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Journal articles on the topic 'Myeloproliferative syndrome'

Author: Grafiati

Published: 4 June 2021

Last updated: 17 February 2022

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1

Binder and Fehr. "Myeloproliferative Syndrome." Therapeutische Umschau 61, no. 2 (February 1, 2004): 131–42. http://dx.doi.org/10.1024/0040-5930.61.2.131.

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Myeloproliferative Syndrome sind hämatopoietische Stammzellerkrankungen, die zur autonomen Proliferation einer oder mehrer Blutzellreihen führen. Sie werden wegen gemeinsamer klinischer und hämatologischer Merkmale, ihrer klonalen Hämatopoiese und der genetischen Instabilität mit unterschiedlicher Transformationstendenz in eine akute Leukämie als Gruppe verwandter hämatopoietischer Neoplasien zusammengefasst. In der vorliegenden Übersicht werden relevante Aspekte der klinischen Präsentation und Prognose, sowie aktuelle diagnostische und therapeutische Maßnahmen der Polycythaemia vera, Essentiellen Thrombozythämie und Chronisch Idiopathischen Myelofibrose diskutiert.

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2

Morgan, R., F. Hecht, ML Cleary, J. Sklar, and MP Link. "Leukemia with Down's syndrome: translocation between chromosomes 1 and 19 in acute myelomonocytic leukemia following transient congenital myeloproliferative syndrome." Blood 66, no. 6 (December 1, 1985): 1466–68. http://dx.doi.org/10.1182/blood.v66.6.1466.1466.

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Abstract A girl with Down's syndrome was born with a myeloproliferative disorder. The child had spontaneous regression of the myeloproliferation, with acute leukemia developing at a later date. Morphologic, cytochemical, immunologic, and immunoglobulin gene configuration studies all supported the diagnosis of acute nonlymphocytic leukemia. High-resolution chromosome studies revealed that the leukemic cells consistently contained a translocation between chromosomes 1 and 19: der(19)t(1;19)(q25;p13). Spontaneous regression of the transient myeloproliferative syndrome of the newborn with Down's syndrome may not always be permanent, and the transient myeloproliferative syndrome may sometimes represent an early sign of acute nonlymphocytic leukemia.

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3

Morgan, R., F. Hecht, ML Cleary, J. Sklar, and MP Link. "Leukemia with Down's syndrome: translocation between chromosomes 1 and 19 in acute myelomonocytic leukemia following transient congenital myeloproliferative syndrome." Blood 66, no. 6 (December 1, 1985): 1466–68. http://dx.doi.org/10.1182/blood.v66.6.1466.bloodjournal6661466.

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A girl with Down's syndrome was born with a myeloproliferative disorder. The child had spontaneous regression of the myeloproliferation, with acute leukemia developing at a later date. Morphologic, cytochemical, immunologic, and immunoglobulin gene configuration studies all supported the diagnosis of acute nonlymphocytic leukemia. High-resolution chromosome studies revealed that the leukemic cells consistently contained a translocation between chromosomes 1 and 19: der(19)t(1;19)(q25;p13). Spontaneous regression of the transient myeloproliferative syndrome of the newborn with Down's syndrome may not always be permanent, and the transient myeloproliferative syndrome may sometimes represent an early sign of acute nonlymphocytic leukemia.

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4

Ofstad, J. "The Myeloproliferative Syndrome." Acta Medica Scandinavica 167, no. 1 (April 24, 2009): 29–36. http://dx.doi.org/10.1111/j.0954-6820.1960.tb03512.x.

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5

Pierce, Rosalie G., and Maj Michael Oswald. "FAMILIAL MYELOPROLIFERATIVE SYNDROME." Southern Medical Journal 92, Supplement (November 1999): S56. http://dx.doi.org/10.1097/00007611-199911001-00122.

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6

Pérez-Encinas, M., J. L. Bello, S. Pérez-Crespo, R. De Miguel, and S. Tome. "Familial myeloproliferative syndrome." American Journal of Hematology 46, no. 3 (July 1994): 225–29. http://dx.doi.org/10.1002/ajh.2830460312.

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7

Sarah, OJohn-Olabode, AOyekunle Anthony, AAdeyemo Titilope, and SAkanmu Alani. "The 8p12 myeloproliferative syndrome." Nigerian Medical Journal 55, no. 2 (2014): 176. http://dx.doi.org/10.4103/0300-1652.129669.

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8

Schofield, Jill R., and William A. Robinson. "A new myeloproliferative syndrome." American Journal of Hematology 48, no. 3 (March 1995): 186–91. http://dx.doi.org/10.1002/ajh.2830480309.

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9

Mulhim, Ibrahim AI. "Down’s syndrome with transient myeloproliferative syndrome." Indian Journal of Pediatrics 57, no. 2 (March 1990): 253–55. http://dx.doi.org/10.1007/bf02722097.

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10

Patnaik, Mrinal M., and Terra L. Lasho. "Genomics of myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes." Hematology 2020, no. 1 (December 4, 2020): 450–59. http://dx.doi.org/10.1182/hematology.2020000130.

