Table 3.

Post-HSCT course in 28 patients with SLE-related APS

ACLA
Anticoagulation
Immunosuppression
Patient no.
LA
IgG
IgM
Follow-up, mos.
Thrombotic events
Non-Sapporo manifestations
Before HSCT, Y/N
After HSCT, Y/N
When d/c, mo
SLE remission (mo. of relapse)
After HSCT, P mg/d
When d/c, mo
D-APS             
1   Pos   Pos   —   78   None   No LSE, new H/A   Yes   No   0   Y   None   12  
2   —   Neg   —   60   LAD + LCX×2   None   Yes   Yes   Continued   No (3)   —   —  
3  Neg  —   —   42   None   None   Yes   No   30   Y   5   NA  
4   —   Neg   Neg   36   None   No thrombocytopenia, LR same   Yes   No   0   No (3)   —   —  
5  NRT  —  Neg  30   None   None   Yes   No   12   Y   None   6  
6   —  Neg  —   30   None   Neurologically improved   No   No   NA   Y   None   13  
7   NRT   —   —   24   UE DVT×3   LR and hallucinations improved, thrombocytopenia recurred   Yes   Yes   Restarted at 12   No (12)   —   —  
8   —   —  Neg  24   UE DVT   Calciphylaxis resolved   Yes   Yes   Restarted at 14   Y   None   0  
9  Neg Pos Neg  28   None   No TIA   Yes   No   5   Y   5 + fludrocortisone (AI)   NA  
10   —   —  Pos  11   Splenic infarct, UE DVT   No seizures, thrombocytopenia recurred   Yes   Yes   Restarted at 10   No (3)   —   —  
11  Pos  Neg  Neg  24   LE vein graft   No CVA, LLE ulcer healed   Yes   Yes   Restarted at 10   Y   2.5   NA  
12   NRT   —   —   Lost to follow-up   None   No CVA   Yes   Yes   Continued   Y   —   —  
13   —  Neg Neg  18   None   Calciphylaxis improving, diplopia stable   Yes   No   0   Y   None   12  
14   Neg   —   —   21   None   None   Yes   No   3   Y   Hydrocortisone (AI)   NA  
15  Neg  Neg   Neg   24   None   No seizures   Yes   No   10   Y   None   12  
16  Neg  —   —   15   None   None   Yes   No   9   Y   5   NA  
17   Neg   Neg   Neg   6   None   No seizures, no thrombocytopenia   No   No   NA   Y   10   NA  
18   —   Neg   —   12   None   No seizures, no thrombocytopenia   Yes   No   1   Y   None   1  
19  Pos  —   —   6   None   LR improving, no CVA, immune thrombocytopenia   Yes   Yes   Continued   Y   5   NA  
20   —   Neg   —   6   None   No TIA, H/A same   Yes   Yes   Continued   Y   5   NA  
P-APS             
1   —   —  Neg  33   None   LR persisted   No   No   NA   No (9)   —   —  
2   —  Pos Pos  36   None   Neurologically improved, seizures recurred at 24 mo   Yes   No   0   No (30)   —   —  
3   —  Neg  —   36   None   Neurologically improved   No   No   NA   Y   None   12  
4  NRT Neg  —   18   None   Neurologically improved   Yes   No   6   Y   2.5   NA  
5  Neg  —   —   30   None   No thrombocytopenia, no LSE   Yes   No   3   Y   None   6  
6   Neg   —  Neg  12   None   Neurologically improved, but TM recurred at 9 mo   No   No   NA   No (9)   —   —  
7  Neg Neg Neg  12   None   H/A improved   No   No   NA   Y   5   NA  
8
 		 —
 		 —
 		Neg
 		 12
 		 None
 		 No seizures
 		 Yes
 		 No
 		 6
 		 Y
 		 10
 		 NA
 
