Transcript Hematopoietic and Lymphoid Neoplasm Project

H EMATOPOIETIC AND
LYMPHOID N EOPLASM
C ODING ( PER 2010 RULES )
Kentucky Cancer Registry
Abstractor’s Training, March, 2012
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
LYMPHATIC
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
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)
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.
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
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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
WHO C LASSIFICATION
C ELL L INEAGES
“acute”
“chronic”
“immature”
“mature”
“undifferentiated”
“differentiated”
Reference: Hoffbrand, Pettit, Vyas: Color Atlas of Clinical hematology (4th ed.), 2010
M YELOID L INE –
TABLES 1-4

Myeloproliferative Neoplasms
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Myeloid and Lymphoid Neoplasms with
Eosinophilia and Abnormalities of PDGFRA,
PDGFRB or FGFR1

Myelodysplastic/Myeloproliferative
Neoplasms

Myelodysplastic Syndromes
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.
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.
G R A C A M . D O R E S , M D , M P H , C L A S S I F I C AT I O N ,
C H A R A C T E R I S T I C S , A N D B E H AV I O R O F M Y E L O I D N E O P L A S M S , G I V E N AT T H E
S E E R A D VA N C E D T O P I C S W O R K S H O P O N A P R I L 1 9 , 2 0 1 0 .
F R O M P R E S E N TAT I O N B Y
M YELOID 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
LYMPHOID L INE –6 TABLES

Precursor Lymphoid Neoplasms
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Mature B-Cell Neoplasms
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Mature T-Cell and NK-Cell Neoplasms
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Hodgkin Lymphoma
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Histiocytic and Dendritic Cell Neoplasms
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Post-Transplant Lymphoproliferative
Disorders
AT: W W W . LYM P H O M AT I O N . O R G / A B O U T - D E TA I L S . H T M
WHO C LASSIFICATION
2008: P RINCIPLES

Term ‘NHL’ becoming obsolete
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Neoplasms of precursor cells classified
separately from those of more mature cells
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Leukemia/Lymphoma are different
manifestations of the same disease
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Mature lymphoid neoplasms are
subclassified on the basis of differentiation,
morphology, immunophenotype, genetic
tests, and clinical presentation
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
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Karyotyping of CML for Philadelphia
chromosome
C LASSIFICATION BASED ON :
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
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
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Myeloproliferative neoplasm, unclassifiable
S PECIAL T YPES OF
D IAGNOSES
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
Provisional diagnoses
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NOS histology – could be awaiting further test results
or could be only information available. Update if more
info comes in
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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
D IAGNOSTIC
C ONFIRMATION
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.
…
‘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
H EMATOPOIETIC
D ATABASE
2010 R EFERENCE – THE
H EMATOPOIETIC D ATABASE
Manual (Rules)
Database

Reportability

Disease definition
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Multiple primary rules
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Synonyms
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Site and histology codes
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Definitive diagnostic
method

Grade
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Glossary
Genetic tests and
Immunophenotyping

Tables
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Treatments
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Transformations
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Abstractor notes
MP Calculator
R EVIEW M ANUAL
S ECTIONS

What’s New

Reportability rules

Multiple Primary rules
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Primary site and histology rules
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Grade rules
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Glossary and Appendices