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Abstract Myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) overlap syndromes are uniquely classified neoplasms occurring in both children and adults. This category consists of 5 neoplastic subtypes: chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), BCR-ABL1–negative atypical chronic myeloid leukemia (aCML), MDS/MPN-ring sideroblasts and thrombocytosis (MDS/MPN-RS-T), and MDS/MPN-unclassifiable (U). Cytogenetic abnormalities and somatic copy number variations are uncommon; however, >90% patients harbor gene mutations. Although no single gene mutation is specific to a disease subtype, certain mutational signatures in the context of appropriate clinical and morphological features can be used to establish a diagnosis. In CMML, mutated coexpression of TET2 and SRSF2 results in clonal hematopoiesis skewed toward monocytosis, and the ensuing acquisition of driver mutations including ASXL1, NRAS, and CBL results in overt disease. MDS/MPN-RS-T demonstrates features of SF3B1-mutant MDS with ring sideroblasts (MDS-RS), with the development of thrombocytosis secondary to the acquisition of signaling mutations, most commonly JAK2V617F. JMML, the only pediatric entity, is a bona fide RASopathy, with germline and somatic mutations occurring in the oncogenic RAS pathway giving rise to disease. BCR-ABL1–negative aCML is characterized by dysplastic neutrophilia and is enriched in SETBP1 and ETNK1 mutations, whereas MDS/MPN-U is the least defined and lacks a characteristic mutational signature. Molecular profiling also provides prognostic information, with truncating ASXL1 mutations being universally detrimental and germline CBL mutations in JMML showing spontaneous regression. Sequencing information in certain cases can help identify potential targeted therapies (IDH1, IDH2, and splicing mutations) and should be a mainstay in the diagnosis and management of these neoplasms.

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11

Borregón, P., J. Menárguez, N. Navarro, and M. Campos. "Transient Myeloproliferative Syndrome Associated With Down Syndrome." Actas Dermo-Sifiliográficas (English Edition) 104, no. 1 (January 2013): 82–83. http://dx.doi.org/10.1016/j.adengl.2012.11.011.

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12

Bastida, Pilar, Sixto García-Miñaúr, Begoña Ezquieta, José Luis Dapena, and José Sanchez de Toledo. "Myeloproliferative Disorder in Noonan Syndrome." Journal of Pediatric Hematology/Oncology 33, no. 1 (January 2011): e43-e45. http://dx.doi.org/10.1097/mph.0b013e3181e7571e.

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13

Jackson, Courtney C., L. Jeffrey Medeiros, and Roberto N. Miranda. "8p11 myeloproliferative syndrome: a review." Human Pathology 41, no. 4 (April 2010): 461–76. http://dx.doi.org/10.1016/j.humpath.2009.11.003.

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14

A. Clara, Joseph, Joseph A. Clara, David A. Sallman, Eric Padron, David A. Sallman, and Eric Padron. "Clinical management of myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes." Cancer Biology & Medicine 13, no. 3 (2016): 360–72. http://dx.doi.org/10.20892/j.issn.2095-3941.2016.0043.

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15

McCullough, Kristen B., and Mrinal M. Patnaik. "Myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes – Advances in treatment." Best Practice & Research Clinical Haematology 33, no. 2 (June 2020): 101130. http://dx.doi.org/10.1016/j.beha.2019.101130.

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16

Adams, Bruce D., Russell Baker, J. Abraham Lopez, and Susan Spencer. "Myeloproliferative Disorders and the Hyperviscosity Syndrome." Hematology/Oncology Clinics of North America 24, no. 3 (June 2010): 585–602. http://dx.doi.org/10.1016/j.hoc.2010.03.004.

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17

KUDO, Masatoshi. "Budd-Chiari Syndrome and Myeloproliferative Disorder." Internal Medicine 35, no. 11 (1996): 837–38. http://dx.doi.org/10.2169/internalmedicine.35.837.

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18

Roufosse, Florence, Michel Goldman, and Elie Cogan. "Hypereosinophilic Syndrome: Lymphoproliferative and Myeloproliferative Variants." Seminars in Respiratory and Critical Care Medicine 27, no. 2 (April 2006): 158–70. http://dx.doi.org/10.1055/s-2006-939519.

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19

Radaelli, F., R. Calori, C. Ripamonti, and P. Faccini. "Treatment of myeloproliferative syndrome with hydroxyurea." Annals of Hematology 73, no. 4 (October 2, 1996): 205. http://dx.doi.org/10.1007/s002770050230.

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20

Adams, Bruce D., Russell Baker, J. Abraham Lopez, and Susan Spencer. "Myeloproliferative Disorders and the Hyperviscosity Syndrome." Emergency Medicine Clinics of North America 27, no. 3 (August 2009): 459–76. http://dx.doi.org/10.1016/j.emc.2009.04.001.

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21

Gilbert-Barness, Enid, and Lewis A. Barness. "Isovaleric Acidemia with Promyelocytic Myeloproliferative Syndrome." Pediatric and Developmental Pathology 2, no. 3 (May 1999): 286–91. http://dx.doi.org/10.1007/s100249900125.

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Isovaleric acidemia, an autosomal recessive disorder, is due to isovaleryl-coenzyme A dehydrogenase deficiency and is one of the branched-chain aminoacidopathies. Isovaleric acidemia may present in the neonatal period with an acute episode of severe metabolic acidosis, ketosis, and vomiting and may lead to coma and death in the first 2 months of life. This report concerns an infant who presented at 10 days of age because of lethargy, poor feeding, hypothermia, cholestasis, and thrombocytopenia, leukopenia, and profound pancytopenia. Death occurred at 19 days of age. Autopsy showed mild fatty change in the liver and extramedullary hematopoiesis, generalized Escherichia coli sepsis, and myelodysplasia of the bone marrow with arrest of the myeloid series at the promyelocytic stage. The appearance resembled promyelocytic leukemia, but the diagnostic 15:17 translocation was not present. The maturation arrest in granulopoiesis in isovaleric acidemia appears to be most likely due to a direct metabolic effect on granulocyte precursor cells.

