PB and BM finding of MPN

 

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Diagnosis Major Minor Criteria D.D.


C.M.L. BCR-ABL1 positive     – Leukemoid reaction
– CMML
– Atypical CML (Ph-neg, BCR-ABL1 -neg)
– Chronic neutrophilic leukemia or ET


P.V. Elevated hemoglobin concentration (>16.5 g/dL in men; >16.0 g/dL in women)
or
Elevated hematocrit (>49% in men; >48% in women)
or
Increased red blood cell mass (>25% above mean normal predicted value)
Subnormal erythropoietin level 3 major
or
first 2 major plus the minor
Secondary erythrocytosis
 
     
  Presence of JAK2 V617ZF or JAK2 exon 12 mutation      
 
     
  Bone marrow biopsy showing age-adjusted hypercellularity with trilineage growth (panmyelosis) with pleomorphic mature megakaryocytes      


E.T. Platelet count > 450 × 109/L) Presence of a clonal marker all 4 major
or
first 3 major and 1 minor
Secondary (Reactive) Thrombocytosis:
 

   
  Normocellular bone marrow biopsy showing proliferation mainly of the megakaryocyte lineage with increased numbers of enlarged, mature megakaryocytes with hyperlobulated nuclei and very rarely minor (grade 1) increase in reticulin fibers absence of evidence for reactive thrombocytosis   – Inflammatory diseases
– Bacterial infections and tuberculosis
– Advanced malignancies
– Postsplenectomy or asplenia
– Iron deficiency
– Bone marrow regeneration, rebound following chemotherapy
– Rebound after vitamin B 12 repletion in pernicious anemia
– Postsurgical procedures
 
     
  Not meeting the WHO criteria for BCR-ABL1 + CML, PV, PMF, MDS, or other myeloid neoplasms    
 
     
  Presence of JAK2, CALR, or MPL mutation      


prefibrotic
P.M.F.
Presence of megakaryocytic proliferation and atypia, without reticulin fibrosis >grade 1, accompanied by an increased age-adjusted bone marrow cellularity, granulocytic proliferation, and often decreased erythropoiesis Leukocytosis ≥11 × 109/L all 3 major and 1 of minor criteria confirmed in two consecutive determinations difficult, may not be resolvable from early PV, early ET
 

   
  Not meeting WHO criteria for PV, CML, ET, MDS, or other myeloid diseases Increased serum lactate dehydrogenase    
 

   
  Presence of JAK2 V617F, CALR , or MPL mutations
or
in the absence of these mutations, presence of other clonal marker
or
no evidence that the marrow fibrosis or other changes are secondary to infection, autoimmune, chronic inflammation, hairy cell leukemia, other malignancies including metastatic tumor, or are due to toxic myelopathies
Anemia not attributed to a comorbid condition    
   
   
    Palpable splenomegaly    

fibrotic
P.M.F.
Presence of megakaryocytic proliferation and atypia, with either reticulin and or collagen fibrosis grade 2 or 3 Leukoerythroblastosis all 3 major and 1 minor Other conditions causing marrow fibrosis:
– metastatic disease,
– certain infections,
– autoimmune myelofibrosis
 

   
  Not meeting WHO criteria for PV, CML, ET, MDS, or other myeloid diseases Leukocytosis ≥11 × 109/L   Other hematopoietic disorders causing marrow fibrosis:
– Hodgkin lymphoma,
– non-Hodgkin lymphoma,
– hairy cell leukemia
 

   
  Presence of JAK2 V617F, CALR , or MPL mutations
or
in the absence of these mutations, presence of other clonal marker
or
no evidence that the marrow fibrosis or other changes are secondary to infection, autoimmune, chronic inflammation, hairy cell leukemia, other malignancies including metastatic tumor, or are due to toxic myelopathies
Increased serum lactate dehydrogenase   Myeloid disorders causing marrow fibrosis:
– post-PV and ET myelofibrosis,
– CML in accelerated phase,
– MDS with fibrosis,
– MDS/MPN with fibrosis,
– AML with fibrosis (including acute panmyelosis with myelofibrosis),
– mast cell disease
   
   
    Anemia not attributed to a comorbid condition    


C.N.L. Leukocytosis with WBC ≥25 × 109/L; segmented neutrophils and band stages account for ≥80% of the WBCs and neutrophilic precursors (promyelocytes, myelocytes, and metamyelocytes) <10% of WBC
monocyte count either <1 × 109/L or <10%
no dysgranulopoiesis
    Reactive neutrophilia, including leukemoid reaction and secondary neutrophilia due to plasma cell proliferation
 
     
  hypercellular bone marrow with increased neutrophilic granulocytes, <5% myeloblasts, a normal pattern of neutrophil maturation, and normal megakaryocytes     CML and CML-neutrophilic variant
 
     
  Not meeting the WHO criteria for BCR-ABL1+ CML, PV, ET, or PMF     Atypical CML
 
     
  No rearrangement of PDGFA, PDGFB, FGFR1 , or PCM1-JAK2      
 
     
  Presence of CSF3R T618I or other activating CSF3R mutation
or
in the absence of CSF3R mutation, persistent neutrophilia (at least 3 months), splenomegaly and no identifiable cause of reactive neutrophilia or demonstration of clonality of myeloid cells