Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study

Table 3 Treatment and outcomes of patients with immune-mediated thrombotic thrombocytopenic purpura.

	primary iTTP (n = 18)^a	SLE-TTP (n = 10)^a	Hazard ratio SLE-TTP/primary iTTP	p values
Therapies
PE, n	17 (94.4)	7 (70.0)		0.12
Number of PE sessions needed for clinical remission, n	10.0 (7.0, 15.0)^b	8.0 (0, 14.8)		1.00
High-dose steroids, n	18 (100.0)	10 (100.0)		1.00
Steroid pulse therapy, n	3 (16.7)	9 (90.0)		< 0.01
Immunosuppressants, n	1 (5.6)	9 (90.0)		< 0.01
CYC, n	1 (5.6)	8 (80.0)		< 0.01
MMF, n	0 (0.0)	1 (10.0)		0.37
Rituximab, n	10 (55.6)	2 (20.0)		0.11
IVIG, n	1 (5.6)	5 (50.0)		0.01
Therapeutic responses
Follow-up, months	12.8 (0.3, 34.6)	32.7 (14.2, 43.0)		0.14
Clinical response	11 (61.1)	10 (100.0)	6.4 [1.3, 30.9] ^d	0.02^d
Clinical remission	11 (61.1)	10 (100.0)	7.6 [1.2, 50.1] ^d	0.03^d
Exacerbation	2 (18.2)^b	2 (20.0)		1.00
Refractory	7 (46.7) ^c	2 (20.0)		0.23
Death	7 (38.9)	0 (0.0)		0.03

Abbreviations: CYC cyclophosphamide, iTTP immune-mediated TTP, IVIG intravenous immunoglobulin, MMF mycophenolate mofetil, SLE systemic lupus erythematosus, SLE-TTP SLE-associated TTP, PE plasma exchange, TTP thrombotic thrombocytopenic purpura
^a Values are expressed as the means ± SDs or the medians (interquartile ranges). Attributes values are expressed as numbers (percentages).
^b Patients who died during hospitalization and did not achieve haematological remission were not included in the analysis.
^c Three patients who died before completing five sessions of TPE were not included in the analysis.
^d HR and p values are adjusted for age and treatment, including steroid pulse therapy and IVIG treatment.

ISSN: 1750-1172