Refractory anaemia with ringed sideroblasts associated with marked thrombocytosis (RARS-T) with superimposed 5q-syndrome

Polish Journal of Pathology

Volumn 61, Issue 2, 2010, Pages 105-109

  Ziarkiewicz, Mateusz ^a   Dwilewicz Trojaczek, Jadwiga ^a   Pastwińska, Anna ^a   Chmarzyńska, Elzbieta ^a   Paszkowska Kowalewska, Małgorzata ^a   Koperski, Łukasz ^a   Jedrzejczak, Wiesław Wiktor ^a   Ziarkiewicz Wróblewska, Bogna ^a,b  

^a MEDICAL UNIVERSITY OF WARSAW   (Poland)

^b INSTITUTE OF ONCOLOGY   (Poland)

Author keywords

5q syndrome; MDS with isolated del(5q); RARS T

Indexed keywords

ANTINEOPLASTIC AGENT; DRUG DERIVATIVE; HYDROXYUREA; LENALIDOMIDE; THALIDOMIDE;

5Q- SYNDROME; AGED; ARTICLE; BONE MARROW CELL; CASE REPORT; CHROMOSOME 5; CHROMOSOME DELETION; DRUG COMBINATION; FLUORESCENCE IN SITU HYBRIDIZATION; GENETICS; HUMAN; MACROCYTIC ANEMIA; MALE; PATHOLOGY; REFRACTORY ANEMIA; SIDEROBLASTIC ANEMIA; THROMBOCYTOSIS;

AGED; ANEMIA, MACROCYTIC; ANEMIA, REFRACTORY; ANEMIA, SIDEROBLASTIC; ANTINEOPLASTIC AGENTS; BONE MARROW CELLS; CHROMOSOME DELETION; CHROMOSOMES, HUMAN, PAIR 5; DRUG THERAPY, COMBINATION; HUMANS; HYDROXYUREA; IN SITU HYBRIDIZATION, FLUORESCENCE; MALE; THALIDOMIDE; THROMBOCYTOSIS;

EID: 79952116554     PISSN: 12339687     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (15)

1. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th edition, IARC Lyon 2008.
2. Gupta R, Soupir CP, Johari V et al. Thrombocytosis with sideroblastic Erythropoesis: a mixed myeloproliferative myelodysplastic syndrome. Leuk Lymphoma 34: 615-619.
3. Hellstrom-Lindberg E, Cazzola M. The Role of JAK2 Mutations in RARS and Other MDS. Hematology Am Soc Hematol Educ Program. 2008; 52-59.
4. Szpurka H, Tiu R, Murugesan G, Aboudola S, et al. Refractory anemia with ringed sideroblasts associated with marked thrombocytosis (RARS-T), another myeloproliferative condition characterized by JAK2 V617F mutation. Blood 2006; 108: 2173-2181.
5. Washington LT, Doherty D, Glassman A, et al. Myeloid disorders with deletion of 5q as the sole karyotypic abnormality: the clinical and pathologic spectrum. Leuk Lymphoma. 2002; 43: 761-765.
6. Giagounidis AA, Germing U, Haase S, et al. Clinical, morphological, cytogenetic, and prognostic features of patients with myelodysplastic syndromes and del(5q) including band q31. Leukemia 2004;18: 113-119.
7. Thiele J, Kvasnicka HM. Clinicopathological criteria for differential diagnosis of thrombocythemias in various myeloproliferative disorders. Semin Thromb Hemost. 2006; 32: 219-230.
8. Thiele J, Kvasnicka HM. Hematopathologic findings in chronic idiopathic myelofibrosis. Semin Oncol 2005; 32: 380-394.
9. Tefferi A, Lasho TL, Schwager SM, et al. The JAK2 (V617F) tyrosine kinase mutation in myelofibrosis with myeloid metaplasia: lineage specifity and clinical correlates. Br J Haematol 2005; 131: 320-328.
10. Sheftel AD, Richardson DR, Prchal J, Ponka P. Mitochondrial iron metabolism and sideroblastic anemia. Acta Haematol 2009; 122: 120-133.
11. Orazi A. Histopathology in the diagnosis and classification of acute myeloid leukaemia, myelodysplastic syndromes, and myeloblastic/myeloproliferative diseases. Pathobiology 2007; 74: 97-114.
12. Pellegrini W, Facchetti F, Marocolo D, et al. Expression of CD34 by megakaryocytes in myelodysplastic syndromes. Haematologica 2000; 85: 1117-1118.
13. List A, Dewald G, Bennett J, et al. Lenalidomide in the Myelodysplastic Syndrome with Chromosome 5q Deletion. N Engl J Med 2006; 355: 1456-1465.
14. Huls G, Mulder AB, Rosati S, et al. Efficacy of single-agent lenalidomide in patients with JAK2(V617F) mutated refractory anemia with ring sideroblasts and thrombocytosis. Blood. 2010 Mar 1. [Epub ahead of print]
15. Quintás-Cardama A, Kantarjian HM, Manshouri T, et al. Lenalidomide plus prednisone results in durable clinical, histopathologic, and molecular responses in patients with myelofibrosis. J Clin Oncol 2009; 27: 4760-4766.