Characteristics and management of primary and other immune thrombocytopenias: Spanish registry study

Figures & data

Figure 1. Study flow-chart.

Table 1. Characteristics of de novo diagnosed patients with immune thrombocytopenia (ITP) in Spain between 2009 and 2011.

	Primary ITP (n = 433)	Secondary ITP* (n = 51)
Age, years, median (range)	52.2 (0.9, 98.5)	58.2 (0.4, 87.7)
Women, n (%)	238 (55.0)	32 (62.7)
Pregnancy, n (%)	15 (3.5)	2 (3.9)
Spain as a country of origin, n (%)	390 (90.1)	46 (90.2)
ITP diagnosis
Time since diagnosis, months, mean (SD)/range	0.7 (0.7) / 0.0 to 6.0	1.0 (0.7) / 0.0 to 2.6
Possible pseudothrombocytopenia, n (%)	9 (2.1)	0 (0)
Abnormal plasma coagulation, n (%)	24 (5.5)	7 (13.7)
Splenomegaly, n (%)	6 (1.4)	2 (3.9)
Family history of thrombocytopenia, n (%)
Yes	13 (3.0)	1 (2.0)
No	389 (89.8)	47 (92.2)
Unknown	31 (7.2)	3 (5.9)
Comorbidities, n (%)
Hypertension	102 (23.6)	10 (19.6)
Recent infections	62 (14.3)	7 (13.7)
Smoking	52 (12.0)	7 (13.7)
Cardiovascular disease	55 (12.7)	4 (7.8)
Diabetes mellitus	47 (10.9)	4 (7.8)
Gastrointestinal disease	30 (6.9)	4 (7.8)
Cancer	25 (5.8)	7 (13.7)
Thyroid disease	25 (5.8)	5 (9.8)
Alcoholism	11 (2.5)	0 (0)
Other substances abuse^†	2 (0.5)	0 (0)
Prior treatments, n (%)	71 (16.4)	7 (13.7)
Anti-inflammatory drugs	30 (6.9)	6 (9.8)
Antiplatelet drugs	27 (6.2)	1 (2.0)
Drugs possibly related to ITP	20 (4.6)^§	5 (9.8)^‡
Vitamin K Antagonists	15 (3.5)	2 (3.9)
Other anticoagulants	5 (1.2)	2 (3.9)
Hematology tests, median (Q1, Q3)/(range)
Platelet count, ×10^9/l	11.4 (4, 31)/(0–99)	12 (5, 49)/(1–99)
Hemoglobin, mmol/l	8.3 (7.5, 9.1)/(4.3–11.4)	7.9 (7.1, 8.8)/(3.8–11.5)
Leukocyte count, ×10^9/l	7 (6, 9)/(2–26)	6 (5, 8)/(2–22)

*Systemic lupus erythematosus (9 cases); lymphoma (9); hepatitis C virus (HCV) (5); drug induced (3); Evans syndrome (3); pregnancy (2); human immunodeficiency virus (HIV) (2); collagenosis (2); antiphospholipid syndrome (2); hepatitis B virus (HBV) (1); HCV/HBV (1); liver cirrhosis (1); Herpes Zoster infection (1); urinary tract infection (1); thyroid disease (1); hemolytic anemia (1); ulcerative colitis (1); celiac disease (1); Waldenström´s disease (1); not specified (4).

^†cocaine (1), cocaine/cannabis (1).

^§acetylsalicylic acid (1); amoxicillin (1); amoxicillin/clavulanate (2); antibiotic (1); carbamazepine/ibuprofen/enalapril (1); clopidogrel (1); deflazacort (1); valproic acid (1); diclofenac (1); doxazosin (1); doxycycline (1); etanercept (1); fosfomycin/metamizol (1); ibuprofen (1); irbesartan plus hydrochlorothiazide (1); metamizol (1); simvastatin (1); simvastatin/fluoxetine/glimepiride (1); simvastatin/losartan plus hydrochlorothiazide (1).

^‡ Acetylsalicylic acid (1); amoxicillin (1); carnitine/arginine/quinine (1); chloroquine (1); meropenem/vancomycin/pentoxifylline (1).

Q1 = 25th percentile; Q3 = 75th percentile; SD = standard deviation.

Figure 2. Utilization and outcome of diagnostic procedures other than platelet count undertaken in the study population to confirm diagnosis of (a) primary (n = 433) and (b) secondary (n = 51) immune thrombocytopenia.

Figure 3. Therapeutic management of patients with primary ITP in Spain.

Table 2. Patterns of use of corticosteroids as first-line treatment for primary ITP.

	Number (%) of primary ITP patients (n = 433)	Treatment duration (days) Median (Q1,Q3)	Daily dose Mean (SD)
Corticosteroid/s as 1st line treatment	324 (74.8)
Corticosteroids monotherapy	176 (40.6)
Corticosteroids + IVIg (± other therapies)	142 (32.8)
Corticosteroids + other therapies (excl. IVIg)	6 (1.4)
Type of corticosteroid
Prednisone	282 (65.1)	77 (40, 136)	Initial: 1.2 (0.4) mg/kg/67 (24) mg Final: 0.7 (0.5) mg/kg/16 (32) mg
Metilprednisolone	52 (12.0)	6 (4, 27)	0.2 (0.3) g
Dexamethasone	27 (6.2)	4 (4, 8)	40 (0) mg
Deflazacort	5 (1.1)	112 (17, 123)	35.2 (21.8) mg
Prednisolone	2 (0.5)	17 (9, -)	2.3 (1.3) mg/kg

Q1 = 25th percentile; Q3 = 75th percentile; SD = standard deviation.

Table 3. Characteristics of the patients with primary ITP at diagnosis according to first-line treatment.

	No treatment (n = 82)	Corticosteroids monotherapy (n = 176)	Corticosteroids + IVIg (n = 142)
Age, years, mean (SD)	54.9 (23.6)	49.1 (25.4)	51.6 (25.3)
Women, %	47.8	63.3	46.2
Hematology tests, median (Q1, Q3)
Platelet count, ×10^9/l	54 (32, 80)	12 (5, 24)	4 (2, 10)
Hemoglobin, g/dl	8.7 (8.0, 9.6)	8.3 (7.6, 9.0)	8.0 (7.1, 9.0)
Leukocyte count, ×10^9/l	6 (5, 8)	7 (6, 9)	7 (6, 9)
Symptoms at diagnosis, %
Cutaneous bleeding	20.7	68.8	76.0
Oral cavity bleeding	6.5	27.6	49.0
Epistaxis	3.3	16.6	26.9
Muscle hematoma	1.1	6.0	7.7
Gastrointestinal bleeding	1.1	2.5	7.7
Metrorrhagia	0	6.0	5.8
Hematuria	0	0.5	4.8
Intracranial hemorrhage	0	0	5.8
Articular hematoma	0	0	0

Q1 = 25th percentile; Q3 = 75th percentile; SD = standard deviation.