Page 180 - 2019_01-Haematologica-web

E.M. Staley et al.
Table 3. Univariate analysis identifies the important laboratory parameters that predict mortality in 73 unique patients with iTTP.
Laboratory values
Platelet counts (x109/L) ΔD2-D0
ΔD5-D0
Hemoglobin (g/dL) Hematocrit (%) WBC (x109/L)
PT (sec)
aPTT (sec) Fibrinogen (mg/dL) D-dimer (mg/L) LDH (U/L)
Survived (n=64)
12.8 (11-15)# 2.4 (1-4.5) 25.4 (21.1-33.7) 8.6 (8-9.4) 24.5 (23-26) 10.2 (8.8-12.2) 14.6 (14.4-15) 30 (29-33) 421 (398-480) 2062 (1370-3497) 1053 (912-7332) -366 (-444 to -275) -180 (-213 to -132)
0.4 (0.1-1.0)
0.1 (0.1-0.4) 1.2 (1.1-1.5) 2.3 (1.8-2.8) 1.7 (1.3-2.1) 6.5 (6.2-6.8) 3.5 (3.4-3.8) 1.3 (1.1-1.8) 4695 (3939-3317) 276 (225-327) 199 (173-256)
33 (27-41)
59 (47-87)
2.5 (2-3)
2.7 (2-3.1)
14.8 (12.7-17.8)
1.2 (0.5-6.2)
Died (n=9) P 15.5 (7.9-24) 0.55
3.7 (-5-16.9) 0.92 1.9 (-4.3-53.3) 0.09 9.8 (6.8-11) 0.49 29 (18-34) 0.54 13.3 (4.9-21.7) 0.54 15.5 (13.4-20.1) 0.38 34 (27-63) 0.10 488 (353-804) 0.18 7,083 (708-11871) 0.09 1546 (787-7332) 0.16 -187 (-310 to 283) 0.08
ΔD2-D0
ΔD5-D0
Troponin I (ng/mL)
-109 (-282 to 43)
3.3 (0.8-197)
0.9 (0.1-97)
1.7 (1-3.6) 0.15
2.9 (1.4-10.2) 0.49 2.3 (1.2-3.7) 0.30 5.9 (4.4-7.9) 0.13
3.2 (1.6-3.7)
At Emergency Department
Prior to TPE
Creatinine (mg/dL)
Total bilirubin (mg/dL)
Indirect bilirubin (mg/dL)
Total protein (g/L)
Albumin (g/L)
ADAMTS13 inhibitor (U/mL)
Anti-ADAMTS13 IgG (U/mL)
VWF-Ag (%)
VWF-CBA (%)
HNP1-3 (ng/mL)
0.03*
0.008**
0.01*
Histone/DNA (U/mL)
0.03* 0.9 (0.5-3.6) 1.00 2881(653-11103) 0.39 353 (165-742) 0.25 284 (133-926) 0.19 33 (16-239) 0.86 126 (27-257) 0.07 3.8 (1.5-8) 0.09 3 (1.4-9.1) 0.86 14.7 (7.5-30.8) 0.59 1.2 (0.5-6.2) 0.59
Bb (mg/mL)
C4d (mg/mL)
iC3b (mg/mL)
sC5b (mg/mL)
WBC: white blood cell; ΔLDH: change in lactate dehydrogenase; PT: prothrombin time; aPTT: activated partial thromboplastin time;VWF-Ag: von Willebrand factor antigen;VWF- CBA:vonWillebrandfactorcollagen-bindingactivity;IgG:immunoglobulinG;#Alldata areexpressedasthemedianvaluesand95%confidentialintervalinparenthesis.Mann- Whitney test was performed to determine the statistical significance. Here, * and ** indicate P values <0.05 and <0.01, respectively.
of iTTP were included in this study (Figure 1B).
The median age of the patients was 41 years, with 56% being female and 44% male. Seventy-seven percent of patients were of African-American decent, while Caucasian and Hispanic patients comprised only 22% and <1%, respectively. A substantial number of patients had one or several comorbidities, including hypertension (52%), diabetes mellitus (18%), and systemic lupus ery- thematosus (SLE) (11%). Only 3 patients (4%) were preg- nant at the time of the diagnosis. In addition, 49% of patients reported a history of smoking, and 21% reported
use of recreational drugs (Table 1).
Our blood samples were primarily obtained from
patients at their initial presentation (75%) and during a relapse (23%); only one sample was obtained from an exacerbation. This bias is the result of our inclusion crite- ria designed to prioritize the collection and study of unique patients. The clinical presentation on admission primarily included signs and symptoms of the central
nervous system (CNS) (54.7%), abdominal pain (38.4%), and chest pain (9.6%) (Table 1).
The most common blood type in our patient population was type O (63%), followed by A (22%), B (14%), and AB (1%), with 93% being Rh positive. The frequency of type O was statistically higher than that expected (47.9%) (Online Supplementary Table S1), suggesting that patients with type O blood group are not protected from develop- ing iTTP.
All iTTP patients received TPE as primary therapy. The median number of TPEs for all patients was 15 (95% con- fidence interval [CI] of 12-17). Patients also received corti- costeroids (94%), rituximab (38.4%), and vincristine (6.8%) at various times during hospitalization. Only 5.4% of patients underwent splenectomy as part of their treat- ment.
The outcomes of these patients after treatment were classified as the following: single episode to remission (30.1%), exacerbation or relapse to remission (32.9%),
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