Organ Stiffness in the Work-Up of Myelofibrosis and Philadelphia-Negative Chronic Myeloproliferative Neoplasms

Abstract

<jats:p>To define the role of spleen stiffness (SS) and liver stiffness (LS) in myelofibrosis and other Philadelphia (Ph)-negative myeloproliferative neoplasms (MPNs), we studied, by ultrasonography (US) and elastography (ES), 70 consecutive patients with myelofibrosis (MF) (no.43), essential thrombocythemia (ET) (no.10), and polycythemia vera (PV) (no.17). Overall, the median SS was not different between patients with MF and PV (p = 0.9); however, both MF and PV groups had significantly higher SS than the ET group (p = 0.011 and p = 0.035, respectively) and healthy controls (p &lt; 0.0001 and p = 0.002, respectively). In patients with MF, SS values above 40 kPa were significantly associated with worse progression-free survival (PFS) (p = 0.012; HR = 3.2). SS also correlated with the extension of bone marrow fibrosis (BMF) (p &lt; 0.0001). SS was higher in advanced fibrotic stages MF-2, MF-3 (W.H.O. criteria) than in pre-fibrotic/early fibrotic stages (MF-0, MF-1) (p &lt; 0.0001) and PFS was significantly different in the two cohorts, with values of 63% and 85%, respectively (p = 0.038; HR = 2.61). LS significantly differed between the patient cohort with MF and healthy controls (p = 0.001), but not between the patient cohorts with ET and PV and healthy controls (p = 0.999 and p = 0.101, respectively). We can conclude that organ stiffness adds valuable information to the clinical work-up of MPNs and could be employed to define patients at a higher risk of progression.</jats:p>

Digital Droplet PCR in Hematologic Malignancies: A New Useful Molecular Tool

“…In the 99 samples analyzed by both techniques, there was an optimal correlation between QT-PCR and ddPCR, with the latest technique showing half a log higher sensitivity than the former one (5 × 10 −4 vs. 1 × 10 −3 ). PV and MF presented a similar median mutation burden (40.45%), higher than that observed in ET (21.35%) [ 125 ], differences that we also confirmed by different grades of the spleen stiffness observed by ultrasonography [ 126 , 127 , 128 ]. Finally, a Korean group compared a ddPCR assay for JAK2 V617F mutation with the results from pyrosequencing, once again showing the superiority of ddPCR [ 129 ].…”

A Prospective Cross-Sectional Study on the Comparison of Ultrasound Assessment vs. Palpation in Chronic Lymphocytic Leukemia Patients in the Era of Targeted Therapy

“…US features of normal and diseased SupLNs and spleen have been previously published [ 11 , 13 , 14 , 15 , 16 , 17 , 18 ]. The performances of the last-generation US machines equipped with high-resolution linear probes allow the detection of echo-poor LN down to 3 mm in size and of LN vessels in LN of about 4–5 mm [ 4 ].…”

“…Briefly, 2–5 MHz bandwidth (GE) and 3.5–5 MHz (Esaote) convex probe were used for abdominal scanning, and a linear matrix array probe bandwidth of 4–16 MHz (GE) and a 7.5 MHz linear probe (Esaote) were used for SupLN assessment. The physician MD3 who performed the US examinations was a hematologist member and teacher at the Italian School for basic and emergency ultrasound (SIUMB) of the University of Pisa with more than 20 years’ experience in lymphoproliferative disorders and US [ 11 , 13 , 14 , 15 , 16 , 17 , 18 ]. Parameters assessed by palpation of SupLNs were: presence or absence of LNs in the superficial palpable bilateral regions (inguinal, axillary, cervical, and supraclavicular) [ 1 , 19 ]; if SupLNs were palpable, they were classified as pathological if >1 cm according to what previously reported [ 1 , 19 ].…”

Shear-Wave Ultrasonographic Elastography in Pediatric Spleen and Its Role in Differential Diagnosis

“…Furthermore, spleen sonoelastography can assist in the identification of suitable candidates for liver transplantation and in determining the optimal approach for portal vein reconstruction in patients with biliary atresia following Kasai portoenterostomy [31]. Spleen stiffness in myelofibrosis is directly related to bone marrow fibrosis and can be utilised as a measure to evaluate the effectiveness of treatment [32]. Spleen sonoelastography is additionally valuable for monitoring the functionality of transjugular intrahepatic portosystemic shunts [33].…”