## BACKGROUND. Extramedullary acute promyelocytic leukemia (APL) is rare, and said to be more common after treatment with all-trans retinoic acid (ATRA) than after any other treatment. ## METHODS. The case of a child with extramedullary relapse of APL after initial treatment with ATRA and that
Acute promyelocytic leukemia. A childhood cluster
β Scribed by Rodney D. Gilbert; C. D. Karabus; A. E. Mills
- Publisher
- John Wiley and Sons
- Year
- 1987
- Tongue
- English
- Weight
- 264 KB
- Volume
- 59
- Category
- Article
- ISSN
- 0008-543X
No coin nor oath required. For personal study only.
β¦ Synopsis
Nine children with acute promyelocytic leukemia (APL) are presented. This series of children represents 7% of all acute leukemias and 21% of acute myelogenous leukemias seen during the same period at the Red Cross War Memorial Children's Hospital. These figures are much higher than the incidence quoted in other series of childhood leukemia. In addition, most of those children came from a confined geographic area. Two of the patients were younger than 2 years of age. The youngest patient with APL previously reported in the literature was 24 months.
Cancer 59:933-935, 1987.
CUTE PROMYELOCYTIC LEUKEMIA (APL) is an un-
A common variant of acute myelogenous leukemia. Clinically, it is characterized by disseminated intravascular coagulation (DIC),2-4 severe bleeding tendency5-' and absence of hepatosplenomegaly and lymphadenopathy.' The peripheral blood shows a leukemic picture composed mostly of promyelocytes. APL is rare in childhood, with published series reporting an incidence of 0% to 2% of all childhood leukemia^,',^.^ and about 4% of acute myelogenous leukemias. I Only one small series has shown an incidence of 7% of all leukemias.'* The Red Cross War Memorial Children's Hospital is a major pediatric referral center; and receives patients from the whole of the Cape Province, as well as occasional patients from other provinces and from neighboring countries. The APL was diagnosed in nine children during the period January 1,198 1 to December 3 1,1985. Of these, seven were from the Eastern Cape, a relatively small part of the total area served by this hospital. This represents a significant geographic and temporal clustering of a rare condition.
π SIMILAR VOLUMES
Because extramedullary depositions of APL are uncommon, information concerning the therapeutic Department of Orthopaedics University Ho Λpital H. Mondor role of ATRA in extramedullary disease is insufficient. In their article, Wiernik et al. state that at least one Creteil, France Gaetano Bacci, M.D