Statements pertinent to epidemiology, diagnosis, and prognosis of RT
| Statement number . | Grade . | Epidemiology and prevalence . |
|---|---|---|
| 1.1.1 | B | RT occurs in 2% to 10% of patients with CLL. |
| 1.1.2 | B | RT can occur in patients who have not received any treatment or who have received only targeted therapies for their CLL. |
| 1.1.3 | R | More data are required to determine whether risk of RT changes with exposure to different treatment types. |
| 1.1.4 | G | There are no therapeutic strategies proven to reduce the risk of RT in CLL. |
| Statement number | Grade | Diagnosis |
| 1.2.1 | G | RT should be suspected in patients with clinical decline, B-symptoms, elevated LDH, rapidly enlarging lymphadenopathy, and/or discordant response to CLL treatment. |
| rk | There should be strong consideration for RT in patients with discordant enlarging lymphadenopathy (eg, 1 nodal group growing rapidly compared with others). | |
| 1.2.2 | B | In patients with a clinical suspicion of RT, a PET-CT scan should be attained. |
| 1.2.3 | G | The most accessible lesion with the highest avidity should be targeted for biopsy. |
| rk | SUV avidity of <5 suggests a low likelihood of RT. | |
| rk | In cases in which the most accessible lesion has avidity, that is, much lower than a less-accessible lesion (eg, an axillary node with an SUV of 6 vs a retroperitoneal node with an SUV of 22), we recommend attempting to biopsy the lesion with higher avidity. | |
| 1.2.4 | B | Biopsy of the affected tissue for histology assessment is needed to diagnose RT. |
| 1.2.5 | G | We strongly recommend attaining an excisional biopsy for diagnosis. |
| rk | Given the complexity in diagnosing RT from CLL, an excisional biopsy is recommended. However, we recognize there are clinical scenarios in which an excisional biopsy is not possible. If an excisional biopsy cannot be performed, we recommend multiple core biopsies. Fine needle aspirate is insufficient and cannot be used in the diagnosis of RT. | |
| 1.2.6 | G | All efforts should be made to have pathology reviewed by an expert hematopathologist. |
| 1.2.7 | B | Clonal relationship of the RT tissue and antecedent CLL cells should be tested, because it is one of the strongest prognostic factors for RT survival: patients with clonally unrelated RT have a markedly better prognosis. |
| Statement number | Grade | Prognosis |
| 1.3.1 | B | The prognosis of RT is poor. |
| 1.3.2 | B | Current standard-of-care treatment with R-CHOP–like regimens has poor efficacy. |
| rk | In clinical practice, R-CHOP followed by consolidative allogeneic stem cell transplantation in eligible patients is the most commonly applied regimen. |
| Statement number . | Grade . | Epidemiology and prevalence . |
|---|---|---|
| 1.1.1 | B | RT occurs in 2% to 10% of patients with CLL. |
| 1.1.2 | B | RT can occur in patients who have not received any treatment or who have received only targeted therapies for their CLL. |
| 1.1.3 | R | More data are required to determine whether risk of RT changes with exposure to different treatment types. |
| 1.1.4 | G | There are no therapeutic strategies proven to reduce the risk of RT in CLL. |
| Statement number | Grade | Diagnosis |
| 1.2.1 | G | RT should be suspected in patients with clinical decline, B-symptoms, elevated LDH, rapidly enlarging lymphadenopathy, and/or discordant response to CLL treatment. |
| rk | There should be strong consideration for RT in patients with discordant enlarging lymphadenopathy (eg, 1 nodal group growing rapidly compared with others). | |
| 1.2.2 | B | In patients with a clinical suspicion of RT, a PET-CT scan should be attained. |
| 1.2.3 | G | The most accessible lesion with the highest avidity should be targeted for biopsy. |
| rk | SUV avidity of <5 suggests a low likelihood of RT. | |
| rk | In cases in which the most accessible lesion has avidity, that is, much lower than a less-accessible lesion (eg, an axillary node with an SUV of 6 vs a retroperitoneal node with an SUV of 22), we recommend attempting to biopsy the lesion with higher avidity. | |
| 1.2.4 | B | Biopsy of the affected tissue for histology assessment is needed to diagnose RT. |
| 1.2.5 | G | We strongly recommend attaining an excisional biopsy for diagnosis. |
| rk | Given the complexity in diagnosing RT from CLL, an excisional biopsy is recommended. However, we recognize there are clinical scenarios in which an excisional biopsy is not possible. If an excisional biopsy cannot be performed, we recommend multiple core biopsies. Fine needle aspirate is insufficient and cannot be used in the diagnosis of RT. | |
| 1.2.6 | G | All efforts should be made to have pathology reviewed by an expert hematopathologist. |
| 1.2.7 | B | Clonal relationship of the RT tissue and antecedent CLL cells should be tested, because it is one of the strongest prognostic factors for RT survival: patients with clonally unrelated RT have a markedly better prognosis. |
| Statement number | Grade | Prognosis |
| 1.3.1 | B | The prognosis of RT is poor. |
| 1.3.2 | B | Current standard-of-care treatment with R-CHOP–like regimens has poor efficacy. |
| rk | In clinical practice, R-CHOP followed by consolidative allogeneic stem cell transplantation in eligible patients is the most commonly applied regimen. |
B, background statement; G, good practice statement; LDH, lactate dehydrogenase; R, research statement; rk, remark to earlier statement.