Recommended definitions for future therapy studies in CD30+ LPDs
| Response . | Definition . |
|---|---|
| I. Response in skin | |
| A. LYP response in skin | |
| Complete response (CR) | 100% clearance of skin lesions |
| Partial response (PR) | 50%-99% clearance of skin disease from baseline without new larger and persistent nodule(s)† in those with papular disease only |
| Stable disease (SD) | < 50% increase to < 50% clearance in skin disease from baseline without new larger and persistent nodule(s) in those with papular disease only |
| Loss of response | Increase of skin score of greater than the sum of nadir plus 50% baseline score in patients with CR or PR |
| Increased disease activity (IDA)* | > 50% increase in skin disease from baseline without larger and persistent nodules† |
| Progressive disease (PD) | (1) Occurrence of larger and persistent nodule(s) (> 2 cm); and (2) extracutaneous spread |
| Relapse | Any disease recurrence in those with CR |
| B. PCALCL response in skin | |
| CR | 100% clearance of skin lesions |
| PR | 50%-99% clearance of skin disease from baseline without new tumors |
| SD | < 25% increase to < 50% clearance in skin disease from baseline |
| PD | (1) ≥ 25% increase in skin disease from baseline; or (2) loss of response: in those with CR or PR, increase of skin score of greater than the sum of nadir plus 50% baseline score |
| Relapse | Any disease recurrence in those with CR |
| II. Nodes: response in lymph nodes for LYP and PCALCL‡ (peripheral and central lymphnodes) | |
| CR | All lymph nodes are now < 1.5 cm in greatest transverse (long axis) diameter by method used to assess lymph nodes at baseline or biopsy negative for lymphoma. In addition, lymph nodes that show lymphoma involvement by biopsy and < 1.5 cm in long axis diameter at baseline must now be ≤ 1 cm in diameter of the short axis or biopsy negative for lymphoma. |
| PR | Cumulative reduction ≥ 50% of the SPD [sum of the maximum linear dimension (major axis) × longest perpendicular dimension (minor axis)] of each abnormal lymph node at baseline and no new lymph node ≥ 1.5 cm or > 1.0 cm in the short axis if long axis is 1- to 1.5-cm diameter |
| SD | Fails to attain the criteria for CR, PR, and PD |
| PD§ | (1) > 50% increase in SPD from baseline of lymph nodes; or (2) any new node ≥ 1.5 cm in greatest transverse diameter or > 1 cm in short axis diameter if 1- to 1.5-cm in long axis that is proven to be lymphoma histologically; or (3) loss of response: in those with PR or CR, > 50% increase from nadir in SPD of lymph nodes |
| Relapse | Any new lymph node ≥ 1.5 cm in long axis diameter in those with CR |
| III. Visceral disease: response in viscera for LYP and PCALCL‡ | |
| CR | Liver or spleen or any organ considered involved at baseline should not be enlarged on physical examination and should be considered normal by imaging. No nodules should be present on imaging of liver or spleen. Any posttreatment mass must be determined by biopsy to be negative for lymphoma. |
| PR | ≥ 50% regression in any splenic or liver nodules, or in measureable disease (SPD) in any organs abnormal at baseline. No increase in size of liver or spleen and no new sites of involvement. |
| SD | Fails to attain the criteria for CR, PR, or PD |
| PD§ | (1) > 50% increase in size (SPD) of any organs involved at baseline; or (2) new organ involvement; or (3) loss of response: in those with PR or CR, > 50% increase from nadir in the size (SPD) of any previous organ involvement |
| Relapse | New organ involvement in those with CR |
| Response . | Definition . |
|---|---|
| I. Response in skin | |
| A. LYP response in skin | |
| Complete response (CR) | 100% clearance of skin lesions |
| Partial response (PR) | 50%-99% clearance of skin disease from baseline without new larger and persistent nodule(s)† in those with papular disease only |
| Stable disease (SD) | < 50% increase to < 50% clearance in skin disease from baseline without new larger and persistent nodule(s) in those with papular disease only |
