Monday, March 30, 2009

Juvenile myelomonocytic leukemia (JMML)

Juvenile myelomonocytic leukemia (JMML) accounts for less than one percent of
childhood leukemias. It is usually diagnosed in children under two years of age and
is more common in boys than in girls. Children with neurofibromatosis 1 (NF 1) are
at increased risk of developing JMML.
The symptoms of JMML are similar to those of the acute leukemias: pale skin, fever,
headaches, sweating, and recurrent infection. Also usually present are enlarged lymph
nodes, enlarged spleen and liver, and a low platelet count. The course of JMML is
unpredictable. Once diagnosed, progressive deterioration usually occurs. However,
infants can survive for several years, but children older than one usually have rapidly
progressive disease.
Because chemotherapy is not generally a successful treatment for JMML, bone marrow
or peripheral blood stem cell transplantation is the best hope for cure. However,
chemotherapy is sometimes used to get the disease under control while preparing for
transplant. Cis retinoic acid may also help to achieve remission in these children.
My daughter was diagnosed with JMML in 1993 at the age of 27
months. Although it is a chronic leukemia, it is particularly fast moving,
and there is no treatment besides BMT. It is also vastly different from the
adult CML. My daughter had a mismatched (5/6) related (my husband’s
sister as donor) BMT four months after she was diagnosed. Today, she is
eight years post-transplant, is in the fourth grade, and is the absolute joy
of my life.
Once parents understand the basics about the type of leukemia their child has, it is
time to begin to decide how and when to tell their child the news.