Leukostasis in Children and Adolescents with Chronic Myeloid Leukemia: Japanese Pediatric Leukemia/Lymphoma Study Group

Abstract

This retrospective study represents the largest series of children and adolescents in which leukostasis of CML has been reported. Our data provide useful insight into the characteristics of leukostasis in recent cases of children and adolescents with CML.

The Semantics of Priapism and the First Sign of Chronic Myeloid Leukemia

“…Depending on the CML phase and biology, patients may have a profoundly elevated WBC at diagnosis, which can present as leukostasis, or hyperviscosity syndrome. Hyperviscosity syndrome is associated with serious clinical manifestations including visual discomfort (retinopathy, retinal hemorrhages), shortness of breath (pulmonary insufficiency), priapism, and other symptoms related to end organ damage [5, 7]. If there is clinical evidence consistent with hyperviscosity syndrome, emergent therapy and in rare cases leukophoresis can be initiated.…”

Sequential use of second‐generation tyrosine kinase inhibitors following imatinib therapy in pediatric chronic myeloid leukemia: A report from the Japanese Pediatric Leukemia/Lymphoma Study Group

“…The Japanese Pediatric Leukemia/Lymphoma Study Group's (JPLSG) CML Committee reviewed the records of 256 Japanese children aged <20 years and diagnosed with CML between 1996 and 2011 at 93 institutes, which was previously reported in detail . The study design was approved by the Ethics Committee of Keio University School of Medicine.…”

Management of chronic myeloid leukemia in children and adolescents: Recommendations from the Children's Oncology Group CML Working Group

“…In fact, most patients with a high WBC count do not need leukapheresis. Unlike in acute leukemias, the thrombosis and leukostasis risk associated with hyperleukocytosis is hypothesized to be less frequent in CML due to the preponderance of maturing WBCs rather than enlarged blasts that account for the leukocytosis in acute leukemia . Early initiation of hydroxyurea may help to decrease the WBC and reduce leukostasis risk without the need for apheresis.…”

“…Leukapheresis may be considered emergently if there are signs of leukostasis and end organ injury such as respiratory distress, priapism, severe retinopathy/papilledema, or CNS symptoms concerning for ischemic or hemorrhagic stroke . Leukapheresis is also useful and may be considered in the management of CML in pregnancy .…”