| Loss of response | Increase of skin score of greater than the sum of nadir plus 50% baseline score in patients with CR or PR |
| Increased disease activity (IDA)* | > 50% increase in skin disease from baseline without larger and persistent nodules† |
| Progressive disease (PD) | (1) Occurrence of larger and persistent nodule(s) (> 2 cm); and (2) extracutaneous spread |
| Relapse | Any disease recurrence in those with CR |
| B. PCALCL response in skin | |
| CR | 100% clearance of skin lesions |
| PR | 50%-99% clearance of skin disease from baseline without new tumors |
| SD | < 25% increase to < 50% clearance in skin disease from baseline |
| PD | (1) ≥ 25% increase in skin disease from baseline; or (2) loss of response: in those with CR or PR, increase of skin score of greater than the sum of nadir plus 50% baseline score |
| Relapse | Any disease recurrence in those with CR |
| II. Nodes: response in lymph nodes for LYP and PCALCL‡ (peripheral and central lymphnodes) | |
| CR | All lymph nodes are now < 1.5 cm in greatest transverse (long axis) diameter by method used to assess lymph nodes at baseline or biopsy negative for lymphoma. In addition, lymph nodes that show lymphoma involvement by biopsy and < 1.5 cm in long axis diameter at baseline must now be ≤ 1 cm in diameter of the short axis or biopsy negative for lymphoma. |
| PR | Cumulative reduction ≥ 50% of the SPD [sum of the maximum linear dimension (major axis) × longest perpendicular dimension (minor axis)] of each abnormal lymph node at baseline and no new lymph node ≥ 1.5 cm or > 1.0 cm in the short axis if long axis is 1- to 1.5-cm diameter |
| SD | Fails to attain the criteria for CR, PR, and PD |
| PD§ | (1) > 50% increase in SPD from baseline of lymph nodes; or (2) any new node ≥ 1.5 cm in greatest transverse diameter or > 1 cm in short axis diameter if 1- to 1.5-cm in long axis that is proven to be lymphoma histologically; or (3) loss of response: in those with PR or CR, > 50% increase from nadir in SPD of lymph nodes |
| Relapse | Any new lymph node ≥ 1.5 cm in long axis diameter in those with CR |
| III. Visceral disease: response in viscera for LYP and PCALCL‡ | |
| CR | Liver or spleen or any organ considered involved at baseline should not be enlarged on physical examination and should be considered normal by imaging. No nodules should be present on imaging of liver or spleen. Any posttreatment mass must be determined by biopsy to be negative for lymphoma. |
| PR | ≥ 50% regression in any splenic or liver nodules, or in measureable disease (SPD) in any organs abnormal at baseline. No increase in size of liver or spleen and no new sites of involvement. |
| SD | Fails to attain the criteria for CR, PR, or PD |
| PD§ | (1) > 50% increase in size (SPD) of any organs involved at baseline; or (2) new organ involvement; or (3) loss of response: in those with PR or CR, > 50% increase from nadir in the size (SPD) of any previous organ involvement |
| Relapse | New organ involvement in those with CR |
Skin tumor burden is assessed by counting the number of lesions before, during, and after therapeutic intervention regardless of morphology (macular, papular, or nodular; ulcerated or nonulcerated). Nodules or tumors > 2 cm should be captured separately. It may be particularly useful for the investigator to note the number of lesions in the body areas.20 Total body photographs offer additional help in tracking lesions and making assessments.
The term increased disease activity (IDA) has been introduced for an increase of number of papulonodular lesions (< 2 cm), which does not imply impaired prognosis.
Larger lesions are defined as > 2 cm in diameter. Persistent lesions are defined as lesions, which do not show spontaneous regression after 12 weeks.
It is still unsolved and a matter of debate whether involvement of lymph nodes and viscera in LYP exists at all or whether the occurrence of CD30+ lymphoma in lymph nodes and viscera represents ALCL, even if clonally related to LYP. Use of FDG-PET scan in this instance is compatible with other NHLs.
Whichever criterion occurs first.