Clinical Characteristics and Prognostic Risks of Philadelphia-Negative Myeloproliferative Neoplasms at Cipto Mangunkusumo General Hospital

Figures & data

Table 1 Baseline Clinical and Laboratory Characteristics of 146 Subjects

	PV, n (%) 46 (31.5%)	ET, n (%) 56 (38.4%)	PMF, n (%) 44 (30.1%)
Age (years), median (range)	54 (22–72)	53.5 (23–79)	55 (22–72)
Female sex	21 (45.7%)	27 (48.2%)	22 (50%)
Symptoms
Fever	4 (8.7%)	4 (7.1%)	7 (15.9%)
Night sweat	0 (0)	0 (0)	0 (0)
Weight loss	2 (4.3%)	3 (5.4%)	8 (18.2%)
Fatigue	0 (0)	11 (19.6%)	18 (40.9%)
Dizziness/ headache	20 (43.5%)	23 (41.1%)	3 (6.8%)
Abdominal symptoms	8 (17.4%)	13 (23.2%)	23 (52.3%)
Others^a	56 (54.3%)	22 (39.3%)	11 (25%)
Asymptomatic	5 (10.9%)	9 (16.1%)	2 (4.5%)
Splenomegaly	16 (34.8%)	12 (21.4%)	30 (68.2%)
Schuffner 1–4	15 (93.75%)	12 (100%)	25 (83.3%)
Schuffner >4	1 (6.25%)	0 (0)	5 (16.7%)
Laboratory (median, range)
Hemoglobin (g/dL)	18.05 (15.4–24.5)	13.55 (7.1–17.2)	8.9 (3.8–14.8)
Hematocrit (%)	57 (47.7–75.5)	40.5 (12.6–50.8)	28.7 (11.1–46.3)
WBC count (×10^9/L)	18.63 (3.24–46.91)	16.12 (6.78–52.54)	16.2 (0.79–65.12)
Platelet count (×10^9/L)	638 (110–2146)	1120 (536–2168)	220 (24–1851)
JAK2V617F mutation status
Positive	32 (82%)	20 (42.5%)	13 (76.5%)
Negative	7 (18%)	27 (57.5%)	4 (23.5%)
Comorbidity
Yes	39 (84.8%)	44 (80.4%)	27 (61.4%)
Cardiovascular disease	15 (32.6%)	9 (16.1%)	8 (18.2%)
Hypertension	25 (54.3%)	27 (48.2%)	11 (25%)
Diabetes mellitus	2 (4.3%)	3 (5.4%)	3 (6.8%)
Smoking	6 (13%)	10 (17.9%)	3 (6.8%)
Others^b	14 (30.4%)	23 (41.1%)	17 (38.6%)
No	7 (15.2%)	11 (19.6%)	17 (38.6%)
Treatment
Observation	5 (10.9%)	2 (3.6%)	29 (65.9%)
Phlebotomy	8 (17.4%)	1 (1.8%)	0
Hydroxyurea	10 (21.7%)	49 (87.5%)	15 (34.1%)
Phlebotomy + hydroxyurea	23 (50%)	4 (7.1%)	0

Notes: ^aErythromelalgia, edema, ecchymosis, epistaxis, dyspnea, hemiparesis; ^bLiver failure, hepatitis, chronic renal failure, asthma, thalassemia.

Abbreviations: WBC, white blood count; NA, not applicable.

Table 2 Thrombotic and Bleeding Events of 146 Subjects

	PV, n (%) 46 (31.5%)	ET, n (%) 56 (38.4%)	PMF, n (%) 44 (30.1%)
Thrombotic events pre/at diagnosis	12 (26.1%)	12 (21.4%)	2 (4.5%)
Ischemic stroke	11 (23.9%)	7 (12.5%)	2 (4.5%)^b
Acute myocardial infarction	1 (2.2%)	3 (5.4%)	1 (2.3%)^b
Peripheral arterial disease	1 (2.44%)	0 (0)	0 (0)
Acute limb ischemia	0 (0)	0 (0)	1 (2.3%)^b
Deep vein thrombosis	0 (0)	1 (1.8%)	0 (0)
Recurrent pregnancy loss	0 (0)	1 (1.8%)	0 (0)
Bleeding events pre/at diagnosis	2 (4.3%)	6 (10.7%)	9 (20.5%)
Cerebral hemorrhage	1 (2.2%)	2 (3.6%)	0 (0)
Gastrointestinal hemorrhage	1 (2.2%)	0 (0)	6 (13.6%)
Skin and/or mucosal bleeding^a	0 (0)	4 (7.1%)	3 (6.8%)
Previous antiplatelet treatment, n (%)	14 (30.4%)	21 (37.5%)	3 (6.8%)

