Medical College of Wisconsin
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Philadelphia chromosome-positive chronic myeloid leukemia and thoracic ganglioneuroma. Previously undescribed association. Am J Pediatr Hematol Oncol 1993 Feb;15(1):111-4

Date

02/01/1993

Pubmed ID

8447551

DOI

10.1097/00043426-199302000-00016

Scopus ID

2-s2.0-0027537339 (requires institutional sign-in at Scopus site)   3 Citations

Abstract

A previously healthy 11 year-old-white boy presented with persistent mild leukocytosis and a posterior mediastinal mass. Surgical excision of the mass showed it to be a ganglioneuroma. Postoperatively, the patient had marked leukocytosis (white blood cell count 57,000/mm3) and a low leukocyte alkaline phosphatase score. Bone marrow aspirate for cytogenetics performed in the evaluation of the posterior mediastinal mass showed the presence of the Philadelphia chromosome (Ph) in 19 of 20 metaphase spreads. The diagnosis of Ph+ chronic myeloid leukemia (CML) was confirmed on repeat cytogenetics. Subsequent detailed skin examination showed six small cafe-au-lait spots but no other evidence of neurofibromatosis. This is the first report to describe an association between ganglioneuroma and Ph+ CML.

Author List

Shea MP, Abshire TC

Author

Thomas Abshire MD Emeritus Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Child
Ganglioneuroma
Humans
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Male
Mediastinal Neoplasms
Neoplasms, Multiple Primary
Neurofibromatoses
Pigmentation Disorders