Monosomy 7 syndrome and JCML share many common clinical manifestations and hematologic findings. Both can also be classified as refractory anemia, refractory anemia with excessive blasts, refractory anemia with excess blasts in transformation, and chronic myelomonocytic leukemia according to FAB system of myelodysplastic syndrome.

However, monosomy 7 syndrome differs from JCML in the following ways :

• Hemoglobin F is rarely observed to be greater than 10%
  
  
• Thrombocytopenia is usually not an early prominent feature.
  
  
• Many patients progress to AML.
  
  
• Neutrophil function is impaired with defective chemotaxis and killing (normal in JCML).
  
  
• Monosomy 7 occurs in families.
  

Treatment
Allogenic bone marrow transplantation is curative therapy. Because of an increased familial incidence of monosomy 7 syndrome, bone marrow cells of a prospective family member donor should have a chromosomal analysis to exclude monosomy 7 prior to bone marrow transplantation.

Chemotherapy
Chemotherapy is only utilized for cytoreduction and palliation and similar therapy to that recommended for JCML can be employed.

Last created on 24-06-2002
Last updated on 01-07-2006