Excellus BCBS New Policy – (#2.02.53) JAK2, MPL, and CALR Testing for Myeloproliferative Neoplasms

Reviewed and approved in June 2021 – Myeloproliferative Neoplasms (MPNs) are a subsect of chronic myeloid disorders that usually exhibit terminal expansion in the peripheral blood as opposed to the bone marrow. MPNs cause thrombocytosis and erythrocytosis in the peripheral blood. The four main types of MPNs are PV (Polycythemia Vera), ET (Essential Thrombocythemia), PMF (primary myelofibrosis), and CML (chronic myeloid leukemia). Somatic (acquired) variants in the JAK2 gene are found in patients with MPNs such as PV, ET, and PMF. Somatic variants in the MPL gene are associated with ET and PMF. Somatic variants in the CALR gene are associated with ET and PMF. The presence of a documented JAK2 variant may aid in the selection of ruxolitinib, a JAK2 inhibitor; ruxolitinib, however, is classified as second-line therapy. According to NCCN, MPL and CALR testing may be consistent with clinical practice in the diagnosis of patients with clinical, laboratory, or pathological findings suggesting ET and PMF. JAK2, MPL, and CALR testing is considered medically necessary in the diagnosis of patients presenting with clinical, laboratory, or pathologic findings suggesting polycythemia vera (PV), essential thrombocythemia (ET), or primary myelofibrosis (PMF) after evaluation for secondary causes, JAK2, MPL, and CALR testing is investigational in all other circumstances.

Source: https://provider.excellusbcbs.com/documents/53971/224494/August+2021+Connection+Newsletter.pdf/81e8bfa0-2f70-3a93-ffe2-3a6346a5345c?t=1627647528496