nach oben

Erschienen in:

01.01.2014 | Original Article

Prognostic impact of blast cell counts in dysplastic bone marrow disorders (MDS and CMML I) with concomitant fibrosis

verfasst von: Sigrid Machherndl-Spandl, W. Sega, H. Bösmüller, U. Germing, Ch. Gruber, K. Nachtkamp, P. Reinecke, W. R. Sperr, F. Wimazal, L. Müllauer, K. Sotlar, H. P. Horny, H. Tüchler, P. Valent, O. Krieger

Erschienen in: Annals of Hematology | Ausgabe 1/2014

Einloggen, um Zugang zu erhalten

Abstract

In a retrospective study, 43 patients with dysplastic neoplasms of the bone marrow (myelodysplastic syndromes and myelodysplastic/myeloproliferative-overlap neoplasms) associated with marked (grades 2–3) fibrosis were examined. Histopathologic and morphologic findings as well as cytogenetic and molecular results were correlated with clinical endpoints. Multilineage dysplasia (34 of 43 patients, 79 %) and hypercellular bone marrow (64 %) were found in most patients. In ten of 35 patients, poor risk karyotypes according to the International Prognostic Scoring System (IPSS) were recorded. The JAK2 V617F mutation was detected in four of 30 patients (13 %), and the KIT D816V mutation was found in two of 30 patients (6 %). Patients were mainly treated with palliative drugs and best supportive care. After an observation time of 1–41 (median 21) months, ten of 43 patients (23 %) had developed a secondary acute leukemia. The median survival of all 43 patients was 21.4 months (range 1.8–88.2 months). Of all prognostic parameters examined, the blast cell count at diagnosis was found to be a most reliable and most predictive marker concerning survival and leukemia progression. This confirms previous studies in dysplastic bone marrow neoplasms without fibrosis.

Bennett JM, Catovsky D, Daniel MT et al (1982) Proposals for the classification of the myelodysplastic syndromes. Br J Haematol 51:189–199PubMed

Brunning RD, Bennet JM, Flandrin G et al. (2001) Myelodysplastic syndromes. In: Jaffe ES, Harris NL, Stein H, Vardiman JW (eds) World Health Organization classification of tumours. Pathology and genetics of tumours of haematopoietic and lymphoid tissues. IARC, Lyon, pp 61–74

Pagliuca A et al (1989) Myelofibrosis in primary myelodysplastic syndromes: a clinicomorphological study of 10 cases. Br J Haematol 71:499–504PubMedCrossRef

Ohyashiki K, Sasao I, Ohyashiki JH et al (1991) Clinical and cytogenetic characteristics of myelodysplastic syndromes developing myelofibrosis. Cancer 68:178–183PubMedCrossRef

Verhoef GE, De Wolf-Peeters C, Ferrant A et al (1991) Myelodysplastic syndromes with bone marrow fibrosis: a myelodysplastic disorder with proliferative features. Ann Hematol 63:235–241PubMedCrossRef

Maschek H et al (1992) Myelofibrosis in primary myelodysplastic syndromes: a retrospective study of 352 patients. Eur J Haematol 48:208–214PubMedCrossRef

Lambertenghi-Deliliers G, Annaloro C, Oriani A et al (1993) Prognostic relevance of histological findings on bone marrow biopsy in myelodysplastic syndromes. Ann Hematol 66:85–91PubMedCrossRef

Marisavljevic D, Rolovic Z, Cemerikic V et al (2004) Myelofibrosis in primary myelodysplastic syndromes. Clin Biol Signif Med Oncol 21(4):325–331

Buesche G, Teoman H, Wilczak W, Ganser A, Hecker H, Wilkens L, Göhring G, Schlegelberger B, Bock O, Georgi A, Kreipe H (2008) Marrow fibrosis predicts early fatal marrow failure in patients with myelodysplastic syndromes. Leukemia 22:313–322PubMedCrossRef

10.

Della Porta MG, Malcovati L, Boveri E et al (2009) Clinical relevance of bone marrow fibrosis and CD34-positive cell clusters in primary myelodysplastic syndromes. J Clin Oncol 27:754–762PubMedCrossRef

11.

Santos FPS, Schlette E, Kantarjian H et al. (2009) Myelodysplastic syndrome with fibrosis: experience of a single-institution with 139 patients. Blood 114 (24): Abstract 2775

12.

Horny HP, Sotlar K, Valent P (2007) Diagnostic value of histology and immunohistochemistry in myelodysplastic syndromes. Leuk Res 31(12):1609–1616PubMedCrossRef

13.

Valent P, Orazi A, Büsche G, Schmitt-Gräff A, George TI, Sotlar K, Streubel B, Beham-Schmid C, Cerny-Reiterer S, Krieger O, Loosdrecht AVD, Kern W, Ogata K, Wimazal F, Csomor J, Varkonyi J, Sperr WR, Werner M, Kreipe H, Horny HP (2010) Standards and impact of hematopathology in myelodysplastic syndromes (MDS). Oncotarget 1:483–496PubMed

14.