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22

Sayar, Zara, Susanna Nallamilli, Maria Efthymiou, Jonathan Lambert, and Hannah Cohen. "Coexistent antiphospholipid syndrome and myeloproliferative neoplasm." Lupus 30, no. 9 (July 1, 2021): 1502–8. http://dx.doi.org/10.1177/09612033211021154.

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Antiphospholipid syndrome (APS) and myeloproliferative neoplasms (MPN) are associated with an increased risk of thrombosis. The optimal management of patients with coexistent APS and MPN has not been defined. A single centre and systematic literature review of patients with coexistent APS and MPN was performed. Cases were divided into two groups based on whether they met international consensus criteria for APS. Of the 12 studies identified, eight were excluded (leaving five of a total 54 patients), as although antiphospholipid antibodies (aPL) were documented, the diagnosis of APS was not conclusively demonstrated. Another ten patients with definite APS were identified at our centre. Fifteen patients (ten females, five males) were therefore included in this analysis (eleven definite APS and four highly likely), median age 44 (range: 13–71) years. Nine had polycythaemia vera and six, essential thrombocythaemia. Thirteen of the 15 patients (86.7%) had thrombotic APS (seven with initial venous events and six arterial) and two (13.3%) had obstetric APS. Nine patients were single-positive, and six double-positive for aPL. None were triple aPL-positive. Four patients at our centre had recurrent thrombotic/obstetric events, including while on anticoagulation/antiplatelet treatment.

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23

Klion, Amy D. "Eosinophilic Myeloproliferative Disorders." Hematology 2011, no. 1 (December 10, 2011): 257–63. http://dx.doi.org/10.1182/asheducation-2011.1.257.

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Abstract Despite recent attempts to define and classify patients with marked eosinophilia and features consistent with myeloproliferative disease, areas of controversy remain. These are particularly apparent in situations in which multiple lineages are involved in a clonal process and clinical manifestations are overlapping. Although the introduction of new molecular diagnostics and targeted therapies has begun to clarify the boundaries between some of these disorders, several questions remain with respect to the classification of patients with myeloproliferative hypereosinophilic syndrome (HES) of unknown etiology.

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24

Ng, Ashley P., Craig D. Hyland, Donald Metcalf, Catherine L. Carmichael, Stephen J. Loughran, Ladina Di Rago, Benjamin T. Kile, and Warren S. Alexander. "Trisomy of Erg is required for myeloproliferation in a mouse model of Down syndrome." Blood 115, no. 19 (May 13, 2010): 3966–69. http://dx.doi.org/10.1182/blood-2009-09-242107.

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Abstract Down syndrome is characterized by multiple phenotypic manifestations associated with trisomy of chromosome 21. The transient myeloproliferative disorder and acute megakaryocytic leukemia associated with Down syndrome are uniquely associated with mutations in the transcription factor GATA1; however, the identity of trisomic genes on chromosome 21 that predispose to these hematologic disorders remains unknown. Using a loss-of-function allele, we show that specific reduction to functional disomy of the Erg gene corrects the pathologic and hematologic features of myeloproliferation in the Ts(1716)65Dn mouse model of Down syndrome, including megakaryocytosis and progenitor cell expansion. Our data provide genetic evidence establishing the need for Erg trisomy for myeloproliferation in Ts(1716)65Dn mice and imply that increased ERG gene dosage may be a key consequence of trisomy 21 that can predispose to malignant hematologic disorders in Down syndrome.

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25

Kshirsagar, VinayakY, Minhajuddin Ahmed, SylviaM Colaco, and Manal Ahmed. "Transient myeloproliferative disorder in Down′s syndrome." Indian Journal of Pathology and Microbiology 55, no. 3 (2012): 426. http://dx.doi.org/10.4103/0377-4929.101773.

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26

Baud, P., A. Tobler, B. Lämmle, and L. Alberio. "Acquired von Willebrand syndrome in myeloproliferative disorder." Hämostaseologie 23, no. 03 (2003): 121–24. http://dx.doi.org/10.1055/s-0037-1619589.

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SummaryWe present a woman (age: 57 years) with an excessive bleeding episode under acetylsalicylic acid after bone marrow puncture due to an acquired von Willebrand syndrome (avWS) in the context of a myeloproliferative disorder. The laboratory features showed a high platelet concentration and a qualitative defect of von Willebrand factor (vWF) with a low normal vWF ristocetin cofactor activity, a normal vWF antigen and a decrease of the larger vWF multimers in plasma.The exact mechanism of avWS is still incompletely resolved. Myeloproliferative diseases are one of several underlying disorders that may cause avWS. The diagnosis of the underlying disease is important because its treatment may lead to an improvement of the vWF abnormality. For symptomatic treatment of bleeding, desmopressin, vWF concentrate infusion, intravenous immunoglobulin and/or fibrinolysis inhibitors can be tried.

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27

Hoo, Joe-Jie, Sharon O'Brien, and Iris Samuel. "Double supernumerary isochromosome 9p in myeloproliferative syndrome." Cancer Genetics and Cytogenetics 29, no. 2 (December 1987): 319–21. http://dx.doi.org/10.1016/0165-4608(87)90243-3.

