A fascinating study has just been released about diagnosis and treatment of patients with ET and MF in Germany, especially as a precise diagnosis impacts on access to approved therapies.

“This study reports on a chart review conducted to evaluate the real life approach regarding clinical characteristics, diagnostic assessment, risk stratification and treatment decisions for MPN patients classified as ET or MF after implementation of the WHO 2016 classification.”

“Of note, the proportion of JAK2 mutated patients was significantly higher in this selected cohort compared to published molecular studies. The presence of JAK2 driver mutations is associated with higher systemic inflammation, thromboembolic risk and disease progression.”

“Classification of MPN subtypes is not only critical to inform patients about risk of disease progression and probability of survival but also regarding access to approved pharmacologic therapies.
As shown in this chart review, more than 10% of patients classified as ET or MF report on significant symptom burden and may benefit from JAK-inhibitor treatment, approved for the treatment of disease-related splenomegaly or symptoms in adult patients with myelofibrosis. Conversely, the need for cytoreductive therapies to achieve TE risk reduction has been perceived and cytoreductive treatment had been initiated according to guideline recommendations (mainly using hydroxycarbamide/hydroxyurea).
This is of major importance considering the large number of patients in this chart review showing clinical and histopathologic findings consistent with pre-fibrotic myelofibrosis.”

Improved histopathologic diagnostics, dynamic risk stratification including genetic risk factors for cases of suspected ET and MF are recommended for precise risk assessment and therapeutic stratification according to WHO criteria.”

The full article titled ‘Diagnosis and treatment of MPN in real life: exploratory and retrospective chart review including 960 MPN patients diagnosed with ET or MF in Germany’ is available HERE.