Research Article

Circulating Calreticulin Is Increased in Myelofibrosis: Correlation with Interleukin-6 Plasma Levels, Bone Marrow Fibrosis, and Splenomegaly

Table 1

Patients characteristics according to mutational status. Compared to CALR-mutated patients, patients with JAK2 mutation were older () and had higher hemoglobin levels ().

CharacteristicsJAK2-mutated patients
(number = 16)
CALR-mutated patients
(number = 10)
MPL-mutated patients
(number = 3)
“Triple-negative” patients
(number = 1)

Median age, years (range)73 (67–84)65.5 (44–82)76 (72–76)67
Male sex, number (%)9 (56)6 (60)1 (33.3)0 (0)
Median allele burden, % (range)89 (0.4–99)56.5 (52–98)NANA
Median WBC, ×109/L (range)10.6 (2.5–157.6)7.15 (2.3–48.3)6.2 (4.9–25)6.7
Median hemoglobin, g/dL (range)11.1 (8.6–15.1)9.3 (7.7–14)9.7 (7.2–9.9)8.5
Median platelet count, ×109/L (range)280 (41–507)195.5 (86–419)196 (46–303)632
High/intermediate 2 IPSS category, number (%) 9 (56)6 (60)3 (100)1 (100)
Unfavorable karyotype, number (%)8 (50)2 (20)2 (66.6)1 (100)
Diagnosis, number (%)
 PMF8 (50)6 (60)3 (100)1 (100)
 PET3 (19)2 (20)
 PPV5 (31)2 (20)
BM fibrosis grade ≥ 2, number (%)12 (75)10 (100)2 (66.6)0 (0)
Patients with splenomegaly, number (%)14 (87.5)8 (80)3 (100)0 (0)

WBC: white blood cells; IPSS: International Prognostic Scoring System; PMF: primary myelofibrosis; NA: not available. Splenomegaly was evaluated by palpation as cm below costal margin. Only patients with a spleen palpable ≥ 5 cm below costal margin by palpation were considered as carrying splenomegaly.