Questions 1-2: What was the date of diagnosis of Chronic Lymphocytic Leukemia?
Report the date of the first pathologic diagnosis (e.g., bone marrow biopsy or flow cytometric analysis of the peripheral blood) of CLL, SLL, or PLL. Enter the date the sample was collected for examination. If the diagnosis was determined at an outside center and no documentation of a pathologic or laboratory assessment is available, the dictated date of diagnosis within a physician’s note may be reported. Do not report the date symptoms first appeared. The date of diagnosis is important because the interval between diagnosis and HCT or cellular therapy is often a significant indicator for the recipient’s prognosis post-infusion.
If the exact pathologic diagnosis date is not known, use the process described for reporting partial or unknown dates in General Instructions, General Guidelines for Completing Forms.
Indicate if documentation (e.g., pathology report) was submitted to the CIBMTR in question 2. For further instructions on how to attach documents in FormsNet3SM, refer to the Training Guide.
Question 3: Did a histologic transformation occur at any time after CLL diagnosis?
Histologic transformation may occur after CLL diagnosis. Indicate if CLL transformed into another disease, such as diffuse large B-cell lymphoma (known as Richter’s transformation or Richter’s syndrome). If CLL transformed, report “yes” and continue with question 4. If CLL did not transform, report “no” and continue with question 8.
Question 4: Date of transformation:
Report the date of assessment that determined the disease transformation. Use the date of the pathologic evaluation (e.g., lymph node biopsy) and enter the date the sample was collected.
If the exact date is not known, use the process described for reporting partial or unknown dates in General Instructions, General Guidelines for Completing Forms.
Questions 5-7: Specify the disease classification after transformation:
Indicate if the new disease classification is diffuse large B-cell lymphoma (Richter syndrome) or other histology.
Richter’s Syndrome occurs when CLL transforms into diffuse large B-cell lymphoma. If the recipient transforms to diffuse large cell lymphoma, report Non-Hodgkin Lymphoma (NHL) on question 357 of Form 2400 (Revision 4) as the primary disease for HCT. In addition to this form, Form 2018 (Hodgkin and Non-Hodgkin Lymphoma Pre-HCT Data) must be completed.
In rare cases, CLL may transform into another disease such as Hodgkin Lymphoma or a T-cell lymphoma. Evolution to a component of B-cell prolymphocytic leukemia (B-PLL) during the natural history of relapsed CLL/SLL is also common. If CLL transforms into another histology, specify using question 6.
Indicate whether documentation (pathology report) was submitted to the CIBMTR in question 7. For further instructions on how to attach documents in FormsNet3SM, refer to the Training Guide.
Question 8-11: Autoimmune disorder(s) at diagnosis:
Autoimmune cytopenias appear in 5-10% of patients with CLL1. Treatment for these disorders may include corticosteroids or even splenectomy if unresponsive. Indicate whether any of the following autoimmune disorders were present at diagnosis:
Immune Hemolytic Anemia: the destruction of red blood cells by the immune system. This disorder is typically diagnosed using a Direct Antiglobulin Test (DAT) also known as the Coombs Test. This assay will determine whether the body is producing antibodies which will target red blood cells.
Immune Thrombocytopenia: the destruction of platelets by the immune system. This is typically a clinical diagnosis and platelet specific antibodies are not routinely ordered due to their low sensitivity and specificity. However, if platelet specific antibodies were tested for and found to be present this would support a diagnosis of immune thrombocytopenia. A clinical diagnosis should be confirmed if the provider notes are unclear.
If the recipient had an autoimmune disorder at diagnosis which is not listed above (e.g., pure red cell aplasia), report “other” and specify the other autoimmune disorder in question 11.
Questions 12-13: Rai stage (at diagnosis):
Using the criteria in Table 1 below, indicate the Rai stage at diagnosis. If the Rai stage at diagnosis is not clear from the available documentation, consult with a physician and have them document the stage. If the Rai stage at diagnosis is unknown, select “unknown” for question 12 and skip question 13.
Table 1. Rai Stage
|Stage 0||Low Risk||Lymphocytosis (> 15,000 × 109/L) in blood or bone marrow only without adenopathy, hepatosplenomegaly, anemia or thrombocytopenia|
|Stage I||Intermediate Risk||Lymphocytosis plus enlarged lymph nodes (lymphadenopathy) without hepatosplenomegaly, anemia, or thrombocytopenia|
|Stage II||Intermediate Risk||Lymphocytosis plus enlarged liver or spleen with or without lymphadenopathy|
|Stage III||High Risk||Lymphocytosis plus anemia (hemoglobin < 11 g/dL) with or without enlarged liver, spleen, or lymph nodes|
|Stage IV||High Risk||Lymphocytosis plus thrombocytopenia (platelet count < 100 × 109/L) with or without anemia or enlarged liver, spleen, or lymph nodes|
Question 14-15: What was the Binet stage at diagnosis?
Using the criteria in Table 2 below, indicate the Binet stage at diagnosis. If the Binet stage at diagnosis is not clear from the available documentation, consult with a physician and have them document the stage. If the Binet stage at diagnosis is unknown, report “unknown” and skip question 15.
: axillary, cervical, inguino-femoral, liver, and spleen.
Table 2. Binet Stage
|Stage A||Two or fewer lymphoid bearing areas enlarged, without anemia thrombocytopenia|
|Stage B||Three or more lymphoid bearing areas enlarged, without anemia or thrombocytopenia|
|Stage C||Presence of anemia (hemoglobin < 10.0 g/dL) or thrombocytopenia (platelet count < 100 × 109/L or 100,000/μL)|
Question 16: Were systemic symptoms (B symptoms) present?
Using the criteria below, indicate if the recipient had “B symptoms” (also known as systemic or constitutional symptoms) at the time of diagnosis. If the symptoms at diagnosis are not clear from the available documentation, consult with a physician and have them document the presence or absence of “B” symptoms. If the symptomology at diagnosis is unknown, select “unknown” for question 16 and continue with question 17.
Table 3. Systemic Symptoms
|A||None of the symptoms listed in B below|
|B|| • Unexplained fever > 38° C (100.4° F);
• Night sweats; or,
• Unexplained weight loss of > 10% of body weight in six months before treatment
Question 17: Was extranodal disease present at diagnosis?
Extranodal disease involves sites other than the lymph nodes, spleen and thymus. Common areas of extranodal involvement include the bone marrow, central nervous system, liver, and lungs. Extranodal involvement is most often detected utilizing imaging techniques or pathologic findings.
If there was extranodal involvement at diagnosis, indicate “yes” and complete questions 18-21.
If there was no evidence of extranodal involvement, select “no” and skip questions 18-21.
Questions 18-21: Specify site(s) of extranodal involvement
Specify the site(s) of extranodal involvement. If “other site” is reported, specify any other sites of involvement in question 21.
1 Hodgson K, Ferrer G, Pereira A, et al. Autoimmune cytopenia in chronic lymphocytic leukaemia: Diagnosis and treatment. Br J Haematol 2011;154:14–22.
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