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28

Zipursky, Alvin, Elizabeth Brown, Hilary Christensen, Robert Sutherland, and John Doyle. "Leukemia and/or myeloproliferative syndrome in neonates with Down Syndrome." Seminars in Perinatology 21, no. 1 (February 1997): 97–101. http://dx.doi.org/10.1016/s0146-0005(97)80025-0.

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29

Cazzola, Mario, Luca Malcovati, and Rosangela Invernizzi. "Myelodysplastic/Myeloproliferative Neoplasms." Hematology 2011, no. 1 (December 10, 2011): 264–72. http://dx.doi.org/10.1182/asheducation-2011.1.264.

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Abstract According to the World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues, myelodysplastic/myeloproliferative neoplasms are clonal myeloid neoplasms that have some clinical, laboratory, or morphologic findings that support a diagnosis of myelodysplastic syndrome, and other findings that are more consistent with myeloproliferative neoplasms. These disorders include chronic myelomonocytic leukemia, atypical chronic myeloid leukemia (BCR-ABL1 negative), juvenile myelomonocytic leukemia, and myelodysplastic/myeloproliferative neoplasms, unclassifiable. The best characterized of these latter unclassifiable conditions is the provisional entity defined as refractory anemia with ring sideroblasts associated with marked thrombocytosis. This article focuses on myelodysplastic/myeloproliferative neoplasms of adulthood, with particular emphasis on chronic myelomonocytic leukemia and refractory anemia with ring sideroblasts associated with marked thrombocytosis. Recent studies have partly clarified the molecular basis of these disorders, laying the groundwork for the development of molecular diagnostic and prognostic tools. It is hoped that these advances will soon translate into improved therapeutic approaches.

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30

Hemsing, Anette Lodvir, Bjørn Tore Gjertsen, Signe Spetalen, Lars Helgeland, and Håkon Reikvam. "Favorable outcome of a patient with an unclassifiable myelodysplastic syndrome/myeloproliferative neoplasm treated with allogeneic hematopoietic stem cell transplantation." SAGE Open Medical Case Reports 9 (January 2021): 2050313X2098841. http://dx.doi.org/10.1177/2050313x20988413.

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The entity myelodysplastic syndrome/myeloproliferative neoplasm overlap syndrome is characterized by the coexistence of both myeloproliferative and myelodysplastic features in the bone marrow. Risk assessment and treatment recommendations have not been standardized, and clinicians rely on updated patient studies and reviews to make decisions for treatment approaches. Histopathological features have traditionally been important, although in the last decade, several studies have reported mutational profiles of this rare disease. Here, we present a case, wherein the patient presented with leukocytosis and the diagnostic work-up revealed features of myelodysplastic syndrome/myeloproliferative neoplasm overlap syndrome. Mutational profiling revealed mutations in four genes associated with myeloid malignancies, namely, EZH2, CUX1, TET2, and BCOR. After initial therapy with hydroxyurea and interferon-α, the patient underwent allogeneic hematopoietic stem cell transplantation, with reduced intensity conditioning and a matched sibling donor. He had no signs of relapsed disease 2 years after the transplant. Based on the patient outcome, we summarize the diagnostic and therapeutic approaches for patients diagnosed with myelodysplastic syndrome/myeloproliferative neoplasm overlap syndrome, and review the current literature, emphasizing the role of genetic mutations and allogeneic hematopoietic stem cell transplantation. Larger and more detailed clinical studies are strongly needed to optimize and standardize diagnostic and therapeutic approaches for this disease.

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31

Score, Joannah, Claire Hidalgo-Curtis, Amy V. Jones, Nils Winkelmann, Alison Skinner, Daniel Ward, Katerina Zoi, et al. "Inactivation of polycomb repressive complex 2 components in myeloproliferative and myelodysplastic/myeloproliferative neoplasms." Blood 119, no. 5 (February 2, 2012): 1208–13. http://dx.doi.org/10.1182/blood-2011-07-367243.

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Abstract The polycomb repressive complex 2 (PRC2) is a highly conserved histone H3 lysine 27 methyltransferase that regulates the expression of developmental genes. Inactivating mutations of the catalytic component of PRC2, EZH2, are seen in myeloid disorders. We reasoned that the other 2 core PRC2 components, SUZ12 and EED, may also be mutational targets in these diseases, as well as associated factors such as JARID2. SUZ12 mutations were identified in 1 of 2 patients with myelodysplastic syndrome/myeloproliferative neoplasms with 17q acquired uniparental disomy and in 2 of 2 myelofibrosis cases with focal 17q11 deletions. All 3 were missense mutations affecting the highly conserved VEFS domain. Analysis of a further 146 myelodysplastic syndrome/myeloproliferative neoplasm patients revealed an additional VEFS domain mutant, yielding a total mutation frequency of 1.4% (2 of 148). We did not find mutations of JARID2 or EED in association with acquired uniparental disomy for chromosome 6p or 11q, respectively; however, screening unselected cases identified missense mutations in EED (1 of 148; 1%) and JARID2 (3 of 148; 2%). All 3 SUZ12 mutations tested and the EED mutation reduced PRC2 histone methyltransferase activity in vitro, demonstrating that PRC2 function may be compromised in myeloid disorders by mutation of distinct genes.

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32

Arceci, Robert J. "Down Syndrome, Transient Myeloproliferative Syndrome, and Leukemia: Bridging Development and Neoplasia." Journal of Pediatric Hematology/Oncology 24, no. 1 (January 2002): 1. http://dx.doi.org/10.1097/00043426-200201000-00001.