ACLA
Anticoagulation
Immunosuppression
Patient no.
LA
IgG
IgM
Follow-up, mos.
Thrombotic events
Non-Sapporo manifestations
Before HSCT, Y/N
After HSCT, Y/N
When d/c, mo
SLE remission (mo. of relapse)
After HSCT, P mg/d
When d/c, mo
D-APS             
1   Pos   Pos   —   78   None   No LSE, new H/A   Yes   No   0   Y   None   12  
2   —   Neg   —   60   LAD + LCX×2   None   Yes   Yes   Continued   No (3)   —   —  
3  Neg  —   —   42   None   None   Yes   No   30   Y   5   NA  
4   —   Neg   Neg   36   None   No thrombocytopenia, LR same   Yes   No   0   No (3)   —   —  
5  NRT  —  Neg  30   None   None   Yes   No   12   Y   None   6  
6   —  Neg  —   30   None   Neurologically improved   No   No   NA   Y   None   13  
7   NRT   —   —   24   UE DVT×3   LR and hallucinations improved, thrombocytopenia recurred   Yes   Yes   Restarted at 12   No (12)   —   —  
8   —   —  Neg  24   UE DVT   Calciphylaxis resolved   Yes   Yes   Restarted at 14   Y   None   0  
9  Neg Pos Neg  28   None   No TIA   Yes   No   5   Y   5 + fludrocortisone (AI)   NA  
10   —   —  Pos  11   Splenic infarct, UE DVT   No seizures, thrombocytopenia recurred   Yes   Yes   Restarted at 10   No (3)   —   —  
11  Pos  Neg  Neg  24   LE vein graft   No CVA, LLE ulcer healed   Yes   Yes   Restarted at 10   Y   2.5   NA  
12   NRT   —   —   Lost to follow-up   None   No CVA   Yes   Yes   Continued   Y   —   —  
13   —  Neg Neg  18   None   Calciphylaxis improving, diplopia stable   Yes   No   0   Y   None   12  
14   Neg   —   —   21   None   None   Yes   No   3   Y   Hydrocortisone (AI)   NA  
15  Neg  Neg   Neg   24   None   No seizures   Yes   No   10   Y   None   12  
16  Neg  —   —   15   None   None   Yes   No   9   Y   5   NA  
17   Neg   Neg   Neg   6   None   No seizures, no thrombocytopenia   No   No   NA   Y   10   NA  
18   —   Neg   —   12   None   No seizures, no thrombocytopenia   Yes   No   1   Y   None   1  
19  Pos  —   —   6   None   LR improving, no CVA, immune thrombocytopenia   Yes   Yes   Continued   Y   5   NA  
20   —   Neg   —   6   None   No TIA, H/A same   Yes   Yes   Continued   Y   5   NA  
P-APS             
1   —   —  Neg  33   None   LR persisted   No   No   NA   No (9)   —   —  
2   —  Pos Pos  36   None   Neurologically improved, seizures recurred at 24 mo   Yes   No   0   No (30)   —   —  
3   —  Neg  —   36   None   Neurologically improved   No   No   NA   Y   None   12  
4  NRT Neg  —   18   None   Neurologically improved   Yes   No   6   Y   2.5   NA  
5  Neg  —   —   30   None   No thrombocytopenia, no LSE   Yes   No   3   Y   None   6  
6   Neg   —  Neg  12   None   Neurologically improved, but TM recurred at 9 mo   No   No   NA   No (9)   —   —  
7  Neg Neg Neg  12   None   H/A improved   No   No   NA   Y   5   NA  
8
 		 —
 		 —
 		Neg
 		 12
 		 None
 		 No seizures
 		 Yes
 		 No
 		 6
 		 Y
 		 10
 		 NA
 

D/c indicates discontinued; P, prednisone; Pos, positive; Neg, negative; NRT, not retested (Pos, Neg, and NRT: were positive during pretransplant testing); —, none; NA, not applicable; LAD, left descending coronary artery; LCX, left circumflex coronary artery; AI, adrenal insufficiency; TM, transverse myelitis. Other abbreviations are explained in the text or Tables 1 and 2.

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