Favorable outcome of patients who have 13q deletion: a suggestion for revision of the WHO 'MDS-U' designation.
To characterize bone marrow failure with del(13q), we reviewed clinical records of 22 bone marrow failure patients possessing del(13q) alone or del(13q) plus other abnormalities. All del(13q) patients were diagnosed with myelodysplastic syndrome-unclassified due to the absence of apparent dysplasia. Elevated glycosylphosphatidylinositol-anchored protein-deficient blood cell percentages were detected in all 16 with del(13q) alone and 3 of 6 (50%) patients with del(13q) plus other abnormalities. All 14 patients with del(13q) alone and 2 of 5 (40%) patients with del(13q) plus other abnormalities responded to immunosuppressive therapy with 10-year overall survival rates of 83% and 67%, respectively. Only 2 patients who had abnormalities in addition to the del(13q) abnormality developed acute myeloid leukemia. Given that myelodysplastic syndrome-unclassified with del(13q) is a benign bone marrow failure subset characterized by good response to immunosuppressive therapy and a high prevalence of increased glycosylphosphatidylinositol-anchored protein-deficient cells, del(13q) should not be considered an intermediate-risk chromosomal abnormality.
Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, 13-1 Takaramachi, Kanazawa, Ishikawa 920-8640, Japan.
Kohei
K
Hosokawa
Takamasa
T
Katagiri
Naomi
N
Sugimori
Ken
K
Ishiyama
Yumi
Y
Sasaki
Yu
Y
Seiki
Aiko
A
Sato-Otsubo
Masashi
M
Sanada
Seishi
S
Ogawa
Shinji
S
Nakao
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000741:Anemia, Aplastic / Q000235:genetics* / Q000401:mortality / Q000628:therapy; D000753:Anemia, Refractory / Q000145:classification* / Q000235:genetics* / Q000401:mortality / Q000628:therapy; D000911:Antibodies, Monoclonal / Q000276:immunology; D002872:Chromosome Deletion; D002882:Chromosomes, Human, Pair 13 / Q000235:genetics*; D005260:Female; D005434:Flow Cytometry; D005500:Follow-Up Studies; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000235:genetics* / Q000401:mortality / Q000628:therapy; D011379:Prognosis; D015996:Survival Rate; D014944:World Health Organization; D055815:Young Adult
D000911:Antibodies, Monoclonal