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33

Galván, Oriol Plans, Hipólito Pérez Moltó, Ariadna Fabià-Mayans, Blanca Xicoy, José Luis Mate, and Pilar Ricart Martí. "Hydroxyurea-Induced Pneumopathy in a Patient With Myeloproliferative Syndrome." Clinical Medicine Insights: Case Reports 11 (January 1, 2018): 117954761877068. http://dx.doi.org/10.1177/1179547618770688.

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Hydroxyurea (HU) is a drug frequently used in the treatment of chronic myeloproliferative neoplasms. The most common side effects of this drug are pancytopenia, digestive and skin disorders. Respiratory complications are rare and there are less than 20 cases described, only 5 of which underwent an anatomopathological study. We present the case of a patient with chronic myeloproliferative neoplasm who developed interstitial pneumonitis probably due to HU according to histological study.

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34

Chauveau, Aurélie, Jean-Christophe Ianotto, Valérie Ugo, and Eric Lippert. "Calreticulin mutations in myeloproliferative syndrome: the missing link?" Hématologie 20, no. 1 (January 2014): 15–19. http://dx.doi.org/10.1684/hma.2014.0894.

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35

Mutlu, Mehmet, Erol Erduman, and Yakup Aslan. "Case Report: Transient Myeloproliferative Disorder of Down Syndrome." Tuberculin Skin Test in Children 10, no. 1 (May 31, 2010): 44–46. http://dx.doi.org/10.5222/j.child.2010.044.

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36

Ha, Jung Sook, Won Mok Lee, Ji Hye Kim, Nam Hee Ryoo, Dong Suk Jeon, Jae Ryong Kim, Heung Sik Kim, and Byung Kyu Choi. "GATA1Mutation in Transient Myeloproliferative Disorder of Down Syndrome." Korean Journal of Hematology 43, no. 1 (2008): 43. http://dx.doi.org/10.5045/kjh.2008.43.1.43.

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37

Girard, P., C. Leonard, J. Quillard, P. Eydoux, P. Danel, Jp Dommergues, and G. Tchernia. "Myelofibrosis, myeloproliferative syndrome and monosomy C in children." European Paediatric Haematology and Oncology 2, no. 1 (January 1985): 13–21. http://dx.doi.org/10.3109/08880018509141198.

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38

Marques-Salles, Terezinha de Jesus, Eliane Maria Soares-Ventura, Nathalia Lopes de Oliveira, Mariluze Silva, Reijane Assis, Vera Lúcia Lins de Morais, Luize Otero, et al. "Myeloproliferative syndrome of monosomy 7: a brief report." Genetics and Molecular Biology 31, no. 1 (2008): 36–38. http://dx.doi.org/10.1590/s1415-47572008000100007.

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39

Suarez, J. M. Villa, C. Miralles Adell, A. Espuch Oliver, M. P. Jiménez Gámiz, and T. De Haro Muñoz. "Transient myeloproliferative disorder in newborns with Down’s syndrome." Clinica Chimica Acta 493 (June 2019): S248—S249. http://dx.doi.org/10.1016/j.cca.2019.03.513.

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40

Desplechin, Arnaud, Arsène Mekinian, Julien Rossignol, Guillaume Denis, Phillippe Gosset, Nathalie Cambier, and Christian Rose. "Isolated megakaryoblastic bone sarcoma revealing acute myeloproliferative syndrome." Joint Bone Spine 77, no. 3 (May 2010): 277–78. http://dx.doi.org/10.1016/j.jbspin.2010.03.005.

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41

Stein, Brady L., and Karlyn Martin. "From Budd-Chiari syndrome to acquired von Willebrand syndrome: thrombosis and bleeding complications in the myeloproliferative neoplasms." Blood 134, no. 22 (November 28, 2019): 1902–11. http://dx.doi.org/10.1182/blood.2019001318.

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42

Fernández-Fernández, Francisco José, Juan Carlos Álvarez – Fernández, Esperanza Romero – Picos, Juan Antonio Garrido, and Pascual Sesma. "Neutrophilic Dermatosis of the Dorsal Hands Associated with a “Myeloproliferative Neoplasm, Unclassifiable“ and a Simultaneous Cancer of Colon." Acta Medica (Hradec Kralove, Czech Republic) 53, no. 3 (2010): 153–56. http://dx.doi.org/10.14712/18059694.2016.75.

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Neutrophilic dermatosis of the dorsal hands is a variant of Sweet's syndrome. We herein describe an 83-year old woman with a neutrophilic dermatosis of the dorsal hands associated with a "myeloproliferative neoplasm, unclassifiable" and a simultaneous cancer of colon. To our knowledge, and after a search in PubMed, the association of Sweet's syndrome with a "myeloproliferative neoplasm, unclassifiable" and a simultaneous cancer of colon has not previously been reported.

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43

Patnaik, Mrinal M., and Terra Lasho. "Evidence-Based Minireview: Myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes: a focused review." Hematology 2020, no. 1 (December 4, 2020): 460–64. http://dx.doi.org/10.1182/hematology.2020000163.

