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Blood, 15 November 2001, Vol. 98, No. 10, pp. 2935-2941
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Myelodysplastic syndromes, from French-American-British to World
Health Organization: comparison of classifications on 431 unselected
patients from a single institution
Thomas Nösslinger,
Regina Reisner,
Elisabeth Koller,
Helga Grüner,
Heinz Tüchler,
Hadwiga Nowotny,
Elisabeth Pittermann, and
Michael Pfeilstöcker
From the Third Medical Department for Hematology and
Oncology and the Ludwig Boltzmann Institute for Leukemia Research and
Hematology, Hanusch Hospital, Vienna, Austria.
In 1999 a working group of the World Health Organization (WHO)
published a revised classification for myelodysplastic syndromes (MDS):
RA, RARS, refractory cytopenia with multilineage dysplasia (RC+Dys),
RAEB I and II, del (5q) syndrome, and MDS unclassifiable. Chronic
myelomonocytic leukemia (CMML) and RAEB-t were excluded. Standard
French-American-British (FAB) and new WHO classifications have been
compared in a series of patients (n = 431) from a single center,
analyzing morphologic, clinical, and cytogenetic data. According to the
WHO findings, dysgranulocytopoiesis or dysmegakaryocytopoiesis only
were found in 26% of patients with less than 5% medullary blasts.
These patients are thus unclassified and should remain in the
subgroups RA and RARS. Splitting of heterogeneous RAEB into 2 subgroups
according to blast count was supported by a trend to a statistically
significant difference in the single-center study population. Patients
with CMML whose white blood cell counts are above 13 000/µL may be
excluded from the MDS classification, as warranted by WHO, but a
redistribution of patients with dysplastic CMML according to medullary
blast count leads to more heterogeneity in other WHO subgroups.
Although the natural courses of RAEB-T and acute myeloid leukemia (AML)
with dysplasia are different, comparable median survival durations
after treatment in patients with RAEB-T and AML were in favor of the
proposed 20% medullary blast threshold for AML. The homogeneity of
subgroups was studied by evaluating prognostic scores. A significant
shift into lower IPSS risk groups was evident in the new
classification. These data cannot provide evidence for the new WHO
proposal, which should not be adopted for routine clinical use at
present. Some of its aspects can provide a starting point for further
studies involving refined cytogenetics and clinical results.
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