We present a rare case of a focal intrahepatic extramedullary hematopoiesis (EMH) that mimicked a hypervascular hepatic neoplasm in a 33-year-old woman with idiopathic myelofibrosis. The lesion showed homogeneous and persistent enhancement on both contrast-enhanced CT and gadolinium-enhanced dynamic MR imaging. The lesion did not demonstrate an apparent signal drop on a T2*-weighted sequence following administration of a superparamagnetic iron-oxide agent (SHU 555A). A hepatocellular adenoma was the initial radiological diagnosis. To the best of our knowledge, this is the first report of a histopathologically proven intrahepatic EMH evaluated with dynamic- and SPIO-enhanced MRI.
Adenoma/*diagnosis ; Adult ; Diagnosis, Differential ; *Hematopoiesis, Extramedullary ; Humans ; Liver/*physiology ; Liver Neoplasms/*diagnosis ; *Magnetic Resonance Imaging ; Male ; Primary Myelofibrosis/physiopathology

OBJECTIVETo investigate the value of myeloproliferative neoplasms Symptom Assessment Form total symptom score (MPN-SAF-TSS)in assessing constitutional symptoms among Ph/BCR- ABL negative myeloproliferative neoplasm (MPN)patients.

METHODSA cohort of 628 MPN patients were evaluated by MPN- SAF- TSS.

RESULTSFatigue was the most common symptom (76.0%, 76.2%vs 89.9%)and the highest average severity of all the symptoms (3.46±2.97, 3.47±2.99vs 4.74±3.04 scores)among polycythemia vera (PV), essential thrombocythemia (ET)and primary myelofibrosis (PMF)patients. Using the MPN- SAF- TSS analysis, PMF patients showed highest burden of symptoms (28.9 ± 19.1), followed by PV patients (19.2 ± 16.8), and finally ET patients (17.1 ± 15.3). Instinct differences were observed between PMF and PV patients (χ(2)=6.371,P=0.021), PMF and ET patients (χ(2)= 14.020,P<0.001). No significant difference was found between PV and ET patients (χ(2)=2.281,P=0.191).

CONCLUSIONMPN- SAF- TSS was effective in evaluating the symptomatic burden among Ph/BCRABL negative MPN patients and could be used for serial assessment in this clinical setting.

Humans ; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative ; diagnosis ; physiopathology ; Polycythemia Vera ; complications ; Primary Myelofibrosis ; complications ; Thrombocythemia, Essential ; complications