- Platelets ≥ 100 × 109/L
- Absence of hairy cells on peripheral blood smear and on bone marrow examination
- No palpable lymphadenopathy or hepatosplenomegaly
Requires all of the following:
- ≥ 50% reduction in the absolute hairy cell count in the peripheral blood and the bone marrow
- ≥ 50% improvement of all cytopenias
- ≥ 50% reduction in abnormal lymphadenopathy or hepatosplenomegaly
Not meeting the criteria for any of the other disease response criteria.
Requires one or more of the following:
- ≥ 25% increase in the absolute hairy cell count in the peripheral blood and/or bone marrow
- ≥ 25% decrease in any of the hematologic parameters (i.e., neutrophils, hemoglobin or platelets)
- ≥ 25% increase in abnormal lymphadenopathy or hepatosplenomegaly
No assessment of organomegaly, peripheral blood counts, absolute hairy cell count in the bone marrow or the peripheral blood smear was done at any time after treatment.
Relapse after CR:
- Reappearance of hairy cells in the peripheral blood smear and/or bone marrow (regardless of the degree of infiltration)
- Development of peripheral blood cytopenias
Relapse after PR:
- ≥ 50% increase of residual hairy cells in the marrow
- Development of cytopenias
- Splenomegaly insufficient to qualify as PR
- Reappearance of hairy cells in the bone marrow of those patients who had been classified as partial responders based on residual splenomegaly only
1 Saven, A., Burian, C., Koziol, J. A., & Piro, L. D. (1998). Long-term follow-up of patients with hairy cell leukemia after cladribine treatment. Blood, 92(6), 1918-1926.
To determine the disease status, use the criteria for the leukemia that most closely resembles the disease for which this form is being completed. For questions, contact the CIBMTR Customer Service Center.
Question 378: Date assessed:
Enter the date of the most recent assessment of disease status prior to the start of the preparative regimen. The date reported should be that of the most disease-specific assessment within the pre-transplant work-up period (approximately 30 days). Clinical and hematologic assessments include pathological evaluation (e.g., bone marrow biopsy), radiographic examination (e.g., X-ray, CT scan, MRI scan, PET scan), and laboratory assessment (e.g., CBC, peripheral blood smear), in addition to clinician evaluation and physical examination. Enter the date the sample was collected for pathological and laboratory evaluations; enter the date the imaging took place for radiographic assessments.
If the exact date is not known, use the process for reporting partial or unknown dates as described in General Instructions, Guidelines for Completing Forms.
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