Recurrent granulocytic sarcoma. An unusual variation of acute myelogenous leukemia associated with 8;21 chromosomal translocation and blast expression of the neural cell adhesion molecule.
This study reports on a patient with acute myelogenous leukemia (AML) in remission who had a series of 11 granulocytic sarcomas (chloromas or myeloblastomas) appearing periodically over a 29-month interval in a variety of anatomic sites without evidence of bone marrow recurrence. This isolated extramedullary recurrence of AML is distinctly unusual with only 24 cases described previously. This patient had the greatest number and longest reported interval of recurrent granulocytic sarcomas (GS) before bone marrow relapse. Furthermore, he represents the first case of a patient with GS presenting with both an 8;21 chromosomal translocation and neural cell adhesion molecule (CD56) expression. The authors hypothesize that these two abnormalities identified previously as predisposing factors to GS may, in fact, be synergistic for this phenomenon. His case and the review of the literature demonstrate some of the important clinical and management features of a patient who develops GS while in complete marrow remission from previous AML. Although highly sensitive to radiation therapy, the onset of granulocytic sarcomas is almost always followed by bone marrow relapse and should be treated with aggressive reinduction chemotherapy and local irradiation. Such therapy is associated with the longest interval of disease-free survival.
Byrd
J C
JC
Department of Medicine, Walter Reed Army Medical Center, Washington, DC 20307.
Weiss
R B
RB
D000328:Adult; D015703:Antigens, CD / Q000502:physiology*; D000945:Antigens, Differentiation, T-Lymphocyte / Q000502:physiology*; D001752:Blast Crisis / Q000235:genetics* / Q000276:immunology / Q000378:metabolism; D019002:CD56 Antigen; D015816:Cell Adhesion Molecules, Neuronal / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D002891:Chromosomes, Human, Pair 21; D002898:Chromosomes, Human, Pair 8; D014644:Genetic Variation; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000235:genetics* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D014178:Translocation, Genetic
D015703:Antigens, CD; D000945:Antigens, Differentiation, T-Lymphocyte; D019002:CD56 Antigen; D015816:Cell Adhesion Molecules, Neuronal