18F-FLT (PET/CT) in Prefibrotic/Early Primary Myelofibrosis and Essential Thrombocythemia (PMF/ET-FLT)

This is an interventional diagnostic trial for Essential Thrombocythemia Read More

Inclusion Criteria:

Age 18 year old or above Patient accept to sign inform consent ECOG (Eastern Cooperative Oncology Group) performance less than or equal 2

• Cases fulfilling WHO (World Health Organization) 2016 diagnostic criteria for PMF:

WHO criteria for prefibrotic/early primary myelofibrosis (prePMF)

Major criteria:

1. Megakaryocytic proliferation and atypia, without reticulin fibrosis > grade 1*, accompanied by increased age-adjusted BM cellularity, granulocytic proliferation and often decreased erythropoiesis
2. Not meeting the WHO criteria for BCR-ABL1+ ((BCR-ABL = fusion gene from BCR (breakpoint cluster region gene/BCR gene product) and ABL (Abelson proto-oncogene)) CML (chronic myelogenous leukemia), PV (Polycythemia Vera), ET, myelodysplastic syndromes, or other myeloid neoplasms
3. Presence of JAK2, CALR or MPL mutation or in the absence of these mutations, presence of another clonal marker**or absence of minor reactive BM reticulin fibrosis.

Minor criteria:

Presence of at least one of the following, confirmed in two consecutive determinations:

1. Anemia not attributed to a comorbid condition
2. Leukocytosis >11 x 109/L
3. Palpable splenomegaly
4. LDH (Lactate dehydrogenase) increased to above upper normal limit of institutional reference range.

Diagnosis of prePMF requires meeting all three major criteria, and at least one minor criterion **in the absence of any of the 3 major clonal mutations, the search for the most frequent accompanying gene mutations (e.g. ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, SF3B1) are of help in determining the clonal nature of the disease.

*** minor (grade 1) reticulin fibrosis secondary to infection, autoimmune disorder or other chronic inflammatory conditions, hairy cell leukemia or other lymphoid neoplasm, metastatic malignancy, or toxic (chronic) myelopathies

WHO diagnostic criteria essential thrombocythemia Major criteria Platelet count ≥450 × 109/L Bone marrow biopsy showing proliferation mainly of the megakaryocyte lineage with increased numbers of enlarged, mature megakaryocytes with hyperlobulated nuclei. No significant increase or left shift in neutrophil granulopoiesis or erythropoiesis and very rarely minor (grade 1) increase in reticulin fibers.

Not meeting WHO criteria for BCR-ABL1+ CML, PV, PMF, myelodysplastic syndromes, or other myeloid neoplasms Presence of JAK2, CALR, or MPL mutation Minor criterion Presence of a clonal marker or absence of evidence for reactive thrombocytosis Diagnosis of ET requires meeting all 4 major criteria or the first 3 major criteria and the minor criterion

Exclusion Criteria:

• Patient not fulfilling the inclusion criteria.
• Vulnerable groups: pregnant, minors, prisoners will not be included.
• Bone marrow will be collected as part of the routine diagnostic work-up. No extra bone marrow material will be collected solely for the aim of the study.