ABSTRACT
Enthusiasm about interferons for the treatment of myeloproliferative neoplasms has recently arisen. How does a nontargeted therapy selectively target the malignant clone? Many foundational questions about interferon treatment are unanswered, including who, when, and for how long do we treat. Using an individual case, this review touches on gaps in risk assessment in polycythemia vera (PV) and essential thrombocythemia (ET) and the history of treatment with interferons. How is it that this proinflammatory cytokine effectively treats ET and PV, themselves proinflammatory states? We summarize existing mechanistic and clinical data, the molecular context as a modifier for treatment response, the establishment of treatment goals, and the challenges that lie ahead.
Subject(s)
Antineoplastic Agents/therapeutic use , Interferons/therapeutic use , Polycythemia Vera/drug therapy , Thrombocythemia, Essential/drug therapy , Adult , Female , Humans , Polycythemia Vera/diagnosis , Polycythemia Vera/epidemiology , Risk Assessment , Thrombocythemia, Essential/diagnosis , Thrombocythemia, Essential/epidemiologyABSTRACT
BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF). AIM: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. MATERIAL AND METHODS: Retrospective review of medical records of all patients referred as MPN from 2012 to 2017. Patients with (9;21) translocation were excluded. RESULTS: Data of 462 cases with a median age of 69 years from 10 public hospitals was reviewed. ET was the most frequently Ph-MNP found. The incidence of Ph-MPN was 1.5 x 100.000 cases. The JAK2 V617F mutation study was performed in 96% of patients and only 30% had a bone marrow biopsy. Thrombotic events were observed in 29% of patients. Bleeding events were observed in 7%. Five-year overall survival was 87%. CONCLUSIONS: ET is the most frequent Ph-MPN. The mean incidence was lower than reported in the literature, in part because of a sub diagnosis.
Subject(s)
Myeloproliferative Disorders , Polycythemia Vera , Primary Myelofibrosis , Thrombocythemia, Essential , Aged , Chile/epidemiology , Humans , Janus Kinase 2/genetics , Mutation , Myeloproliferative Disorders/diagnosis , Myeloproliferative Disorders/epidemiology , Myeloproliferative Disorders/genetics , Polycythemia Vera/diagnosis , Polycythemia Vera/epidemiology , Polycythemia Vera/genetics , Primary Myelofibrosis/diagnosis , Primary Myelofibrosis/epidemiology , Primary Myelofibrosis/genetics , Thrombocythemia, Essential/diagnosis , Thrombocythemia, Essential/geneticsABSTRACT
BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF). AIM: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. MATERIAL AND METHODS: Retrospective review of medical records of all patients referred as MPN from 2012 to 2017. Patients with (9;21) translocation were excluded. RESULTS: Data of 462 cases with a median age of 69 years from 10 public hospitals was reviewed. ET was the most frequently Ph-MNP found. The incidence of Ph-MPN was 1.5 x 100.000 cases. The JAK2 V617F mutation study was performed in 96% of patients and only 30% had a bone marrow biopsy. Thrombotic events were observed in 29% of patients. Bleeding events were observed in 7%. Five-year overall survival was 87%. CONCLUSIONS: ET is the most frequent Ph-MPN. The mean incidence was lower than reported in the literature, in part because of a sub diagnosis.
Subject(s)
Myeloproliferative Disorders , Polycythemia Vera , Primary Myelofibrosis , Thrombocythemia, Essential , Aged , Chile/epidemiology , Humans , Janus Kinase 2/genetics , Mutation , Myeloproliferative Disorders/diagnosis , Myeloproliferative Disorders/epidemiology , Myeloproliferative Disorders/genetics , Polycythemia Vera/diagnosis , Polycythemia Vera/epidemiology , Polycythemia Vera/genetics , Primary Myelofibrosis/diagnosis , Primary Myelofibrosis/epidemiology , Primary Myelofibrosis/genetics , Thrombocythemia, Essential/diagnosis , Thrombocythemia, Essential/geneticsABSTRACT
INTRODUCTION: Polycythemia vera (PV) is a disorder characterized by clonal proliferation of myeloid cells and increased red blood cell mass. Recently, the revised 2016 WHO classification of myeloid neoplasms decreased the threshold levels of hemoglobin and hematocrit for the diagnosis of PV. However, the new proposed cutoffs have remarkable overlap with the normal reference values reported and the clinical impact of these new cutoffs has not been widely assessed in the general population. METHODS: We retrospectively examined 248 839 patients with presumptively normal complete blood cell results, consecutively obtained in an outpatient setting. RESULTS: The proportion of men with Hb >165 g/L was 5.99%, Hct>49% was 2.4%, and Hb >165 g/dL or Hct>49% was 6.48%, while the proportion of women with Hb >160 g/L was 0.22%, Hct>48% was 0.11%, and Hb >160 g/L or Hct>48% was 0.28%. CONCLUSION: The isolated use of the proposed Hb/Hct levels as a definer of polycythemia may lead to a substantial increase in unnecessary diagnostic tests. In cases with borderline levels of hemoglobin, the diagnostic workup of PV should only be indicated in the presence of clinical and/or laboratorial features associated with MPN.
Subject(s)
Blood Cell Count , Polycythemia Vera/blood , Polycythemia Vera/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Erythrocyte Indices , Female , Humans , Incidence , Male , Middle Aged , Myeloproliferative Disorders/blood , Myeloproliferative Disorders/diagnosis , Myeloproliferative Disorders/epidemiology , Polycythemia Vera/diagnosis , Population Surveillance , Retrospective Studies , Young AdultABSTRACT
BACKGROUND: The chronic myeloproliferative diseases (CMPDs) include chronic myelogenous leukemia (CML), primary (essential) thrombocythemia (PT), agnogenic myeloid metaplasia (AMM), and polycythemia vera (PV). Certain hematological malignancies have a different prevalence in our country than in countries with Caucasian populations. Data indicate that the prevalence of CML in our country is similar to that found in Caucasians; however, the prevalence of the other CMPDs has not been studied. METHODS: In a total of 8069 individuals studied between June 1983 and March 2001 in the Centro de Hematologia y Medicina Interna de Puebla, we assessed the prevalence of CML, PT, AMM, and PV. Some of the clinical features of these individuals were also assessed. RESULTS AND CONCLUSIONS: Forty-nine patients with CML, 14 with PT, 7 with AMM, and 3 with PV were identified. The clinical presentations of these CMPDs were not different from those described in Caucasians. CONCLUSIONS: We found that CML was more than 3 times more frequent than PT, that both PV and AMM were exceptional, and that PT, AMM, and PV were significantly less frequent in Mexican than in Caucasian populations (P < .01).