Aim: To investigate the potential association between the chronic myeloid d
isorders (CMDs), including the chronic myeloproliferative disorders, and pu
lmonary hypertension (PH).
Methods: Retrospective chart review of patients who had received diagnoses
of both CMD and PH. Patients with a known cause of PH were excluded. The di
agnosis of a CMD was based on established criteria. The diagnosis of PH was
based on echocardiographic data or right heart catheterization data.
Results: Twenty-six patients satisfied the criteria for both a CMD and PH.
Twelve patients had myeloid metaplasia with myelofibrosis (MMM), 5 patients
had essential thrombocythemia (ET), 6 patients had polycythemia vera, 2 pa
tients had a myelodysplastic syndrome, and 1 patient had chronic myeloid le
ukemia. Twenty-two patients (92%) received treatment for their CMDs, which
included therapy with hydroxyurea (18 patients), anagrelide (7 patients), a
nd busulfan (3 patients). PH was diagnosed a median of 8 years after recogn
ition of the CMD (range, 0 to 26 years). The median right ventricular systo
lic pressure (RVsys) was 71 mm Hg (range, 32 to 105 mm Hg). RVsys correlate
d with the platelet count in patients with MMM (r = 0.30) and ET (r = 0.6)
and with the hemoglobin levels in patients with PV (r = 0.77). Treatment of
CMD did not seem to affect the severity of the pulmonary artery pressures
as measured by serial echocardiography. With a median survival time of 18 m
onths after the diagnosis of PH, the cause of death in the majority of the
patients was cardiopulmonary.
Conclusions: The current study suggests a higher than expected incidence of
PH in patients with MMM, PV, and ET. Prognosis in such a setting is poor a
nd may not be influenced by aggressive treatment of the underlying hematolo
gic disorder.