Clonal Megakaryocyte Dysplasia with Normal Blood Values Is a Distinct Myeloproliferative Neoplasm

In 1991, we reported 18 persons with a clinical-pathologic entity and termed atypical myeloproliferative disorder because they did not meet the contemporary diagnostic criteria for a myeloproliferative neoplasm. We sought to gain further knowledge on this disease entity. This retrospective cohort st...

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Vydáno v:	Acta haematologica Ročník 145; číslo 1; s. 30
Hlavní autoři:	Barosi, Giovanni, Rosti, Vittorio, Massa, Margherita, Campanelli, Rita, Villani, Laura, Catarsi, Paolo, Carolei, Adriana, Abbà, Carlotta, Lodigiani, Corrado, Primignani, Massimo, Gregato, Giuliana, Bertolini, Francesco, Magrini, Umberto, Gale, Robert Peter
Médium:	Journal Article
Jazyk:	angličtina
Vydáno:	Switzerland 01.01.2022
Témata:	Adult Amino Acid Substitution Bone Marrow - metabolism Bone Marrow - pathology Calreticulin - genetics Calreticulin - metabolism Female Hematologic Neoplasms - blood Hematologic Neoplasms - genetics Hematologic Neoplasms - pathology Humans Janus Kinase 2 - genetics Janus Kinase 2 - metabolism Male Megakaryocytes - metabolism Megakaryocytes - pathology Middle Aged Mutation, Missense Myeloproliferative Disorders - blood Myeloproliferative Disorders - genetics Myeloproliferative Disorders - pathology Retrospective Studies Thrombosis Prefibrotic myelofibrosis Megakaryocyte dysplasia Atypical myeloproliferative disorder Myelofibrosis Myeloproliferative neoplasm
ISSN:	1421-9662, 1421-9662
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Shrnutí:	In 1991, we reported 18 persons with a clinical-pathologic entity and termed atypical myeloproliferative disorder because they did not meet the contemporary diagnostic criteria for a myeloproliferative neoplasm. We sought to gain further knowledge on this disease entity. This retrospective cohort study included consecutive subjects registered in the database of the Center for the Study of Myelofibrosis in Pavia, Italy, from 1998 to 2020 (June), and diagnosed with atypical myeloproliferative disorder according to our adjudicated criteria. We studied clinical, histological, cytogenetic, and molecular covariates and risks of thrombosis, disease progression, and death. Data were compared with those of concurrent subjects with prefibrotic myelofibrosis. Fifteen new subjects with atypical myeloproliferative disorder were identified. Seven were male. Median age was 50 years (IQR, 41-54 years). Thirteen were diagnosed with a synchronous symptomatic or incidentally detected thrombotic event. The bone marrow showed megakaryocyte hyperplasia with dysplasia. JAK2V617F was present in 10 subjects and CALR mutation in one. No other somatic mutations were identified in next generation sequencing. After a median follow-up of 101 months (IQR, 40-160 months), no subject had disease progression or blast transformation. Incidence of post-diagnosis or recurrent thrombosis was 3.9 events (95% confidence interval, 3.5-4.0) and 5.0 events (4.6-5.6) per 100 person-years. Features of subjects with atypical myeloproliferative disorder differed markedly from those of 546 subjects with prefibrotic myelofibrosis. Our data indicate that these 15 persons have a distinct myeloproliferative neoplasm. We propose naming this new disorder clonal megakaryocyte dysplasia with normal blood values.
Bibliografie:	ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-1 content type line 23
ISSN:	1421-9662 1421-9662
DOI:	10.1159/000517207