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Abstract Myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) overlap syndromes are unique myeloid neoplasms, with overlapping features of MDS and MPN. They consist of four adult onset entities including chronic myelomonocytic leukemia (CMML), MDS/MPN-ring sideroblasts-thrombocytosis (MDS/MPN-RS-T), BCR-ABL1 negative atypical chronic myeloid leukemia (aCML) and MDS/MPN-unclassifiable (MDS/MPN-U); with juvenile myelomonocytic leukemia (JMML) being the only pediatric onset entity. Among these overlap neoplasms, CMML is the most frequent and is hallmarked by the presence of sustained peripheral blood monocytosis with recurrent mutations involving TET2 (60%), SRSF2 (50%) and ASXL1 (40%); with RAS pathway mutations and JAK2V617F being relatively enriched in proliferative CMML subtypes (WBC ≥13 × 109/L). CMML usually presents in the 7th decade of life, with a male preponderance and is associated with a median overall survival of <36 months. Adverse prognosticators in CMML include increasing age, high WBC, presence of circulating immature myeloid cells, anemia, thrombocytopenia and truncating ASXL1 mutations. While allogeneic stem cell transplantation remains the only curative option, given the late onset of this neoplasm and high frequency of comorbidities, most patients remain ineligible. Hypomethylating agents such as azacitidine, decitabine and oral decitabine/cedazuridine have been US FDA approved for the management of CMML, with overall response rates of 40-50% and complete remission rates of <20%. While these agents epigenetically restore hematopoiesis in a subset of responding patients, they do not impact mutational allele burdens and eventual disease progression to AML remains inevitable. Newer treatment modalities exploiting epigenetic, signaling and splicing abnormalities commonly seen in CMML are much needed.

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Luna-Fineman, Sandra, Kevin M. Shannon, Susan K. Atwater, Jeffrey Davis, Margaret Masterson, Jorge Ortega, Jean Sanders, Peter Steinherz, Vivian Weinberg, and Beverly J. Lange. "Myelodysplastic and Myeloproliferative Disorders of Childhood: A Study of 167 Patients." Blood 93, no. 2 (January 15, 1999): 459–66. http://dx.doi.org/10.1182/blood.v93.2.459.

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Abstract Myelodysplastic syndromes (MDS) and myeloproliferative syndromes (MPS) of childhood are a heterogeneous group of clonal disorders of hematopoiesis with overlapping clinical features and inconsistent nomenclature. Although a number of genetic conditions have been associated with MDS and MPS, the overall contribution of inherited predispositions is uncertain. We report a retrospective study examining clinical features, genetic associations, and outcomes in 167 children with MDS and MPS. Of these patients, 48 had an associated constitutional disorder. One hundred one patients had adult-type myelodysplastic syndrome (A-MDS), 60 had juvenile myelomonocytic leukemia (JMML), and 6 infants with Down syndrome had a transient myeloproliferative syndrome (TMS). JMML was characterized by young age at onset and prominent hepatosplenomegaly, whereas patients with A-MDS were older and had little or no organomegaly. The most common cytogenetic abnormalities were monosomy 7 or del(7q) (53 cases); this was common both in patients with JMML and those with A-MDS. Leukemic transformation was observed in 32% of patients, usually within 2 years of diagnosis. Survival was 25% at 16 years. Favorable prognostic features at diagnosis included age less than 2 years and a hemoglobin F level of less than 10%. Older patients tended to present with an adult-type MDS that is accommodated within the French-American-British system. In contrast, infants and young children typically developed unique disorders with overlapping features of MDS and MPS. Although the type and intensity of therapy varied markedly in this study, the overall outcome was poor except in patients with TMS.

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45

Luna-Fineman, Sandra, Kevin M. Shannon, Susan K. Atwater, Jeffrey Davis, Margaret Masterson, Jorge Ortega, Jean Sanders, Peter Steinherz, Vivian Weinberg, and Beverly J. Lange. "Myelodysplastic and Myeloproliferative Disorders of Childhood: A Study of 167 Patients." Blood 93, no. 2 (January 15, 1999): 459–66. http://dx.doi.org/10.1182/blood.v93.2.459.402k19_459_466.

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Myelodysplastic syndromes (MDS) and myeloproliferative syndromes (MPS) of childhood are a heterogeneous group of clonal disorders of hematopoiesis with overlapping clinical features and inconsistent nomenclature. Although a number of genetic conditions have been associated with MDS and MPS, the overall contribution of inherited predispositions is uncertain. We report a retrospective study examining clinical features, genetic associations, and outcomes in 167 children with MDS and MPS. Of these patients, 48 had an associated constitutional disorder. One hundred one patients had adult-type myelodysplastic syndrome (A-MDS), 60 had juvenile myelomonocytic leukemia (JMML), and 6 infants with Down syndrome had a transient myeloproliferative syndrome (TMS). JMML was characterized by young age at onset and prominent hepatosplenomegaly, whereas patients with A-MDS were older and had little or no organomegaly. The most common cytogenetic abnormalities were monosomy 7 or del(7q) (53 cases); this was common both in patients with JMML and those with A-MDS. Leukemic transformation was observed in 32% of patients, usually within 2 years of diagnosis. Survival was 25% at 16 years. Favorable prognostic features at diagnosis included age less than 2 years and a hemoglobin F level of less than 10%. Older patients tended to present with an adult-type MDS that is accommodated within the French-American-British system. In contrast, infants and young children typically developed unique disorders with overlapping features of MDS and MPS. Although the type and intensity of therapy varied markedly in this study, the overall outcome was poor except in patients with TMS.

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46

Koschmieder, Steffen, and Tim H. Brümmendorf. "Myeloproliferative Neoplasien (MPN)." Der Klinikarzt 47, no. 09 (September 2018): 398–402. http://dx.doi.org/10.1055/a-0686-5746.