Notes: ^aEcchymosis, epistaxis, gum bleeding, ^bOne patient developed three thrombosis events.

Table 3 Thrombotic and Bleeding Events of 65 JAK2V617F Mutation-Positive Subjects

	PV, n (%) 32 (49.2%)	ET, n (%) 20 (30.8%)	PMF, n (%) 13 (20%)
Thrombotic events pre/at diagnosis	7 (21.8%)	3 (15%)	1 (7.7%)
Ischemic stroke	7 (21.8%)	2 (10%)	1 (7.7%)^b
Acute myocardial infarction	1 (3.1%)	1 (5%)	1 (7.7%)^b
Peripheral arterial disease	1 (3.1%)	0 (0)	0 (0)
Acute limb ischemia	0 (0)	0 (0)	1 (7.7%)^b
Deep vein thrombosis	0 (0)	1 (5%)	0 (0)
Recurrent pregnancy loss	0 (0)	0 (0)	0 (0)
Bleeding events pre/at diagnosis	2 (6.2%)	2 (10%)	3 (23.7%)
Cerebral hemorrhage	1 (3.1%)	1 (5%)	0 (0)
Gastrointestinal hemorrhage	1 (3.1%)	0 (0)	3 (23.7%)
Skin and/or mucosal bleeding^a	0 (0)	1 (5%)	0 (0)
Previous antiplatelet treatment, n (%)	9 (28.1%)	8 (40%)	2 (15.4%)

Notes: ^aEcchymosis, epistaxis, gum bleeding, ^bOne patient developed three thrombosis events.

Table 4 IPSS in 46 PV Subjects

	n (%)
Age >66 years	4 (8.7)
Age 57–66 years	14 (30.4)
WBC ≥15×10^9/L	34 (73.9)
Previous venous thrombosis	0 (0)
IPSS score
0 (low risk)	8 (17.4)
1–2 (intermediate risk)	22 (47.8)
≥3 (high risk)	16 (34.8)

Table 5 IPSET-Thrombosis Model in 56 ET Subjects

	n (%)
Age ≥60 years	8 (14.3)
Previous thrombosis event	12 (21.4)
Cardiovascular risk^a ≥1	33 (58.9%
JAK2V617 mutation	20 (35.7)
IPSET-thrombosis score
0–1 (low risk)	26 (46.4)
2 (intermediate risk)	12 (21.4)
≥3 (high risk)	18 (32.1)

Note: ^aHypertension, diabetes mellitus, and/or tobacco smoking.

Table 6 IPSS and DIPSS Model in 44 PMF Subjects

	n (%)
Age >65 years	5 (11.4)
Constitutional symptoms^a	20 (45.5)
Hemoglobin <10 g/ dL	32 (72.7)
WBC >25×10^9/L	10 (22.7)
Circulating blast ≥1%	9 (20.5)
IPSS score
0 (low risk)	5 (11.4)
1 (intermediate-1 risk)	13 (29.5)
2 (intermediate-2 risk)	17 (38.6)
≥3 (high risk)	9 (20.5)
DIPSS score
0 (low risk)	5 (11.4)
1–2 (intermediate-1 risk)	15 (34.1)
3–4 (intermediate-2 risk)	22 (50)
5–6 (high risk)	2 (4.5)

Note: ^aFever, fatigue, and/or weight loss.

Figure 1 Thrombotic and bleeding events of 46 PV subjects according to IPSS score.

Figure 2 Thrombotic and bleeding events of 56 ET subjects according to IPSET-thrombosis score.