{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:54:36Z","timestamp":1760144076845,"version":"build-2065373602"},"reference-count":26,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2024,3,21]],"date-time":"2024-03-21T00:00:00Z","timestamp":1710979200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: The occurrence of myeloproliferative neoplasms (MPNs) that evolve into each other is well-described, as is this occurrence of lymphoproliferative neoplasms (LPNs). However, less is known about rare MPN\/LPN coexistence, and the aim of our study was to analyze charachteristics of these patients after long term follow-up. Methods: Fourteen patients with MPN\/LPN coexistence were diagnosed and treated according to guidelines at a single university center across two decades. Results: The overall median age was 53 years (22\u201369). MPNs patients with subsequent LPNs had a shorter period of second malignancy development and a more aggressive course of LPN, which can cause fatal outcomes. Polycythemia vera and chronic lymphocytic leukemia were most commonly associated (36%). The JAK2V617F mutation had 2\/3 and cytogenetic abnormalities occurred in 1\/3 of patients. MPN\/LPN coexistence cases had significantly higher thrombotic potential (42.8%) and a higher third malignancy accruement frequency (21.4%) versus those without such malignancies. Conclusions: Considering the younger ages at MPN diagnosis, it is recommended to check regularly for blood lymphocytosis or lymphadenopathy occurrences and organomegaly progression faster than expected for MPN, with the aim of timely LPN diagnoses. The presence of molecular-cytogenetic abnormalities in a majority of patients indicate possible genetic instability and increased risk of development of multiple neoplasms, thus elevating thrombotic risk.<\/jats:p>","DOI":"10.3390\/jcm13061816","type":"journal-article","created":{"date-parts":[[2024,3,21]],"date-time":"2024-03-21T06:35:30Z","timestamp":1711002930000},"page":"1816","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Does Double Mean Trouble? Coexistence of Myeloproliferative and Lymphoproliferative Neoplasms"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6194-8298","authenticated-orcid":false,"given":"Danijela","family":"Lekovic","sequence":"first","affiliation":[{"name":"Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia"},{"name":"Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jelena","family":"Ivanovic","sequence":"additional","affiliation":[{"name":"Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5868-0422","authenticated-orcid":false,"given":"Tatjana","family":"Terzic","sequence":"additional","affiliation":[{"name":"Institute of Pathology, Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maja","family":"Perunicic Jovanovic","sequence":"additional","affiliation":[{"name":"Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marija","family":"Dencic Fekete","sequence":"additional","affiliation":[{"name":"Institute of Pathology, Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jelica","family":"Jovanovic","sequence":"additional","affiliation":[{"name":"Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isidora","family":"Arsenovic","sequence":"additional","affiliation":[{"name":"Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vojin","family":"Vukovic","sequence":"additional","affiliation":[{"name":"Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia"},{"name":"Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2025-9653","authenticated-orcid":false,"given":"Jelena","family":"Bila","sequence":"additional","affiliation":[{"name":"Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia"},{"name":"Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrija","family":"Bogdanovic","sequence":"additional","affiliation":[{"name":"Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia"},{"name":"Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2608-1342","authenticated-orcid":false,"given":"Darko","family":"Antic","sequence":"additional","affiliation":[{"name":"Clinic of Hematology, University Clinical Center of Serbia, 11000 Belgrade, Serbia"},{"name":"Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,3,21]]},"reference":[{"key":"ref_1","unstructured":"Swerdlow, S.H., Campo, E., Harris, N.L., Jaffe, E.S., Pileri, S.A., Stein, H., and Thiele, J. (2017). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC Press."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1038\/s41375-022-01613-1","article-title":"The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic\/Dendritic Neoplasms","volume":"36","author":"Khoury","year":"2022","journal-title":"Leukemia"},{"key":"ref_3","unstructured":"Justiz Vaillant, A.A., and Stang, C.M. (2023). StatPearls [Internet], StatPearls Publishing."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"6515","DOI":"10.1182\/blood-2011-04-348755","article-title":"Chronic myeloproliferative neoplasms and subsequent cancer risk: A Danish population-based cohort study","volume":"118","author":"Frederiksen","year":"2011","journal-title":"Blood"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2203","DOI":"10.1038\/s41375-018-0027-y","article-title":"Second malignancies in patients with myeloproliferative neoplasms: A population- based cohort study of 9379 patients","volume":"32","author":"Landtblom","year":"2018","journal-title":"Leukemia"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1486476","DOI":"10.1155\/2019\/1486476","article-title":"Coexistence of Myeloid and Lymphoid Neoplasms: A Single-Center Experience","volume":"2019","author":"Bouchla","year":"2019","journal-title":"Adv. Hematol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"e236","DOI":"10.1016\/j.leukres.2009.06.027","article-title":"JAK2 mutation in a patient with CLL with coexistent myeloproliferative neoplasm (MPN)","volume":"33","author":"Kodali","year":"2009","journal-title":"Leuk. Res."