Evidence of clonality in chronic neutrophilic leukaemia.
BACKGROUND
Chronic neutrophilic leukaemia (CNL) is a rare myeloproliferative disorder of elderly patients characterised by sustained neutrophilia and splenomegaly. The diagnosis of CNL requires the exclusion of BCR/ABL positive chronic myelogenous leukaemia (CML) and of leukaemoid reactions (LRs). The differentiation between CNL and LR is problematic because both conditions share similar morphological features; it is also important because patients with CNL generally have a poor prognosis.


OBJECTIVE
To determine whether CNL and LR could be distinguished on the basis of different clonality patterns.


METHODS
Blood samples from 52 women were studied using the human androgen receptor gene assay (HUMARA).


RESULTS
Monoclonality was found in the neutrophils in all 17 patients with different myeloproliferative syndromes (MPSs), including those with CNL. In four of the patients with CNL, autologous T cells were also monoclonal, suggesting that they belonged to the neoplastic clone. This finding was in contrast to other MPSs in which T cells were almost always polyclonal. Of nine patients with clinically suspected LR, the neutrophils of five were polyclonal, whereas three patients had monoclonal neutrophils, suggesting that they might be in the process of developing an MPS. Among 26 healthy blood donors, 20 had polyclonal neutrophils and five showed skewed clonality patterns. One case of LR and one normal blood donor were scored "not informative" at the HUMARA locus.


CONCLUSIONS
Clonality studies of blood neutrophils using HUMARA aid in distinguishing female patients with monoclonal CNL from those with LR. For the diagnosis of CNL, monoclonality of the neutrophils should be demonstrated whenever possible.
Böhm
J
J
Department of Pathology, University of Freiburg, Medical School, D-79002 Freiburg, Germany. jboehm@ukl.uni-freiburg.de
Kock
S
S
Schaefer
H E
HE
Fisch
P
P
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003937:Diagnosis, Differential; D004303:Dosage Compensation, Genetic; D005260:Female; D006801:Humans; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000175:diagnosis / Q000235:genetics; D015467:Leukemia, Neutrophilic, Chronic / Q000175:diagnosis* / Q000235:genetics; D007955:Leukemoid Reaction / Q000175:diagnosis* / Q000235:genetics; D008875:Middle Aged; D014411:Neoplastic Stem Cells / Q000473:pathology*; D009504:Neutrophils / Q000473:pathology; D011944:Receptors, Androgen / Q000235:genetics / Q000378:metabolism
D011944:Receptors, Androgen