Complications at Diagnosis of Pediatric Chronic Myeloid Leukemia in Chronic Phase

Chronic myeloid leukemia (CML) is uncommon during the first two decades of life, resulting in limited experience of pediatricians in handling this leukemia. This is even more true when it comes to managing rare complications at the time of diagnosis. Most pediatric patients will present with CML in chronic phase, however, with massive hyperleukocytosis. Although rare, issues such as leukostasis, thrombocytosis, and splenomegaly-or a combination of these-may necessitate urgent, life- and organ-saving interventions. Leukostasis typically blocks the small vessels of cerebral, pulmonary, cardiac, retinal, acoustical, osseous, renal, digital, and penile (in males) microvasculature. Combining chemotherapy with leukapheresis or exchange transfusion is required in selected cases. Despite thrombocytosis, no thrombotic complications were observed in pediatric CML, but bleeding signs were observed. These comprised intracerebral hemorrhage, soft tissue bruising, epistaxis, gingival bleeding, gastrointestinal or genitourinary bleeding, and menorrhagia, which may partly be explained by an acquired von Willebrand syndrome. The use of prophylactic low-dose acetylsalicylic acid in children with thrombocytosis cannot be routinely recommended. Splenomegaly may be complicated by splenic infarction, varying from asymptomatic infarction to hemorrhagic shock secondary to massive subcapsular hemorrhage with occasional splenic rupture. This overview explores potential organ-specific complications that may occur at presentation/diagnosis of CML-CP and outlines their management strategies.