Zeitschrift für Hämatologie und thromboembolische Erkrankungen
Offener Zugang

Abstrakt

Bone Marrow Histology Characteristics in MPL515 Mutated Thrombocythemia with Various Degrees of Myelofibrosis: A Cross Sectional Follow-up Study in Eight Cases

Michiels JJ*, De Raeve H, Schwarz J, Campr V, Kim Y and Kim M

MPLW515l/K mutated Essential Thrombocythemia (ET) usually present with increased platelet counts around 1000 x 109/L as the only abnormal laboratory finding with normal values for hemoglobin and leukocytes and minor splenomegaly on palpation only in a few. Early stage MPL515 mutated ET show the presence of clustered small and giant megakaryocytes with pronounced deeply lobulated nuclei, which are not seen in JAK2V617F positive ET, prodromal PV, and classical PV. Indicating that MPL515 mutated ET has no clinical, laboratory and bone marrow features of prodromal PV. Clustering of large mature large to giant megakaryocytes with pronounced hyper lobulated nuclei in a normocellular bone marrow is the hallmark of JAK2-wild type MPL515 mutated thrombocythemia. Bone marrow histology in MPL515 mutant patients revealed isolated megakaryocytic proliferation in a normocellular bone marrow at diagnosis with a reduction of erythropoiesis. MPL515 Thrombocythemia do not have PV features at diagnosis, do not evolve into PV during follow-up, have normal LAP score, serum EPO and ferritin levels. In contrast to JAK2V617F positive ET, no spontaneous endogenous erythroid colonies (EEC) was found in any of evaluated MPLW515L cases. Spontaneous megakaryocyte growth in culture with an overall normal response to thrombopoietin (TPO) has been demonstrated in two MPL515 mutated cases.