Surgical Pathology Criteria–

Chronic Myelogenous Leukemia (CML)

Differential Diagnosis

Myeloproliferative and Mixed MDS/MPNs with Neutrophilia and/or Monocytosis
  CML-CP Atypical CML CMML-1 CNL
Ph Chromosome and/or BCR-ABL1 Translocation Almost 100% Absent Absent Absent

WBC

 Left shifted neutrophilia* Left shifted neutrophilia Monocytosis, may have left shifted neutrophilia Neutrophilia
Blood basophils Increased Not increased Not increased Not increased
Blood monocytes Variable,** <1x103/μL Variable, <1x103/μL Always >1x103/μL <1x103/μL
Blood immature granulocytes >20% 10-20% Usually <10% <10%
Blood blasts Usually <2% >2% <5% <1%
Granulocytic dysplasia Absent ++ +/- Absent
*rare p230 BCR-ABL1 isoform may show mature neutrophilia, **rare p190 BCR-ABL1 isoform associated with monocytosis

 

 Hematolymphoid Disorders with Thrombocytosis
  CML PV PMF, Cellular Phase ET RARS-T 5q-
Tyrosine kinase ABL p230 variant* JAK2 JAK2 or MPL 50% JAK2 or MPL 50% JAK2 50% JAK2 or MPL 10%
Blood ↑PMN, basophils, platelets Normo or hypochromatic anemia, may have ↑basophils, platelets Anemia, leukocytosis, ↑or nl platelets ↑platelets, often abnormal Dimorphic RBC, ↑platelets Macrocytic anemia, ↑or nl platelets
Marrow ↑↑Myeloids, ↑dwarf megas Panmyelosis, ±erythroid hyperplasia, atypical megas Panmyelosis, atypical tightly clustered megas ↑↑Megas Erythroid dysplasia, ring sideroblasts, ↑megas Erythroid hypoplasia, ↑small megas
Marrow Fibrosis Varies ↑ in spent phase ↑↑ in fibrotic phase Minimal Varies Minimal
Splenomegaly ++ ++ +++ ± ± Absent
Clinical/Other B symptoms Hypertension, thrombosis, pruritus, ↓EPO B symptoms, bleeding, gout Thrombosis, hemorrhage Anemia Anemia
EPO = erythropoietin, * rare p230 BCR-ABL1 isoform can present with thrombocytosis and /or neutrophilia

 

 

Common Myeloproliferative Neoplasms - Overview
  CML PV PMF ET
Tyrosine kinase ABL JAK2 JAK2 or MPL JAK2 or MPL
Blood ↑WBC with IG, basophils Normo or hypochromatic anemia, may have ↑platelets & basophils Leukoerythroblastic with teardrops ↑Platelets, often abnormal
Marrow ↑↑Myeloids Panmyelosis, ± erythroid hyperplasia, atypical megas Panmyelosis, atypical megas ↑↑Megas
Marrow fibrosis Varies ↑In spent phase ↑↑In fibrotic phase Minimal
Splenomegaly ++ ++ +++ ±
Clinical Presentation /Other B symptoms Hypertension, thrombosis, pruritus,↓EPO B symptoms, bleeding, gout Thrombosis, hemorrhage
CML = chronic myeloid leukemia; PV = polycythemia vera; PMF = primary myelofibrosis; ET = essential thrombocythemia; IG = immature granulocytes; Megas = megakaryocytes; EPO = erythropoietin

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