Acute basophilic leukemia lacking basophil-specific antigens: the importance of cytokine receptor expression in differential diagnosis.
De novo acute basophilic leukemia (ABL) is a rare form of myeloid leukemia. The low prevalence of ABL makes it difficult to define its clinical characteristics and to establish an effective therapeutic protocol. We present here a case of de novo ABL in a 64-year-old Japanese man. The diagnosis of ABL depended on the following: (1) metachromasia with toluidine blue stain, (2) intracytoplasmic theta granules identified by electron microscopy, and (3) findings obtained from extensive immunophenotypic analysis. Although blast cells lacked basophil-specific antigens such as CDw17, CD88, and FcepsilonRI, an expression profile of cytokine receptors including CD116 (GM-CSF receptor), CD117 (c-kit), and CD123 (IL-3 receptor alpha) helped to define the cellular lineage in our case. The patient achieved complete remission with intensive chemotherapy composed of idarubicin and cytosine arabinoside and was disease free during the following 30 months. We propose that immunophenotyping, especially focusing on cytokine receptors, is useful in diagnosing ABL.
Yokohama
Akihiko
A
Third Department of Internal Medicine, Gunma University, School of Medicine, Maebashi, Japan. ayoko@showa.gunma-u.ac.jp
Tsukamoto
Norifumi
N
Hatsumi
Nahoko
N
Suto
Miwa
M
Akiba
Tohru
T
Uchiumi
Hideki
H
Maehara
Tadashi
T
Matsushima
Takafumi
T
Karasawa
Masamitsu
M
Murakami
Hirokazu
H
Shinonome
Shougo
S
Saito
Hirohisa
H
Nojima
Yoshihisa
Y
D015703:Antigens, CD / Q000097:blood*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001853:Bone Marrow / Q000473:pathology; D003561:Cytarabine / Q000008:administration & dosage; D003937:Diagnosis, Differential; D006801:Humans; D015255:Idarubicin / Q000008:administration & dosage; D015471:Leukemia, Basophilic, Acute / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D008297:Male; D008407:Mast Cells / Q000276:immunology* / Q000473:pathology; D008875:Middle Aged; D018121:Receptors, Cytokine / Q000235:genetics*; D017455:Receptors, IgE / Q000097:blood
D015703:Antigens, CD; D018121:Receptors, Cytokine; D017455:Receptors, IgE; D003561:Cytarabine; D015255:Idarubicin