Table 1.

Pediatric Myelodysplastic and Myeloproliferative Disorders

Myelodysplastic syndromes (French-American-British) 
 Refractory anemia  
 Refractory anemia with ring sideroblasts 
 Refractory anemia with excess blasts  
 Refractory anemia with excess blasts in transformation  
 Chronic myelomonocytic leukemia 
Juvenile myelomonocytic leukemia (diagnostic criteria)* 
 Suggestive clinical  
  Hepatosplenomegaly 
  Lymphadenopathy  
  Pallor  
  Fever  
  Skin rash 
 Minimal laboratory (all 3 fulfilled)  
  No Philadelphia chromosome, no bcr/abl rearrangement  
  Peripheral blood monocyte count >1 × 109/L  
  Bone marrow blasts <20% 
 Criteria requested for definite diagnosis (at least 2) 
  Hemoglobin F increased for age  
  Myeloid precursors on peripheral blood smear  
  White blood count >10 × 109/L  
  Clonal abnormality (including monosomy 7)  
  GM-CSF hypersensitivity of myeloid progenitors in vitro  
Myeloproliferative syndromes  
 Chronic myeloid leukemia 
 Polycythemia vera  
 Primary thrombocythemia 
 Myelofibrosis/agnogenic myeloid metaplasia  
 Transient myeloproliferative syndrome 
Myelodysplastic syndromes (French-American-British) 
 Refractory anemia  
 Refractory anemia with ring sideroblasts 
 Refractory anemia with excess blasts  
 Refractory anemia with excess blasts in transformation  
 Chronic myelomonocytic leukemia 
Juvenile myelomonocytic leukemia (diagnostic criteria)* 
 Suggestive clinical  
  Hepatosplenomegaly 
  Lymphadenopathy  
  Pallor  
  Fever  
  Skin rash 
 Minimal laboratory (all 3 fulfilled)  
  No Philadelphia chromosome, no bcr/abl rearrangement  
  Peripheral blood monocyte count >1 × 109/L  
  Bone marrow blasts <20% 
 Criteria requested for definite diagnosis (at least 2) 
  Hemoglobin F increased for age  
  Myeloid precursors on peripheral blood smear  
  White blood count >10 × 109/L  
  Clonal abnormality (including monosomy 7)  
  GM-CSF hypersensitivity of myeloid progenitors in vitro  
Myeloproliferative syndromes  
 Chronic myeloid leukemia 
 Polycythemia vera  
 Primary thrombocythemia 
 Myelofibrosis/agnogenic myeloid metaplasia  
 Transient myeloproliferative syndrome 
*

Niemeyer et al.17 

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