Kentucky Cancer Registry

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Hematopoietic and Lymphoid Neoplasm Project

• Kentucky Cancer Registry
• Spring training, 2010

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Why New Rules?

• Medical advances
• Immunophenotyping, molecular and genetic testing
• New, more specific histology terms
• Revised WHO Classification of Hematopoietic and
  Lymphoid Neoplasms
• New reportability conditions
• 9751/3, 9975/3, 9831/3, and transformations

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New Reference the Hematopoietic Database

• Manual (Rules)

• Database

• Disease definition
• Synonyms
• Definitive diagnostic method
• Genetic tests and Immunophenotyping
• Treatments
• Transformations
• Abstractor notes

• Reportability
• Multiple primary rules
• Site and histology codes
• Grade
• Glossary
• Tables
• MP Calculator

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Hematopoietic Database
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Brief demo of database features

• Casefinding lists
• Hematopoietic Manual
• Search feature
• By diagnosis or by code
• Displayed information
• Abstractor notes
• Multiple Primary Calculator

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WHO Classification
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Cell Lineages
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• acute chronic
• immature mature
• undifferentiated differentiated
• Reference Hoffbrand, Pettit, Vyas Color Atlas
  of Clinical hematology (4th ed.), 2010

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Myeloid Line 6 Tables

• Myeloproliferative Neoplasms
• Myelodysplastic/Myeloproliferative Neoplasms
• Myelodysplastic Syndromes
• Myeloid and Lymphoid Neoplasms with Eosinophilia
  and Abnormalities of PDGFRA, PDGFRB or FGFR1

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• from presentation by Graca M. Dores, MD, MPH,
  Classification, Characteristics, and Behavior of
  Myeloid Neoplasms, given at the SEER Advanced
  Topics Workshop on April 19, 2010.

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• from presentation by Graca M. Dores, MD, MPH,
  Classification, Characteristics, and Behavior of
  Myeloid Neoplasms, given at the SEER Advanced
  Topics Workshop on April 19, 2010.

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from presentation by Graca M. Dores, MD, MPH,
Classification, Characteristics, and Behavior of
Myeloid Neoplasms, given at the SEER Advanced
Topics Workshop on April 19, 2010.
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Myeloid Tables 5 and 6

• Acute Myeloid Leukemia and Related Precursor
  Neoplasms
• Acute Leukemias of Ambiguous Lineage
• Show no clear differentiation along a single
  lineage
• Includes those with mixed phenotypes

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Lymphoid Line 6 Tables

• Precursor Lymphoid Neoplasms
• Mature B-Cell Neoplasms
• Mature T-Cell and NK-Cell Neoplasms
• Hodgkin Lymphoma
• Histiocytic and Dendritic Cell Neoplasms
• Post-Transplant Lymphoproliferative Disorders

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at www.lymphomation.org/about-details.htm
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WHO Classification 2008 Principles

• Term NHL becoming obsolete
• Neoplasms of precursor cells classified
  separately from those of more mature cells
• Leukemia/Lymphoma are different manifestations of
  the same disease
• Mature lymphoid neoplasms are subclassified on
  the basis of differentiation, morphology,
  immunophenotype, genetic tests, and clinical
  presentation

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Classification based on

• Morphology and biologic features
• Lymphoma, Myeloid sarcoma, and plasma cell tumors
  are diagnosed by tissue biopsy
• Leukemias by blood counts and BM biopsy
• Genetic tests DNA analysis
• FISH for follicular lymphoma
• Karyotyping of CML for Philadelphia chromosome

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Classification based on

• Immunophenotype
• Identifies molecules associated with some
  lymphomas and leukemias which are expressed on
  the outer surface of the cell
• Includes IHC and flow cytometry
• Examples are Burkitt lymphoma and Adult T cell
  leukemia/lymphoma
• Clinical features
• Myeloproliferative neoplasm, unclassifiable

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Special Types of Diagnoses

• Provisional diagnoses
• NOS histology could be awaiting further test
  results or could be only information available.
  Update if more info comes in
• NOS with possible/probable specific histology
  DO NOT USE these ambiguous terms code the NOS
  term until more definitively diagnosed
• Ex. MPN (9960/3), probably PV (9950/3). Code 9960
  until you have a definitive diagnosis
• Diagnosis of exclusion based on equivocal tests
  and clinical presentation
• Examples MDS, refractory thrombocytopenia

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Diagnostic Confirmation
Code Label Definition
1 Positive histology Tissue microscopically examined and positive
2 Positive cytology Fluid cells microscopically examined and positive
3 Positive histology PLUS Positive immunophenotyping AND/OR Positive genetic studies Histology is positive for cancer, and there are also positive immunophenotyping and/or genetic test results.

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Review Manual Sections

• Whats New
• Reportability rules
• Multiple Primary rules
• Primary site and histology rules
• Grade rules
• Glossary and Appendices

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Hematopoietic Database
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Review database Features

• Display codes
• Search terms and codes
• Definitions, synonyms
• Display special studies, treatment,
  transformations
• Abstractors notes
• MP Calculator