Table 6 Individual clinical course data... | American Society of Hematology

Table 6

Individual clinical course data for patients with ADAMTS13 activity < 10% at their initial diagnosis

Patient	Initial episode of TTP	ADAMTS13 activity during remission (%)*				Relapse before remission samples	Relapse after remission samples	Rituximab treatment
Patient	Initial episode of TTP	2004	2006	2007/2008	2009	Relapse before remission samples	Relapse after remission samples	Rituximab treatment
1	1995	< 5⁴	< 5⁰	—	22	1 (1996)	1 (2009, 32 mo after 2006 sample, 2 mo before 2009 sample)	2009
2	1996	100	—	*	*	1 (1996)	0	0
3	1996	100	100	100	85	0	0	0
4	1996	42	87	—	—	1 (1999)	0	0
5	1997	34	100	100	*	4 (1997-2002)	0	0
6	1998	—	—	100	100	0	0	0
7	1999	100	100	100	*	1 (1999)	0	0
8	1999	66	74	49	45	4 (1999-2000)	0	0
9	1999	—	95	*	*	0	0	0
10	1999	85	24	56	58	4 (2000-2004)	1 (2006, 23 mo after 2004 sample)	0
11	2000	8⁰	10⁴	11⁰	17¹	0	0	0
12	2000	—	—	51	80	0	0	0
13	2000	14⁰	*	*	*	0	1 (2004, 1 mo after sample, died)	0
14	2000	90	60	64	100	1 (2003)	2 (2008, 2009, 12 and 18 months after 2007 sample)	2009
15	2000	—	—	85	99	0	0	0
16	2000	—	100	40	10⁰	2 (2001, 2005)	0	2005
17	2000	12¹	11⁰	*	*	0	2 (2008, 17 and 23 months after sample)	0
18	2001	9⁰	17⁰	< 5⁰	*	0	Sudden death (no autopsy) 6 months after sample	0
19	2001	78	100	100	100	0	0	0
20	2001	100	100	100	100	0	0	0
21	2001	20⁰	12¹	15¹	11¹	0	0	0
22	2001	—	—	—	51	3 (2007, 2008, 2008)	0	2008
23	2001	13⁰	100	100	100	0	0	0
24	2001	57	7¹	5¹	< 5⁴	0	0	0
25	2001	13²	100	100	38	1 (2004, 3 mo before sample)	1 (2006, 25 mo after previous sample, 2 mo before next sample)	2006
26	2002	99	26	41	< 5⁴	0	0	0
27	2003	71	96	82	82	0	0	0
28	2003	—	100	—	100	0	0	2003
29	2004	—	93	92	44	1 (2005)	0	2005
30	2005	—	100	100	100	0	0	2005
31	2005	—	36	39	41	0	0	0
32	2006	—	5¹	91	8¹	0	0	2006
33	2006	—	52	59	100	0	0	2006
34	2006	—	75	70	< 5³†	0	1 (2009, diagnosed at ″remission″ visit)	0
35	2006	—	—	< 5⁴	< 5⁴	0	0	0
36	2006	—	—	100	100	0	0	0
37	2007	—	—	100	100	0	0	0
38	2007	—	—	100	90	0	0	2007
39	2008	—	—	—	92	0	0	0
40	2008	—	—	—	88	0	0	0
41	2008	—	—	—	94	0	0	2008

Patient	Initial episode of TTP	ADAMTS13 activity during remission (%)*				Relapse before remission samples	Relapse after remission samples	Rituximab treatment
Patient	Initial episode of TTP	2004	2006	2007/2008	2009	Relapse before remission samples	Relapse after remission samples	Rituximab treatment
1	1995	< 5⁴	< 5⁰	—	22	1 (1996)	1 (2009, 32 mo after 2006 sample, 2 mo before 2009 sample)	2009
2	1996	100	—	*	*	1 (1996)	0	0
3	1996	100	100	100	85	0	0	0
4	1996	42	87	—	—	1 (1999)	0	0
5	1997	34	100	100	*	4 (1997-2002)	0	0
6	1998	—	—	100	100	0	0	0
7	1999	100	100	100	*	1 (1999)	0	0
8	1999	66	74	49	45	4 (1999-2000)	0	0
9	1999	—	95	*	*	0	0	0
10	1999	85	24	56	58	4 (2000-2004)	1 (2006, 23 mo after 2004 sample)	0
11	2000	8⁰	10⁴	11⁰	17¹	0	0	0
12	2000	—	—	51	80	0	0	0
13	2000	14⁰	*	*	*	0	1 (2004, 1 mo after sample, died)	0
14	2000	90	60	64	100	1 (2003)	2 (2008, 2009, 12 and 18 months after 2007 sample)	2009
15	2000	—	—	85	99	0	0	0
16	2000	—	100	40	10⁰	2 (2001, 2005)	0	2005
17	2000	12¹	11⁰	*	*	0	2 (2008, 17 and 23 months after sample)	0
18	2001	9⁰	17⁰	< 5⁰	*	0	Sudden death (no autopsy) 6 months after sample	0
19	2001	78	100	100	100	0	0	0
20	2001	100	100	100	100	0	0	0
21	2001	20⁰	12¹	15¹	11¹	0	0	0
22	2001	—	—	—	51	3 (2007, 2008, 2008)	0	2008
23	2001	13⁰	100	100	100	0	0	0
24	2001	57	7¹	5¹	< 5⁴	0	0	0
25	2001	13²	100	100	38	1 (2004, 3 mo before sample)	1 (2006, 25 mo after previous sample, 2 mo before next sample)	2006
26	2002	99	26	41	< 5⁴	0	0	0
27	2003	71	96	82	82	0	0	0
28	2003	—	100	—	100	0	0	2003
29	2004	—	93	92	44	1 (2005)	0	2005
30	2005	—	100	100	100	0	0	2005
31	2005	—	36	39	41	0	0	0
32	2006	—	5¹	91	8¹	0	0	2006
33	2006	—	52	59	100	0	0	2006
34	2006	—	75	70	< 5³†	0	1 (2009, diagnosed at ″remission″ visit)	0
35	2006	—	—	< 5⁴	< 5⁴	0	0	0
36	2006	—	—	100	100	0	0	0
37	2007	—	—	100	100	0	0	0
38	2007	—	—	100	90	0	0	2007
39	2008	—	—	—	92	0	0	0
40	2008	—	—	—	88	0	0	0
41	2008	—	—	—	94	0	0	2008

Clinical course of patients who had ADAMTS13 < 10% at the time of their initial diagnosis with TTP and who had ADAMTS13 activity measured during remission. Data are presented for the 37 patients who had ADAMTS13 activity < 10% at the time of their initial diagnosis of TTP and who also had ADAMTS13 activity measured during clinical remission, documented by clinical evaluation, platelet count, and hematocrit. ADAMTS13 activity and inhibitor were measured by the FRETS-VWF73 method. Nine patients were treated with rituximab for an acute episode, either initial or relapse, in the years indicated. One patient was treated for an acquired factor VIII inhibitor 11 months after her relapse in 2005 (patient 28).

ADAMTS13 inhibitor levels are indicated by superscript numbers: 0 indicates no inhibitor; 1 indicates > 0 to < 1 BU/mL; 2 indicates 1 to < 2 BU/mL; 3 indicates 2 to > 2 BU/mL; and 4 indicates >> 2 BU/mL. — indicates that the patient was not available for the evaluation.

The patient had died before the planned measurements were performed.

†

Diagnosed with a relapsed acute episode by thrombocytopenia and microangiopathic hemolytic anemia, in spite of being asymptomatic, when she was seen for this scheduled elective evaluation.

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