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1002\/hon.2701","article-title":"Myeloproliferative and lymphoproliferative disorders: State of the art","volume":"38","author":"Rumi","year":"2020","journal-title":"Hematol. Oncol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2199","DOI":"10.1182\/blood-2008-03-143602","article-title":"Increased risks of polycythemia vera, essential thrombocythemia, and myelofibrosis among 24,577 first-degree relatives of 11,039 patients with myeloproliferative neoplasms in Sweden","volume":"112","author":"Landgren","year":"2008","journal-title":"Blood"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"S20","DOI":"10.1186\/1755-1536-5-S1-S20","article-title":"Primary Myelofibrosis and the \u201cBad Seeds in Bad Soil\u201d concept","volume":"5","year":"2012","journal-title":"Fibrogenes. Tissue Repair"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1182\/blood-2017-10-810739","article-title":"Aggressive B-cell lymphomas in patients with myelofibrosis receiving JAK1\/2 inhibitor therapy","volume":"132","author":"Porpaczy","year":"2018","journal-title":"Blood"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"E185","DOI":"10.1002\/ajh.25489","article-title":"Ruxolitinib treatment and risk of B-cell lymphomas in myeloproliferative neoplasms","volume":"94","author":"Rumi","year":"2019","journal-title":"Am. J. Hematol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"701604","DOI":"10.3389\/fonc.2021.701604","article-title":"Co-occurrence of myeloid and lymphoid neoplasms: Clinical characterization and impact on outcome. A single-center cohort study","volume":"11","author":"Bucelli","year":"2021","journal-title":"Front. Oncol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1996","DOI":"10.1038\/s41375-019-0487-8","article-title":"Second cancer in Philadelphia negative myeloproliferative neoplasms (MPN-K). A nested case-control study","volume":"33","author":"Barbui","year":"2019","journal-title":"Leukemia"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"3196","DOI":"10.1182\/bloodadvances.2019000646","article-title":"Second primary malignancies in ruxolitinib-treated myelofibrosis: Real-world evidence from 219 consecutive patients","volume":"3","author":"Maffioli","year":"2019","journal-title":"Blood Adv."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1002\/ajh.25049","article-title":"Lymphoproliferative disorders in patients with chronic myeloproliferative neoplasms: A systematic review","volume":"93","author":"Marchetti","year":"2018","journal-title":"Am. J. Hematol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2068","DOI":"10.1158\/1055-9965.EPI-09-0353","article-title":"Increased risk of lymphoid neoplasms in patients with Philadelphia chromosome-negative myeloproliferative neoplasms","volume":"18","author":"Vannucchi","year":"2009","journal-title":"Cancer Epidemiol. Biomark. Prev."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"2432","DOI":"10.3324\/haematol.2019.225839","article-title":"Myeloproliferative and lymphoproliferative malignancies occurring in the same patient: A nationwide discovery cohort","volume":"105","author":"Holst","year":"2020","journal-title":"Haematologica"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1111\/bjh.14736","article-title":"Clinicopathological analysis of primary splenic diffuse large B-cell lymphoma","volume":"178","author":"Shimono","year":"2017","journal-title":"Br. J. Haematol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"556","DOI":"10.7150\/ijms.27610","article-title":"Clinical Significance of BCL2, C-MYC and BCL6 Genetic Abnormalities, Epstein-Barr Virus Infection, CD5 Protein Expression, Germinal Center B Cell\/Non-Germinal Center B-Cell Subtypes, Co-expression of MYC\/BCL2 Proteins and Co-expression of MYC\/BCL2\/BCL6 Proteins in Diffuse Large B-Cell Lymphoma: A Clinical and Pathological Correlation Study of 120 Patients","volume":"16","author":"Ting","year":"2019","journal-title":"Int. J. Med. Sci."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1007","DOI":"10.1002\/ajh.22171","article-title":"The coexistence of chronic lymphocytic leukemia and myeloproliferative neoplasms: A retrospective multicentric GIMEMA experience","volume":"86","author":"Laurenti","year":"2011","journal-title":"Am. J. Hematol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e20","DOI":"10.1038\/bcj.2011.18","article-title":"Coexistence of primary myelofibrosis and chronic lymphocytic leukaemia: Treatment of two different diseases with one agent","volume":"1","author":"Burgstaller","year":"2011","journal-title":"Blood Cancer J."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"4170","DOI":"10.1182\/blood-2019-127982","article-title":"Frequency of Thrombosis Is Higher in MPN Patients Who Develop Second Cancer Than in Controls","volume":"134","author":"Ghirardi","year":"2019","journal-title":"Blood"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"e000172","DOI":"10.1136\/esmoopen-2017-000172","article-title":"Multiple primary tumours: Challenges and approaches, a review","volume":"2","author":"Vogt","year":"2017","journal-title":"ESMO Open"},{"key":"ref_25","first-page":"629384","article-title":"Multiple Primary Tumors Over a Lifetime","volume":"33","author":"Copur","year":"2019","journal-title":"Oncology"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"854","DOI":"10.1016\/j.leukres.2008.09.002","article-title":"Zap-70 positive chronic lymphocytic leukemia co-existing with Jak 2 V671F positive essential thrombocythemia: A common defective stem cell?","volume":"33","author":"Tabaczewski","year":"2009","journal-title":"Leuk. Res."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/6\/1816\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:17:20Z","timestamp":1760105840000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/6\/1816"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,21]]},"references-count":26,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2024,3]]}},"alternative-id":["jcm13061816"],"URL":"https:\/\/doi.org\/10.3390\/jcm13061816","relation":{},"ISSN":["2077-0383"],"issn-type":[{"type":"electronic","value":"2077-0383"}],"subject":[],"published":{"date-parts":[[2024,3,21]]}}}