HEALTHCARE professionals are lacking guidance with regard to treating myelofibrosis (MF) with secondary portal hypertension (PHT) or pulmonary hypertension (PH), according to data presented at the EBMT 2024 Annual Meeting. MF is a rare cancer of the blood which causes extensive scarring of the bone marrow, inhibiting haematopoiesis. The sole curative therapy is allogeneic haematopoietic stem cell transplantation (allo-HCT); however, PHT or PH can occur secondary to MF, and may complicate the course of treatment.
To evaluate the course of treatment in MF-associated PHT and PH, the team of researchers conducted an electronic survey directed at healthcare professionals in 63 EBMT centres performing ≥5 MF transplants per year. Of the centres approached, 41 (65%) responded. Of this cohort, 88% perform a radiological assessment of hepatomegaly, with 78% doing the same for splenomegaly pre-transplant. Fifty-four percent of the centres routinely screened for PH, while 12% did not screen whatsoever. In the instance that radiological screening confirmed PH, it was not deemed a contraindication of allo-HCT in 78% of centres. Where patients showed clinical signs of PH prior to transplant, hepatologists and/or gastroenterologists were invariably consulted. However, a mere 7% of centres claimed to involve such specialists in all cases, even where signs of PH were not observed. For the remaining centres, pre-existing liver function abnormalities, imaging abnormalities, or signs of PH were incentives for patient referral.
Notably, 61% of centres claimed that screening for gastro-oesophageal varices was not routinely practised. Where gastro-oesophageal varices were identified, they were either always (n=6) or occasionally (n=8) considered to contraindicate allo-HCT as a course of treatment. If patients had a recorded history of portal vein thrombosis, 78% of centres would invariably refuse allo-HCT. Non-portal splanchnic vein thrombosis was weighted with far less severity, with only one centre viewing it as a contraindication to transplant. Where cavernomas were observed, a mere 44% of centres would proceed to transplant. Need for transjugular intrahepatic portosystemic shunt was disregarded as a contraindication to transplant by 63% of centres.
Ultimately, the study highlights extensive heterogeneity in clinical practice in the treatment of MF across centres. This indicates the need for specific, formal guidelines outlining the course of pre- and post-transplant monitoring, and the decision to proceed with, or else contraindicate, transplant.