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ZusammenfassungMyeloproliferative Neoplasien (MPN) sind klonale Stammzellerkrankungen, die typischerweise mit der Vemehrung einer oder mehrerer Blutzellreihen, einer Knochenmarkhyperplasie und einer (Hepato)-Splenomegalie als Zeichen der extramedullären Hämatopoese einhergehen. Gefürchtete Komplikationen der MPN sind Thromboembolien und schwergradige Blutungen, eine Knochenmarkverfaserung (Myelofibrose) mit konsekutiver hämatopoetischer Insuffizienz sowie der Übergang in eine akute Leukämie. Man unterscheidet die 4 klassischen MPN (Chronische Myeloische Leukämie [CML], Polycythämia vera [PV], Essentielle Thrombozythämie [ET], Primäre Myelofibrose [PMF]) von den nicht-klassischen MPN (Chronische Eosinophilen-Leukämie [CEL], Chronische Neutrophilen-Leukämie [CNL] und MPN-unklassifizierbar [MPN-U]). Sowohl der Verlauf der Erkrankungen als auch deren Symptomatik sind sehr heterogen, und eine exakte Diagnosestellung ist wichtig für die adäquate Einschätzung der Prognose und für die Beratung und Behandlung der betroffenen Patienten. Zur Diagnostik gehören neben Anamnese und ausführlicher körperlicher Untersuchung die Erhebung von Blutbild und Differenzialblutbild, eine Knochenmarkuntersuchung (Zytologie, Histologie, Chromosomenanalyse) sowie die molekulargenetische Untersuchung des peripheren Blutes (je nach MPN-Subtyp Bcr-Abl- oder Fip1L1-PDGFRA-Transkripte, JAK2617F-, CALR- und MPL-Mutationen, CSF3R- und SETBP1-Mutationen, etc). Differenzialdiagnostisch müssen u. a. die systemische Mastoztyose und die MDS/MPN-Overlap-Syndrome von den MPN abgegrenzt werden. Die Therapie ist abhängig von der Prognose der Erkrankung (Abschätzung durch verschiedene Prognose-Scores) und reicht von „Watchful waiting“ über Aderlässe, Thrombozytenaggregationshemmung und Antikoagulation bis hin zur medikamentösen Zytoreduktion, Entzündungshemmung und allogenen Stammzelltransplantation. Die Diagnose und Therapieweichenstellung gehört in die Hände des erfahrenen Spezialisten, und vor Therapiebeginn sollten dem Patienten die Teilnahme an Bioregistern und klinischen Studien erläutert werden. Informationen hierzu sind über die „German Study Group for MPN“ (GSG-MPN) erhältlich (https://www.cto-im3.de/gsgmpn/).

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Klion, Amy D., Pierre Noel, Cem Akin, Melissa A. Law, D. Gary Gilliland, Jan Cools, Dean D. Metcalfe, and Thomas B. Nutman. "Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness." Blood 101, no. 12 (June 15, 2003): 4660–66. http://dx.doi.org/10.1182/blood-2003-01-0006.

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Abstract Since serum tryptase levels are elevated in some patients with myeloproliferative disorders, we examined their utility in identifying a subset of patients with hypereosinophilic syndrome (HES) and an underlying myeloproliferative disorder. Elevated serum tryptase levels (> 11.5 ng/mL) were present in 9 of 15 patients with HES and were associated with other markers of myeloproliferation, including elevated B12 levels and splenomegaly. Although bone marrow biopsies in these patients showed increased numbers of CD25+ mast cells and atypical spindle-shaped mast cells, patients with HES and elevated serum tryptase could be distinguished from patients with systemic mastocytosis and eosinophilia by their clinical manifestations, the absence of mast cell aggregates, the lack of a somatic KIT mutation, and the presence of the recently described fusion of the Fip1–like 1 (FIP1L1) gene to the platelet-derived growth factor receptor α gene (PDGFRA). Patients with HES and elevated serum tryptase were more likely to develop fibroproliferative end organ damage, and 3 of 9 died within 5 years of diagnosis in contrast to 0 of 6 patients with normal serum tryptase levels. All 6 patients with HES and elevated tryptase treated with imatinib demonstrated a clinical and hematologic response. In summary, elevated serum tryptase appears to be a sensitive marker of a myeloproliferative variant of HES that is characterized by tissue fibrosis, poor prognosis, and imatinib responsiveness.

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48

Ermolova, T. V., B. N. Kotiv, V. V. Oleinik, and O. O. Yukhnova. "Budd-Chiari syndrome at the young woman with thrombophilia and myeloproliferative disease." Experimental and Clinical Gastroenterology 1, no. 10 (March 2, 2020): 101–4. http://dx.doi.org/10.31146/1682-8658-ecg-170-10-101-104.

