Korean J Hematol.  2000 May;35(2):189-194.

A Case of Juvenile Myelomonocytic Leukemia in a Six Months Old Infant

• Chang EA
• Kim MK
• Lim YA
• Hyun BH
• Kim SW
• Park KH
• Hong J

Abstract

Myelodysplastic syndrome (MDS) in childhood is a rare hematologic malignancy and its classification has been the subject of some controversy. Cases of pediatric MDS are subdivided into those with features of adult-type MDS and those with myeloproliferative features occasionally observed in infancy and early childhood. There appears to be an international consensus to rename the disease juvenile myelomonocytic leukemia (JMML), which includes all leukemias of childhood previously classed as chronic myelomonocytic leukemia (CMML), juvenile chronic myelogenous leukemia (JCML), and infantile monosomy 7 syndrome. We experienced a 6-month-old female infant with JMML who developed extensive extramedullary hematopoiesis. The patient developed abdominal distention, hepatosplenome-galy, anemia, thrombocytopenia, and leukocytosis with significant monocytosis and was found to have a high hemoglobin F level of 30%. Her bone marrow biopsy section and aspirate smears revealed normocellularity with no increment of blast cells and no dysplastic changes. Cytogenetic analysis revealed a normal 46, XX karyotype. Her liver, spleen, lymph nodes, and appendix were found to be heavily infiltrated by partially differentiated myelomonocytic cells. These findings supported the diagnosis of JMML with extensive extramedullary hematopoiesis.

Keyword

Juvenile myelomonocytic leukemia; Childhood myelodysplastic syndrome; Fetal hemoglobin; Infant; Extramedullary hematopoiesis

MeSH Terms

Anemia
Appendix
Biopsy
Bone Marrow
Classification
Consensus
Cytogenetic Analysis
Diagnosis
Female
Fetal Hemoglobin
Hematologic Neoplasms
Hematopoiesis, Extramedullary
Humans
Infant*
Karyotype
Leukemia
Leukemia, Myelomonocytic, Chronic
Leukemia, Myelomonocytic, Juvenile*
Leukocytosis
Liver
Lymph Nodes
Monosomy
Myelodysplastic Syndromes
Spleen
Thrombocytopenia
Fetal Hemoglobin