Malcovati L, Germing U, Kuendgen A et al (2007) Time-dependent prognostic scoring system for predicting survival and leukemic evaluation in myelodysplastic syndromes. J Clin Oncol 25:3503–3510PubMedCrossRef

15.

Valent P, Horny HP, Bennet JM, Fonatsch C, Germing U, Greenberg P et al (2007) Definitions and standards in the diagnosis and treatment of the myelodysplastic syndromes: consensus statements and report from working conference. Leuk Res 31:727–736PubMedCrossRef

16.

Thiele J, Kvasnicka HM, Facchetti F et al (2005) European consensus on grading bone marrow fibrosis and assessment of cellularity. Haematologica 90:1128–1132PubMed

17.

Brunning R, Orazi A, Germing U et al (2008) Myelodysplastic syndromes, chapter 5. In: Swerdlow S, Campo E, Harris NL et al (eds) World Health Organization classification of tumours of haematopoietic and lymphoid tissue, 4th edition. IARC, Lyon, pp 88–103

18.

Greenberg P, Cox C, LeBeau M et al (1997) International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997(89):2079–2088

19.

Malcovati L, Della Porta MG, Strupp C et al (2011) Impact of the degree of anemia on the outcome of patients with myelodysplastic syndrome and its integration into the WHO classification-based Prognostic Scoring System (WPSS). Haematologica 96:1433–1440PubMedCrossRef

20.

Greenberg PL, Tuechler H, Schanz J et al (2012) Revised International Prognostic Scoring System for myelodysplastic syndromes (IPSS-R). Blood 120(12):2454–2465PubMedCrossRef

21.

Cervantes F, Dupriez B, Pereira A et al (2009) New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. Blood 113(13):2895–2901PubMedCrossRef

22.

Schanz J, Tüchler H, Sole F et al (2012) New comprehensive cytogenetic scoring system for primary myelodysplastic syndromes (MDS) and oligoblastic acute myeloid leukemia after MDS derived from an international database merge. J Clin Oncol 30:820–829PubMedCrossRef

23.

Kremer M, Horn T, Dechow T et al (2006) The JAK2 V617F mutation occurs frequently in myelodysplastic / myeloproliferative diseases, but is absent in true myelodysplastic syndromes with fibrosis. Leukemia 20:1315–1316PubMedCrossRef

24.

Olsen RJ, Dunphy CH, O’Malley DP et al (2008) The implication of identifying JAK 2 (V617F) in myeloproliferative neoplasms and myelodysplastic syndromes with bone marrow fibrosis. J Hematop 1(2):111–117PubMedCrossRef

25.

Fritsche-Polanz R, Jordan JH, Feix A et al (2001) Mutation analysis of c-kit in patients with myelodysplastic syndromes without mastocytosis and cases of systemic mastocytosis. Br J Haematol 113:357–364PubMedCrossRef

26.

Sotlar K, Bache A, Stellmacher F et al (2008) Systemic mastocytosis associated with chronic idiopathic myelofibrosis. J Mol Diagn 10(1):58–66PubMedCentralPubMedCrossRef

27.

R Development Core Team, R Foundation for Statistical Computing (2009) R: a language and environment for statistical computing [Internet]. Vienna [cited 2009 Apr 17]. Available from: http://www.r-project.org/

28.

Therneau TM, Lumley T (2009) Survival analysis, including penalised likelihood, original report by Thomas Lumley [Internet] [cited 2009 Apr 17]. Available from: http://www.r-project.org/

29.

Steensma DP, Dewald GW, Lasho TL et al (2005) The JAK2 V617F activating tyrosine kinase mutation is an infrequent event in both “atypical” myeloproliferative disorders and myelodysplastic syndromes. Blood 106:1207–1209PubMedCrossRef

30.

Suvajdzic N, Marisavljevic D, Kraguljac N et al (2004) Acute panmyelosis with myelofibrosis: clinical, immunophenotypic and cytogenetic study of twelve cases. Leuk Lymphoma 45:1873–1879PubMedCrossRef

31.

Orazi A (2007) Histopathology in the diagnosis and classification of acute myeloid leukemia, myelodysplastic syndromes, and myelodysplastic / myeloproliferative diseases. Pathobiology 74(2):97–114PubMedCrossRef

32.

Orazi A, Germing U (2008) The myelodysplastic/myeloproliferative diseases with dysplastic features. Leukemia 22:1308–1319PubMedCrossRef

33.

Bacher U, Schnittger S, Kern W, Weiss T, Haferlach T, Haferlach C (2009) Distribution of cytogenetic abnormalities in myelodysplastic syndromes, Philadelphia negative myeloproliferative neoplasms, and the overlap MDS/MPN category. Ann Hematol 88:1207–1213PubMedCrossRef

34.

