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Myeloproliferative neoplasm — Clinical Guidelines

Overview

Myeloproliferative neoplasms (MPNs) are clonal hematopoietic cancers characterized by excessive proliferation of one or more cell lines, including polycythemia vera, essential thrombocythemia, and primary myelofibrosis. These conditions often have an indolent course but are associated with significant thrombotic and hemorrhagic risks 1 15.

Diagnosis

Key Mutations: JAK2, CALR, and MPL mutations are crucial for diagnosis 1 10 16.

Cytogenetic Analysis: Important for confirming diagnosis and guiding management, especially in Philadelphia-negative MPNs 1 9.

Clinical Criteria: World Health Organization (WHO) criteria used for morphological diagnosis; interobserver reproducibility varies 14.

Laboratory Tests: Elevated blood cell counts (e.g., platelets, red cells) and decreased erythropoietin levels in essential thrombocythemia 20.

Imaging and Biopsy: Bone marrow biopsy essential for morphological assessment 14.

Management

First-Line Treatments:

- Hydroxyurea: Commonly used for cytoreduction in polycythemia vera 13. - Anagrelide: Used for platelet control in essential thrombocythemia 13. - Ruxolitinib: Effective for splenomegaly and symptoms in myelofibrosis 17.

Thromboprophylaxis:

- Risk Assessment: Use of whole blood platelet aggregation studies (WBPA) for tailored prophylaxis 2. - JAK Inhibitors: Evidence supports their use for reducing thrombotic events 11 (Evidence: Moderate).

Adjunctive Treatments:

- BET/JAK Inhibition: Emerging therapeutic approach targeting inflammation 8. - Management of Comorbidities: Address hyperhomocysteinemia, especially in patients with thrombotic risk 19.

Special Populations

Elderly Patients: Increased risk of thrombotic events; careful monitoring and individualized prophylaxis recommended 6.

Comorbidities: Hyperhomocysteinemia prevalent; folate and cobalamin supplementation considered 19.

Pregnancy: Limited data; individualized management focusing on maternal and fetal safety 13.

Pediatrics: Rare; management tailored to specific subtype and clinical presentation 13.

Key Recommendations

Utilize molecular markers (JAK2, CALR, MPL) for accurate diagnosis and risk stratification 1 10 (Evidence: Strong).

Implement risk-adapted thromboprophylaxis strategies, including WBPA studies, to manage thrombotic risk effectively 2 (Evidence: Moderate).

Consider JAK inhibitors for patients with intermediate to high-risk MPNs to reduce major morbid events 11 (Evidence: Moderate).

Monitor for hyperhomocysteinemia and address deficiencies in folate and cobalamin to mitigate thrombotic risk 19 (Evidence: Moderate).

Tailor treatment plans considering patient-specific factors such as age and comorbidities 6 19 (Evidence: Expert opinion).

References

1 Decamp M, Klein E, Godon C, Lestringant V, Roynard P, Theisen O et al.. Cytogenetics in the management of myeloproliferative neoplasms, mastocytosis and myelodysplastic/myeloproliferative neoplasms: Guidelines from the Group Francophone de Cytogénétique Hématologique (GFCH). Current research in translational medicine 2023. link 2 Manoharan A, Gemmell R, Cavanaugh L, Shadood N. Thrombosis in Myeloproliferative Neoplasms: A Single Center Experience of Using Whole Blood Platelet Aggregation Studies for Risk Assessment and Thromboprophylaxis. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2022. link 3 Mughal TI, Pemmaraju N, Bejar R, Gale RP, Bose P, Kiladjian JJ et al.. Perspective: Pivotal translational hematology and therapeutic insights in chronic myeloid hematopoietic stem cell malignancies. Hematological oncology 2022. link 4 Sah SK, Shah S, Tiwari SB, Poudel BS, Singh B, Sharma P et al.. Essential Thrombocythemia among Patients with Myeloproliferative Neoplasms in Haematology Unit of a Tertiary Care Centre: A Descriptive Cross-sectional Study. JNMA; journal of the Nepal Medical Association 2022. link 5 Felser S, Gube M, Gruen J, Coutre PI, Schulze S, Muegge LO et al.. Association Between Cancer-Related Fatigue and Falls in Patients With Myeloproliferative Neoplasms: Results of a Multicenter Cross-Sectional Survey From the East German Study Group for Hematology and Oncology (OSHO #97). Integrative cancer therapies 2022. link 6 Koschmieder S. How I Manage Thrombotic/Thromboembolic Complications in Myeloproliferative Neoplasms. Hamostaseologie 2020. link 7 . New Classification for Myeloproliferative Neoplasms. Cancer discovery 2018. link 8 . Myeloproliferative Neoplasms May Be Sensitive to Dual BET/JAK Inhibition. Cancer discovery 2018. link 9 Bilhou-Nabéra C, Bidet A, Eclache V, Lippert E, Mozziconacci MJ. Cytogenetics in the management of Philadelphia-negative myeloproliferative neoplasms: an update by the Groupe francophone de cytogénétique hématologique (GFCH). Annales de biologie clinique 2016. link 10 Mambet C, Matei L, Necula LG, Diaconu CC. A link between the driver mutations and dysregulated apoptosis in BCR-ABL1 negative myeloproliferative neoplasms. Journal of immunoassay & immunochemistry 2016. link 11 Pemmaraju N, Mesa R. Evidence for Janus kinase (JAK) inhibitors for the prevention of major morbid events in patients with myeloproliferative neoplasms (MPNs). Hematology. American Society of Hematology. Education Program 2015. link 12 Pahl HL. Many roads lead to MPN. Blood 2014. link 13 Meier B, Burton JH. Myeloproliferative disorders. Emergency medicine clinics of North America 2014. link 14 Madelung AB, Bondo H, Stamp I, Loevgreen P, Nielsen SL, Falensteen A et al.. World Health Organization-defined classification of myeloproliferative neoplasms: morphological reproducibility and clinical correlations--the Danish experience. American journal of hematology 2013. link 15 Falanga A, Marchetti M. Thrombotic disease in the myeloproliferative neoplasms. Hematology. American Society of Hematology. Education Program 2012. link 16 Kiladjian JJ. The spectrum of JAK2-positive myeloproliferative neoplasms. Hematology. American Society of Hematology. Education Program 2012. link 17 Constantinescu SN, Vainchenker W. Small-molecule inhibitors in myeloproliferative neoplasms: are we aiming for the right targets?. Hematology. American Society of Hematology. Education Program 2012. link 18 Papadakis E, Hoffman R, Brenner B. Thrombohemorrhagic complications of myeloproliferative disorders. Blood reviews 2010. link 19 Faurschou M, Nielsen OJ, Jensen MK, Hasselbalch HC. High prevalence of hyperhomocysteinemia due to marginal deficiency of cobalamin or folate in chronic myeloproliferative disorders. American journal of hematology 2000. link65:2<136::aid-ajh8>3.0.co;2-6) 20 Viala JJ, Ville D, Sebban C, Assouline D, Devaux Y, Hanss M. Plasma erythropoietin in essential thrombocythaemia. Nouvelle revue francaise d'hematologie 1993. link 21 Hoo JJ, O'Brien S, Samuel I. Double supernumerary isochromosome 9p in myeloproliferative syndrome. Cancer genetics and cytogenetics 1987. link90243-3) 22 Redlin L, Francis RS, Orlando MM. Renal abnormalities in agnogenic myeloid metaplasia. Radiology 1976. link 23 Ghosh ML. Primary haemorrhagic thrombocythaemia with Philadelphia chromosome. Postgraduate medical journal 1972. link

Myeloproliferative neoplasm