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In the clinic of various diseases (especially hematological) Budd-Chiari syndrome occupies a signifi cant place. The occurrence of blood fl ow disorders in the portal vein system can be a manifestation and the only symptom that manifests itself in a number of diseases. Given the rapid development of portal hypertension, this syndrome requires rapid diagnosis and timely initiated therapeutic measures, as well as comprehensive research to identify the etiological cause of Budd-Chiari syndrome.Objectives: In this paper we demonstrated a clinical case of a young patient with Budd-Chiari syndrome, an unusual course and a combination of etiological factors.Materials and methods. Data from the medical history of the patient who was on treatment at the surgical department of the City Multi-profile Hospital № 2, Military-medical Academy St. Petersburg were used as materials.Result. The patient developed severe ascites 3 months after delivery, for which she was unsuccessfully examined by gynecologists to exclude ovarian tumors. Then, in the course of laboratory and instrumental studies, a final diagnosis was made of Budd-Chiari syndrom (thrombosis of the intrahepatic part of the inferior vena cava in the phase of partial recanalization against the background of chronic myeloproliferative disease and genetically determined thrombophilia.Conclusion: As this case shows, in the development of non-cirrhotic portal hypertension, it is necessary to exclude hepatic thrombosis, in the detection of which it is recommended to conduct a comprehensive examination to exclude all causes of this thrombosis (genetic analysis for thrombophylia, antiphospholipid syndrome, Jak-2 v617f for exclude polycythemia and myelofi brosis, examination for cancer pathology). Manifestation and the only symptom of thrombophilia and idiopathic myelofi brosis may be acute Budd-Chiari syndrome, which requires complex medical and surgical treatment, further observation and especially treatment of causal diseases.

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Andrikovics, Hajnalka, Anikó Szilvási, Nóra Meggyesi, Viktória Király, Gabriella Halm, Sándor Lueff, Sarolta Nahajevszky, et al. "Role of the activating mutation Val617Phe of Janus kinase 2 gene in myeloproliferative diseases and significance of its detection." Orvosi Hetilap 148, no. 5 (February 2007): 203–10. http://dx.doi.org/10.1556/oh.2007.27860.

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The Val617Phe point mutation of Janus kinase 2 gene is believed to participate in the pathogenesis of myeloproliferative syndrome characterised by the clonal alteration of hematopoetic stem cells. According to current results, the frequency of Val617Phe activating mutation is around 80% in polycythaemia vera, 35% in essential thrombocythaemia, and 50% in chronic idiopathic myelofibrosis. The diagnoses of polycythaemia vera, essential thrombocythaemia and idiopathic myelofibrosis were so far based on the exclusion of secondary factors as well as bone marrow biopsy histology. The goal of the present work was to establish simple molecular genetic techniques for the routine testing of Janus kinase 2 gene Val617Phe mutation, and to compare the clinical phenotypes of Val617Phe mutation positive and negative myeloproliferative syndromes. We employed the allele specific polymerase chain technique for detection of Val617Phe mutation in 252 patients with myeloproliferative syndrome. We measured Val617Phe frequency as 85,4% (117/137) in polycytaemia vera, 56,6% (56/99) in essential thrombocythaemia, and 87,5% (14/16) in idiopathic myelofibrosis. We found significantly elevated hemoglobin levels and white blood cell counts (measured at the time of diagnosis) in Val617Phe-positive polycythaemia vera and essential thrombocythaemia patient groups compared to Val617Phe-negative patients. However, the frequencies of splenomegaly and other complications (thrombosis, bleeding, transformation to acute leukemia) were not significantly different between the mutation-positive and negative groups. In conclusion, the non-invasive mutation analysis of the Janus kinase 2 Val617Phe is suitable for routine laboratory application and helps the differential diagnosis of myeloproliferative syndrome. Althought the exact role of Val617Phe mutation testing has not yet been identified on the basis of a broad professional consensus, the testing is suggested in cases of erythrocytoses and thrombocytoses of unknown origin.

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Chen, Jing, Daniel J. DeAngelo, Jeffery L. Kutok, Ifor R. Williams, Benjamin H. Lee, Martha Wadleigh, Nicole Duclos, et al. "PKC412 Inhibits the ZNF198-FGFR1 Fusion Tyrosine Kinase and Is Efficacious in Treatment of t(8;13)(p11;q12) Associated Stem Cell Myeloproliferative Disease." Blood 104, no. 11 (November 16, 2004): 2549. http://dx.doi.org/10.1182/blood.v104.11.2549.2549.

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Abstract Human stem cell leukemia-lymphoma syndrome usually presents as a myeloproliferative disease (MPD) that evolves to acute myeloid leukemia and/or lymphoma. The syndrome associated with t(8;13)(p11;q12) results in expression of the ZNF198-FGFR1 fusion tyrosine kinase. Current empirically-derived cytotoxic chemotherapy is inadequate treatment of this disease. We hypothesized that small molecule inhibitors of the ZNF198-FGFR1 fusion would have therapeutic efficacy. We characterized the transforming activity of ZNF198-FGFR1 in hematopoietic cells in vitro and in vivo. Expression of ZNF198-FGFR1 in primary murine hematopoietic cells caused a myeloproliferative syndrome in mice that recapitulated the human MPD phenotype. Transformation in these assays, and activation of the downstream effector molecules PLCγ, STAT5 and PI3K/AKT, required the proline-rich, but not the zinc-finger domains of ZNF198. A small molecule tyrosine kinase inhibitor, PKC412 (N-benzoyl-staurosporine) effectively inhibited ZNF198-FGFR1 tyrosine kinase activity and activation of downstream effector pathways, and inhibited proliferation of ZNF198-FGFR1 transformed Ba/F3 cells. Furthermore, treatment with PKC412 resulted in statistically significant prolongation of survival in the murine model of ZNF198-FGFR1 induced myeloproliferative disease. Based in part on these data, PKC412 was administered to a patient with t(8;13)(p11;q12) and was efficacious in treatment of progressive myeloproliferative disease with organomegaly. Therefore, PKC412 may be a useful therapy for treatment of human stem cell leukemia-lymphoma syndrome.

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