Comparisonof presentation, management, and outcomes in the pre-rituximab era (November 1995-November 2003) and the rituximab era (December 2003-December 2015)
| Outcome . | Patients . | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| All . | Enrolled November 1995-November 2003 . | Enrolled December 2003-December 2015 . | ||||||||||
| No. . | % . | Median . | Range . | No. . | % . | Median . | Range . | No. . | % . | Median . | Range . | |
| All patients | 78 | 34 | 44 | |||||||||
| Severe neurologic abnormalities* | 41 | 18 | 53 | 23 | 52 | |||||||
| Coma | 6 | 4 | 12 | 2 | 5 | |||||||
| Stroke | 9 | 3 | 9 | 6 | 14 | |||||||
| Seizure | 12 | 7 | 21 | 5 | 11 | |||||||
| Transient focal abnormalities | 31 | 10 | 29 | 21 | 48 | |||||||
| Laboratory data† | ||||||||||||
| Platelets × 103/µL | 10 | 2-101 | 11 | 2-101 | 10 | 4-63† | ||||||
| Hematocrit, % | 21 | 13-33 | 21 | 13-30 | 22 | 13-33† | ||||||
| Creatinine, mg/dL | 1.3 | 0.7-6.8 | 1.2 | 0.7-5.5 | 1.4 | 0.8-6.8† | ||||||
| LDH, U/L | 1522 | 343-12 078 | 1870 | 491-12 078 | 1369 | 343-5540 | ||||||
| Outcomes | ||||||||||||
| Death before completing 1 PEX | 2 | 0 | 2 | |||||||||
| Death before response | 2 | 2 | 0 | |||||||||
| Response | 74 | 95 | 32 | 94 | 42 | 95 | ||||||
| Exacerbation | 39 | 53 | 18 | 56 | 21 | 50 | ||||||
| Death after response | 6 | 3 | 3 | |||||||||
| Remission | 68 | 87 | 29 | 85 | 39 | 89 | ||||||
| No. of PEX and hospital days (patients who had ≥1 PEX) | 76 | |||||||||||
| PEX‡ | 14 | 2-79 | 20 | 3-74 | 11 | 2-79 | ||||||
| Days in hospital | 19 | 4-109 | 19 | 4-109 | 17 | 6-89 | ||||||
| Days in hospital before diagnosis | 1 | 0-37 | 1 | 0-14 | 1 | 0-37 | ||||||
| Additional treatments received | ||||||||||||
| Corticosteroids | 64 | 82 | 21 | 62 | 43 | 98 | ||||||
| Rituximab | 19 | 24 | 0 | 19 | 43 | |||||||
| Cyclophosphamide | 1 | 0 | 1 | |||||||||
| Vincristine | 4 | 1 | 3 | |||||||||
| Splenectomy | 1 | 1 | 0 | |||||||||
| Twice-daily PEX | 13 | 8 | 5 | |||||||||
| Surviving patients | 68 | 29 | 39 | |||||||||
| Patients who relapsed§ | 15 | 52 | 12 | 31 | ||||||||
| Total No. of relapses | 35 | 16 | ||||||||||
| No. of relapses per patient | ||||||||||||
| 1 | 6 | 8 | ||||||||||
| 2 | 2 | 4 | ||||||||||
| 3 | 3 | 0 | ||||||||||
| 4 | 4 | 0 | ||||||||||
| Outcome . | Patients . | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| All . | Enrolled November 1995-November 2003 . | Enrolled December 2003-December 2015 . | ||||||||||
| No. . | % . | Median . | Range . | No. . | % . | Median . | Range . | No. . | % . | Median . | Range . | |
| All patients | 78 | 34 | 44 | |||||||||
| Severe neurologic abnormalities* | 41 | 18 | 53 | 23 | 52 | |||||||
| Coma | 6 | 4 | 12 | 2 | 5 | |||||||
| Stroke | 9 | 3 | 9 | 6 | 14 | |||||||
| Seizure | 12 | 7 | 21 | 5 | 11 | |||||||
| Transient focal abnormalities | 31 | 10 | 29 | 21 | 48 | |||||||
| Laboratory data† | ||||||||||||
| Platelets × 103/µL | 10 | 2-101 | 11 | 2-101 | 10 | 4-63† | ||||||
| Hematocrit, % | 21 | 13-33 | 21 | 13-30 | 22 | 13-33† | ||||||
| Creatinine, mg/dL | 1.3 | 0.7-6.8 | 1.2 | 0.7-5.5 | 1.4 | 0.8-6.8† | ||||||
| LDH, U/L | 1522 | 343-12 078 | 1870 | 491-12 078 | 1369 | 343-5540 | ||||||
| Outcomes | ||||||||||||
| Death before completing 1 PEX | 2 | 0 | 2 | |||||||||
| Death before response | 2 | 2 | 0 | |||||||||
| Response | 74 | 95 | 32 | 94 | 42 | 95 | ||||||
| Exacerbation | 39 | 53 | 18 | 56 | 21 | 50 | ||||||
| Death after response | 6 | 3 | 3 | |||||||||
| Remission | 68 | 87 | 29 | 85 | 39 | 89 | ||||||
| No. of PEX and hospital days (patients who had ≥1 PEX) | 76 | |||||||||||
| PEX‡ | 14 | 2-79 | 20 | 3-74 | 11 | 2-79 | ||||||
| Days in hospital | 19 | 4-109 | 19 | 4-109 | 17 | 6-89 | ||||||
| Days in hospital before diagnosis | 1 | 0-37 | 1 | 0-14 | 1 | 0-37 | ||||||
| Additional treatments received | ||||||||||||
| Corticosteroids | 64 | 82 | 21 | 62 | 43 | 98 | ||||||
| Rituximab | 19 | 24 | 0 | 19 | 43 | |||||||
| Cyclophosphamide | 1 | 0 | 1 | |||||||||
| Vincristine | 4 | 1 | 3 | |||||||||
| Splenectomy | 1 | 1 | 0 | |||||||||
| Twice-daily PEX | 13 | 8 | 5 | |||||||||
| Surviving patients | 68 | 29 | 39 | |||||||||
| Patients who relapsed§ | 15 | 52 | 12 | 31 | ||||||||
| Total No. of relapses | 35 | 16 | ||||||||||
| No. of relapses per patient | ||||||||||||
| 1 | 6 | 8 | ||||||||||
| 2 | 2 | 4 | ||||||||||
| 3 | 3 | 0 | ||||||||||
| 4 | 4 | 0 | ||||||||||
The rituximab era is defined as beginning with the first patient treated with rituximab who was hospitalized and diagnosed in December 2003. Therefore patients are grouped as those who presented from November 1995 through November 2003 and those who presented from December 2003 through December 2015.
Neurologic abnormalities include abnormalities at presentation and after PEX began.
There was no difference in the presenting platelet count (P = .497), hematocrit (P = .094), or creatinine (P = .740) values. The LDH values in the rituximab era were less than the values in the pre-rituximab era (P = .047).
Patients in the rituximab era received significantly fewer PEX procedures than patients in the pre-rituximab era (P = .006)
Ten relapses in 6 patients occurred after December 2003. The frequency of relapses was not compared because patients in the earlier cohort had more time in which a relapse could occur.