Disease Progression in Myeloproliferative Neoplasms
Disease Progression in Myeloproliferative Neoplasms
Objectives: This session of the Society for Hematopathology/European Association for Haematopathology workshop focused on disease progression in myeloproliferative neoplasms (MPNs).
Methods: The session included typical and unusual presentations of chronic myelogenous leukemia (CML), BCR-ABL1 positive; Philadelphia chromosome–negative (Ph-neg) MPNs; and mastocytosis.
Results: Cases of CML illustrated various manifestations of progression, with emphasis on criteria defining stages of the disease. Issues were discussed related to the patterns of recurrence in patients receiving tyrosine kinase inhibitor therapy, including leukemic transformation occurring in a Ph-neg clone. Ph-neg MPN cases highlighted diagnostic approaches used to establish accelerated and blast phases, including cases with significant myelofibrosis and when an adequate bone marrow aspirate smear is not available. The session also included rare cases of aggressive mastocytosis.
Conclusions: There was agreement that a definitive diagnosis can be challenging in the absence of documented review of prior diagnostic material and clinical history.
Myeloproliferative neoplasms (MPNs) are chronic hematopoietic stem cell malignant neoplasms defined by a proliferation of select myeloid lineages. This group of diseases includes chronic myelogenous leukemia (CML), BCR-ABL1 positive; primary myelofibrosis (PMF); essential thrombocythemia (ET); polycythemia vera (PV); chronic neutrophilic leukemia (CNL); chronic eosinophilic leukemia, not otherwise specified (CEL-NOS); systemic mastocytosis (SM); and poorly defined MPN that are unclassifiable (MPN-U). The clinical course of these neoplasms is highly variable and ranges from a slow protracted course to a progressive course with transformation to an acute leukemic phase. Targeted therapies developed in the past decade have significantly modified the natural history of these disorders and brought about new patterns of disease evolution and recurrence. Disease progression in MPNs was one of the topics of the 2013 Workshop of the Society for Hematopathology/European Association for Haematopathology and is the focus of this report.
Abstract and Introduction
Abstract
Objectives: This session of the Society for Hematopathology/European Association for Haematopathology workshop focused on disease progression in myeloproliferative neoplasms (MPNs).
Methods: The session included typical and unusual presentations of chronic myelogenous leukemia (CML), BCR-ABL1 positive; Philadelphia chromosome–negative (Ph-neg) MPNs; and mastocytosis.
Results: Cases of CML illustrated various manifestations of progression, with emphasis on criteria defining stages of the disease. Issues were discussed related to the patterns of recurrence in patients receiving tyrosine kinase inhibitor therapy, including leukemic transformation occurring in a Ph-neg clone. Ph-neg MPN cases highlighted diagnostic approaches used to establish accelerated and blast phases, including cases with significant myelofibrosis and when an adequate bone marrow aspirate smear is not available. The session also included rare cases of aggressive mastocytosis.
Conclusions: There was agreement that a definitive diagnosis can be challenging in the absence of documented review of prior diagnostic material and clinical history.
Introduction
Myeloproliferative neoplasms (MPNs) are chronic hematopoietic stem cell malignant neoplasms defined by a proliferation of select myeloid lineages. This group of diseases includes chronic myelogenous leukemia (CML), BCR-ABL1 positive; primary myelofibrosis (PMF); essential thrombocythemia (ET); polycythemia vera (PV); chronic neutrophilic leukemia (CNL); chronic eosinophilic leukemia, not otherwise specified (CEL-NOS); systemic mastocytosis (SM); and poorly defined MPN that are unclassifiable (MPN-U). The clinical course of these neoplasms is highly variable and ranges from a slow protracted course to a progressive course with transformation to an acute leukemic phase. Targeted therapies developed in the past decade have significantly modified the natural history of these disorders and brought about new patterns of disease evolution and recurrence. Disease progression in MPNs was one of the topics of the 2013 Workshop of the Society for Hematopathology/European Association for Haematopathology and is the focus of this report.
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