Therapy-associated leukemic Transformation in Myeloproliferative Neoplasms – What do we know?

Publication date: Available online 8 February 2019Source: Best Practice &Research Clinical HaematologyAuthor(s): Danielle Cuthbert, Brady Lee SteinAbstractMyeloproliferative Neoplasms (MPNs) are a group of progressive diseases that share a common pathogenesis, clinical and laboratory features, as well as a spontaneous risk of secondary AML. Certain MPN therapies have been associated with an increased risk of leukemic conversion, with robust data highlighting the highest rates with 32P, chlorambucil, and pipobroman. Herein, we review risk factors for leukemic transformation, including therapy-related MPN-BP, with a focus on the debate surrounding the potential leukemogenicity of hydroxyurea. Lastly, we discuss emerging studies on the association between ruxolitinib and high grade B-cell lymphomas. We conclude that statistical associations have not implicated hydroxyurea monotherapy as leukemogenic. However, it is difficult to definitely disprove an association, as large prospective, controlled studies with decades of follow-up would be needed to draw conclusions. Overall, the concept of therapy-related neoplasms remains important to the field, and mandates judicious selection and sequencing of therapies for MPN patients.
Source: Best Practice and Research Clinical Haematology - Category: Hematology Source Type: research

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ConclusionThe prevalence of PNH clones in MPN is similar to that described in MDS. Whether PNH clones influence MPN phenotype and complications should be studied prospectively in larger patient cohorts and over long term follow up.
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
Publication date: Available online 1 April 2019Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Patrizia Mondello, Cristian Di Mirto, Salvatore Cuzzocrea, Carmela Arrigo, Michael Mian, Vincenzo PitiniAbstractBackgroundDespite the important progress in the research of myeloproliferative neoplasms (MPN), treatment options are still limited. Currently, a cytoreductive approach is the backbone treatment, with hydroxyurea (HU) being the most important agent. However, this drug is not always well-tolerated and has been questionably linked to a potential leukemogenic effect. A valid alternative is interferon alfa (IFN-&al...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
ConclusionWe recommend routine screening for venous thrombosis in any case of MPN when diagnosed and screening for MPNs in any patient with venous thrombosis especially of the portal vein or atypical sites. If MPN patients present with increasing pruritus or abdominal pain, they also should be screened for venous thrombosis. Further research on a large scale in MPN age groups younger than 60 years regarding pathogenesis of thrombosis is highly recommended.
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
ConclusionSymptom burden in patients with PV can persist despite control of blood counts, which suggests some discordance between laboratory values and symptom burden. Consequently, regular monitoring of symptom burden should be factored in to the assessment of disease control.
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
1155Introduction: In FDG-PET imaging, it is necessary to localize the lesion and differentiate FDG uptake due to malignancy or inflammation from physiological or reactive FDG uptake with little pathological significance. In particular, in the diagnosis of bone marrow lesions in the spine, the differential diagnosis must include degeneration, fracture, reactive uptake in response to increased bone marrow hematopoietic function, bone metastasis, myeloproliferative diseases such as lymphoma, changes secondary to treatment, and recurrence. Bone marrow is a hematopoietic tissue with a physiologically high proliferative capacity...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Educational Exhibit Posters Source Type: research
Conclusionwe recommend routine screening for venous thrombosis in any case of MPNs once diagnosed and screening for MPNs in any patient with venous thrombosis especially of the portal vein or atypical sites. If MPN patients presented with increasing pruritus or abdominal pain, they should be also screened for venous thrombosis. Further research on large scale in MPN group age below 60 years regarding pathogenesis of thrombosis is highly recommended.
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
Venous thrombosis screening is not routinely performed in patients with myeloproliferative neoplasms unless the patient is symptomatic. It has been reported that the incidence of thrombosis in elderly patients is much higher than in young patients. The aim of this work was to screen 73 patients with janus kinase 2-positive myeloproliferative disorder (MPN) for venous thrombosis and study its correlation with JAK2 allele burden and with MPN 10 score. Fifty-three patients were younger than 60 years. Only 3 patient were symptomatic with abdominal pain during screening. There was no significant difference in MPN 10 score betwe...
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Tags: Original Study Source Type: research
Identification of JAK-2 mutation even in absence of myeloproliferative disorders (MPNs) was found to be related to venous thromboembolism occurrence. Venous thrombosis screening is not routinely requested in patient with myeloproliferative neoplasm unless the patient is symptomatic and it is stated that the incidence of thrombosis in elderly is much higher than young. The aim of this work was to screen MPN patients for venous thrombosis and study its correlation with both Jak 2 allele burden and with MPN10 score.
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Tags: Original Study Source Type: research
Conclusions The discovery of JAK2V617F mutation in BCR-ABL1-negative MPNs by four different international cooperative groups in 2005 (2–5) led to significant insights on the pathogenesis of these disorders. In fact, this mutation results in a gain-of-function with activation of cytokine and growth factor receptors, and thus of the downstream JAK-STAT pathway (79, 95–98). The JAK2 point mutation in exon 12, present in a small percentage of patients with PV, is able to induce the MPN phenotype through the same pathogenic mechanism (6, 7). In 2006 the MPLW515L/K was reported in ET and PMF patients (44, 45) and d...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
Publication date: Available online 1 April 2019Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Patrizia Mondello, Cristian Di Mirto, Salvatore Cuzzocrea, Carmela Arrigo, Michael Mian, Vincenzo Pitini MAbstractBackgroundDespite the important progress in the research of myeloproliferative neoplasms (MPN), treatment options are still limited. Currently, a cytoreductive approach is the backbone treatment, with hydroxyurea (HU) being the most important agent. However, this drug is not always well tolerated and seems to be associated with a potential leukemogenic effect. A valid alternative is interferon alfa (IFN-&alph...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
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