Pancytopenia followed by a period of spontaneous recovery may precede
the diagnosis of acute lymphoblastic leukemia (pre-ALL). Although both
pre-ALL and myelodysplastic syndromes are preleukemic in a strictly t
emporal sense, there are several marked differences between the two co
nditions. We present eight children with pre-ALL who represented 2% of
all cases of childhood ALL. The bone marrow was normo- or hypocellula
r with increased reticulin fibrosis during the pre-ALL phase. No cytog
enetic abnormalities were found at the pre-ALL phase, but had develope
d at the time of overt leukemia in four of the six children examined.
Based on the findings in our patients and on cases reported in the lit
erature, we argue that pre-ALL is likely to represent a paraneoplastic
syndrome early in the leukemic development that might be mediated via
inhibitory properties related to clonally expanding but still cytogen
etically normal cells. The findings may indicate a multistep pathogene
sis of ALL.