Question: Polycythemia Vera diagnosed in 1992, age 19, now 46. BMB in early 2000’s showed JAK2 mutation. Persistent splenomegaly and venous stasis in calves/ankles since late 1990’s. Received phlebotomies until mid-2000’s, then Hydroxyurea (3g/day, six 500mg caplets) for the past 15 years or so as it provided some relief from pruritus, while phlebotomies had made itching worse. In 2016 hepatic thrombosis found, hepatomegaly and ascites, high bilirubin (TBIL 4.0-6.0) and out-of-range liver enzyme panel, diagnosed with Budd-Chiari, received TIPS in 2017 and put on 10mg/day Xarelto. In the past five months, Hgb and Hct crashed and remained anemic (last week 9.1 and 31.4) despite being taken off Hydroxyurea four months ago – weird, as for 27 years Hgb and Hct would skyrocket if I went off treatment even a couple of weeks -- WBC elevated and hovers between 19 and 37, and twice PLT’s crashed to single digits with bruising and bleeding in multiple locations – taken off Xarelto about 14 weeks ago. I also have recurrent severe gut pain, occasional fever in the evenings, extreme fatigue, and four hospitalizations since the beginning of 2019. Ability to digest meals is diminishing (pain/nausea/vomiting), and I fast for days at a time, lost 20 pounds but retaining fluid for some reason (swollen feet/ankles, abdominal bloating). Imaging shows similar splenomegaly, etc. as in the past, TIPS is patent, no intestinal ischemia, liver enzymes are not great, but no worse than last three years. Recent BMB shows only slightly more fibrosis than 18 years ago, not enough to diagnose MF, blast cells only at five percent, so no leukemia. Oncology and gastroenterology are stumped. They say all this is very unusual, doesn’t fit any pattern of Polycythemia Vera, Budd-Chiari, or other disease, and order CBC and CMP every two weeks to monitor.