1. Kentucky Cancer Registry Abstractor’s Training, March, 2012 H EMATOPOIETIC AND L YMPHOID N EOPLASM C ODING ( PER 2010 RULES )

2. C IRCULATORY SYSTEM  Includes  The cardiovascular system Heart and blood vessels Carries nutrients, oxygen, and waste products to and from cells  The lymphatic system Lymphatic vessels, lymph nodes, lymphoid tissue in spleen, thymus, tonsils, and small intestine

3. L YMPHATIC SYSTEM  It is the interconnected system of spaces and vessels between body tissues and organs which carry lymph. It regulates fluid balance in the body and helps fight infection.  Functions:  Drainage of tissue fluid and transport of lymph to the venous system  Defense of body, through filtration and phagocytosis

4. H EMATOPOIESIS  Hematopoiesis is the formation of blood cells  Blood is made up of plasma and formed elements:  Red blood cells (erythrocytes)  White blood cells (leukocytes)  Platelets (thrombocytes)

5. H EMATOPOIESIS  Red blood cells and platelets are formed in the bone marrow (myeloid tissue)  White blood cells are produced in bone marrow and in lymphoid tissue. Some leukocytes (granulocytes, lymphocytes, and monocytes) originate in bone marrow but multiply in lymphoid tissue.

6. H ISTORICAL CLASSIFICATION  Originally thought to be separate systems and separate diseases (leukemias and lymphomas). Classified by size and shape of tumor cells; pattern of spread  Medical advances led to re-classification  Immunophenotyping, molecular and genetic testing  Revised WHO Classification of Hematopoietic and Lymphoid Neoplasms; published in 2008; coding changes in 2010

7. WHO C LASSIFICATION

8. C ELL L INEAGES

9. “acute”“chronic” “immature”“mature” “undifferentiated”“differentiated” Reference: Hoffbrand, Pettit, Vyas: Color Atlas of Clinical hematology (4 th ed.), 2010

10. M YELOID L INE – T ABLES 1-4  Myeloproliferative Neoplasms  Myeloid and Lymphoid Neoplasms with Eosinophilia and Abnormalities of PDGFRA, PDGFRB or FGFR1  Myelodysplastic/Myeloproliferative Neoplasms  Myelodysplastic Syndromes

11. 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.

13. FROM PRESENTATION BY G RACA M. D ORES, MD, MPH, C LASSIFICATION, C HARACTERISTICS, AND B EHAVIOR OF M YELOID N EOPLASMS, GIVEN AT THE SEER A DVANCED T OPICS W ORKSHOP ON A PRIL 19, 2010.

14. M YELOID T ABLES 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

15. L YMPHOID L INE –6 T ABLES  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

16. AT : WWW. LYMPHOMATION. ORG / ABOUT - DETAILS. HTM WWW. LYMPHOMATION. ORG / ABOUT - DETAILS. HTM

17. WHO C LASSIFICATION 2008: P RINCIPLES  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

18. C LASSIFICATION 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

19. C LASSIFICATION 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

20. S PECIAL T YPES OF D IAGNOSES  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

21. D IAGNOSTIC C ONFIRMATION CodeLabelDefinition 1Positive histologyTissue microscopically examined and positive 2Positive cytologyFluid cells microscopically examined and positive 3Positive 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. …

22. ‘O THER ’ T REATMENT  Do NOT collect blood transfusions as treatment for any of these diseases.  Collect phlebotomy as treatment for polycythemia vera ONLY  Collect blood thinners (such as aspirin and heparin, etc.) for:  9740/3 Mast cell sarcoma 9741/3 Systemic mastocytosis 9742/3 Mast cell leukemia 9875/3 Chronic myelogenous leukemia BCR/ABL1 positive 9950/3 Polycythemia vera 9961/3 Primary myelofibrosis 9962/3 Essential thrombocythemia 9963/3 Chronic neutrophilic leukemia 9975/3 Myelodysplastic/myeloproliferative neoplasm, unclassifiable

26. H EMATOPOIETIC D ATABASE

27. 2010 R EFERENCE – THE H EMATOPOIETIC D ATABASE Manual (Rules)  Reportability  Multiple primary rules  Site and histology codes  Grade  Glossary  Tables MP Calculator Database  Disease definition  Synonyms  Definitive diagnostic method  Genetic tests and Immunophenotyping  Treatments  Transformations  Abstractor notes

28. R EVIEW M ANUAL S ECTIONS  What’s New  Reportability rules  Multiple Primary rules  Primary site and histology rules  Grade rules  Glossary and Appendices