Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: EC:3.4.24.11 (
CD10
)
9,792
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Chronic neutrophilic leukemia
(
CNL
) is a rare myeloproliferative disorder characterized by sustained neutrophilic leukocytosis and absence of the Philadelphia chromosome. Most patients with
CNL
have normal karyotypes, and no specific cytogenetic abnormality has been identified. We report here a patient with
CNL
that evolved to myeloid blast crisis. A 73-year-old man was admitted to the hospital because of marked leukocytosis (leukocyte count 112.5 x 10(9)/L with 91% segmented neutrophils) and massive hepatosplenomegaly that was diagnosed as
CNL
with a normal karyotype. After treatment with hydroxyurea for 7 months, the disease progressed to a blast crisis. Bone marrow showed myeloid hyperplasia with 21% myeloblasts, 15% promyelocytes, and marked dysplastic changes of neutrophils. Blastic cells were positive for
CD10
, CD13, CD14, CD33, CD34, and HLA-DR. Chromosome analysis of the bone marrow cells showed 46,XY,+X in all 20 metaphase spreads. We reviewed 15 cases of
CNL
terminating in the blast crisis and confirmed that all cases transformed into myeloid crises and had poor prognoses. Furthermore, to our knowledge, this is the first case showing the acquired gain of an extra X chromosome as a sole abnormality in
CNL
. The gain of an extra X chromosome may play an important role in the progression from chronic phase to the blast crisis of
CNL
.
...
PMID:Acquired gain of an X chromosome as the sole abnormality in the blast crisis of chronic neutrophilic leukemia. 1199 3