Haase D (2008) Cytogenetic features in myelodysplastic syndromes. Ann Hematol 87:515–526PubMedCentralPubMedCrossRef

35.

Haase D, Germing U, Schanz J, Pfeilstöcker M, Noesslinger T, Hildebrandt B, Kuendgen A, Luebbert M, Kunzmann R, Giagounidis AA, Aul C, Truemper L, Krieger O, Stauder R, Mueller TH, Wimazal F, Valent P, Fonatsch C, Steidl C (2007) New insights into the prognostic impact of the karyotype in MDS and correlation with subtypes: evidence from a core dataset of 2124 patients. Blood 110:4385–4395PubMedCrossRef

36.

Yip S-F, So C-C, Chan AY-Y, Liu HS-Y et al (2006) The lack of association between JAK2 V617F mutation and myelodysplastic syndrome with or without myelofibrosis. Leukemia 20:1165PubMedCrossRef

37.

Ohyashiki K, Aota Y, Akahane D et al (2005) The JAK2 V617F tyrosine kinase mutation in myelodysplastic syndromes (MDS) developing myelofibrosis indicates the myeloproliferative nature in a subset of MDS patients. Leukemia 19:2359–2360PubMedCrossRef

Titel: Prognostic impact of blast cell counts in dysplastic bone marrow disorders (MDS and CMML I) with concomitant fibrosis
verfasst von: Sigrid Machherndl-Spandl
W. Sega
H. Bösmüller
U. Germing
Ch. Gruber
K. Nachtkamp
P. Reinecke
W. R. Sperr
F. Wimazal
L. Müllauer
K. Sotlar
H. P. Horny
H. Tüchler
P. Valent
O. Krieger
Publikationsdatum: 01.01.2014
Verlag: Springer Berlin Heidelberg
Erschienen in: Annals of Hematology / Ausgabe 1/2014
Print ISSN: 0939-5555
Elektronische ISSN: 1432-0584
DOI: https://doi.org/10.1007/s00277-013-1945-4

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

^{Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag}

Kostenlos registrieren

Neu im Fachgebiet Innere Medizin

Battle of Experts: Sport vs. Spritze bei Adipositas und Typ-2-Diabetes

11.05.2024 DDG-Jahrestagung 2024 Kongressbericht

Im Battle of Experts traten zwei Experten auf dem Diabeteskongress gegeneinander an: Die eine vertrat die Auffassung „Sport statt Spritze“ bei Adipositas und Typ-2-Diabetes, der andere forderte „Spritze statt Sport!“ Am Ende waren sie sich aber einig: Die Kombination aus beidem erzielt die besten Ergebnisse.

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Klimawandel und Gesundheit: Was Sie in der Hausarztpraxis wissen müssen und tun können

Springer Medizin

Prognostic impact of blast cell counts in dysplastic bone marrow disorders (MDS and CMML I) with concomitant fibrosis

Abstract

Leitlinien kompakt für die Innere Medizin

Neu im Fachgebiet Innere Medizin

Battle of Experts: Sport vs. Spritze bei Adipositas und Typ-2-Diabetes

Vorsicht, erhöhte Blutungsgefahr nach PCI!

Triglyzeridsenker schützt nicht nur Hochrisikopatienten

Gibt es eine Wende bei den bioresorbierbaren Gefäßstützen?

Update Innere Medizin

Klimawandel und Gesundheit: Was Sie in der Hausarztpraxis wissen müssen und tun können

Springer Medizin

Abstract

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 1/2014

A phase 1/2 study of oral panobinostat combined with melphalan for patients with relapsed or refractory multiple myeloma

Younger patients with chronic myeloid leukemia do well in spite of poor prognostic indicators: results from the randomized CML study IV

Real life experience with alemtuzumab treatment of patients with lower-risk MDS and a hypocellular bone marrow

An atypical manifestation of acquired von Willebrand syndrome (AVWS) associated with systemic lupus erythematosus (SLE)

No difference between posaconazole and fluconazole antifungal prophylaxis and mycological diagnostics except costs in patients undergoing AML chemotherapy: a 1-year "real-life" evaluation

Lenalidomide with dexamethasone treatment for relapsed/refractory myeloma patients in Korea—experience from 110 patients

Leitlinien kompakt für die Innere Medizin

Neu im Fachgebiet Innere Medizin

Battle of Experts: Sport vs. Spritze bei Adipositas und Typ-2-Diabetes

Vorsicht, erhöhte Blutungsgefahr nach PCI!

Triglyzeridsenker schützt nicht nur Hochrisikopatienten

Gibt es eine Wende bei den bioresorbierbaren Gefäßstützen?

Update Innere Medizin