1. Hematopoietic Neoplasms Best Coding Practices 2015-16 Edition
Presentation developed by
April Fritz, RHIT, CTR 1

2. Heme and Lymph Coding Rules
Still causing issues on review of cases
No changes from 2015 update
Let’s review
Obsolete morphology codes and what to do with them
Blood vs bone marrow as the primary site
Use of other C42._ codes
First course of treatment guidelines
Diagnostic confirmation
Other items to remember
See the Heme DB in action

3. Obsolete Histology Codes
2015 Automated Conversion
Replaced obsolete histology codes
Assigned accurate cell lineage (grade) codes
Revised site codes to be consistent with Heme DB
May shift case to different CS schema
CS algorithms and other revisions/recodes re-run after changes are made
A few codes (9579, 9689, 9716, 9731) required manual review
NO recoding of < 2010 cases
For ≥ 2010 diagnoses, use of obsolete histology codes no longer allowed for
Cases with limited information
Death certificate only cases
Total cases requiring manual review SEER/NPCR approximately Will fail edits if not reviewed and fixed.

4. Obsolete Codes Conversion
9654/3 Hodgkin lymphoma, lymphocyte depletion, diffuse fibrosis
USE 9653/3 Lymphocyte-depleted classical Hodgkin lymphoma
9661/3 Hodgkin granuloma
USE 9650/3 Classical Hodgkin lymphoma
9662/3 Hodgkin sarcoma
9664/3 Hodgkin lymphoma, nodular sclerosis, cellular phase
USE 9663/3 Nodular sclerosis classical Hodgkin lymphoma
9665/3 Hodgkin lymphoma, nodular sclerosis, grade 1
9667/3 Hodgkin lymphoma, nodular sclerosis, grade 2
9670/3 Malignant lymphoma, small B lymphocytic, NOS
USE 9823/3 CLL/small lymphocytic lymphoma
9675/3 Malignant lymphoma, mixed small and large cell, diffuse
USE 9690/3 Follicular lymphoma

5. Obsolete Codes Conversion
9684/3 Malignant lymphoma, large B-cell, diffuse, immunoblastic, NOS
USE 9680/3 Diffuse large B-cell lymphoma (DLBCL), NOS
9728/3 Precursor B-cell lymphoblastic lymphoma
USE 9811/3 B lymphoblastic leukemia/lymphoma, NOS
9729/3 Precursor T-cell lymphoblastic lymphoma
USE 9837/3 T lymphoblastic leukemia/lymphoma
9733/3 Plasma cell leukemia
USE 9732/3 Plasma cell myeloma
9750/3 Malignant histiocytosis
USE 9751/3 Langerhans cell histiocytosis
9752/1 Langerhans cell histiocytosis, unifocal
9753/1 Langerhans cell histiocytosis, multifocal
9754/3 Langerhans cell histiocytosis, disseminated

6. Obsolete Codes Conversion
9760/3 Immunoproliferative disease, NOS
See codes 9761/3 and 9762/3
9764/3 Immunoproliferative small intestinal disease
USE 9762/3 Heavy chain disease
9805/3 Acute biphenotypic leukemia
Assign to one of the new codes in the range.
For acute biphenotypic leukemia, NOS, assign code: 9809/3
9835/3 Precursor cell lymphoblastic leukemia, NOS
USE 9811/3 B lymphoblastic leukemia/lymphoma, NOS
9836/3 Precursor B-cell lymphoblastic leukemia
9960/3 Chronic myeloproliferative disease, NOS
USE 9975/3 Myelodysplastic/myeloproliferative neoplasm unclassifiable
9984/3 Refractory anemia with excess blasts in transformation
USE 9983/3 Refractory anemia with excess blasts
9987/3 Therapy related myelodysplastic syndrome, NOS
USE 9920/3 Therapy-related myeloid neoplasms

7. Blood vs. Bone Marrow as Primary Site
Waldenstrom macroglobulinemia (9761/3)
ONLY histology coded to primary site blood (C42.0)
All other heme diseases coded to primary site bone marrow (C42.1)
Code any leukemia diagnosed on peripheral blood smear to C42.1, not C42.0
In rare situations, lymphoma may be coded to C42.1 as primary site but never C42.0
C42.3 (reticuloendothelial system, NOS) and C42.4 (hematopoietic system, NOS) not allowed as primary site
Converted to C42.1 bone marrow
Watch out for Waldenstroms and lymphoplasmacytic lymphoma (codes of each other are dyslexic). When both occur OR when there is adenopathy with Waldenstrom's, it is coded as the lymphoma.

8. Reminder: What to Code in Treatment
Effective with 2010 diagnoses and forward
Do not collect blood transfusions as treatment
Do collect phlebotomy for polycythemia vera ONLY
Collect blood thinners, anticoagulants and/or anti-clotting agents for heme neoplasms ONLY
Mast cell sarcoma, mast cell leukemia, systemic mastocytosis
Chronic myelogenous leukemia BCR/ABL-positive
Polycythemia vera
Primary myelofibrosis
Essential thrombocythemia
Chronic neutrophilic leukemia
Myelodysplastic/myeloproliferative neoplasm, unclassifiable
Blood thinner examples
Heparin, warfarin, Coumadin, Plavix, Pradaxa, Brilinta
Do not code these drugs for other clotting conditions
Anti-clotting agent example: Aspirin
Do not collect blood thinners used for atrial fibrillation, deep vein thrombosis, pulmonary embolism or prophylactic treatment for hip or knee replacement surgery

9. Blood Thinners / Anticoagulants / Anti-clotting Drugs
Aggreonox (Dipyridamole)
Aggrastat (Tirofiban)
Agrylin (Anagrelide Hcl)
Arixtra (Fondaparinox)
Brilinta (Ticagrelor)
Coumadin (Warfarin)
Eliquis (Apixaban)
Flolan (Epoprostenol sodium)
Fragmin (Dalteparin)
Heparin [generic name]
Integrelin (Eptifibatide)
Lovenox (Exnoxaprin)
Normiflo (Ardeparin)
Orgaran (Danaparoid)
Persantine (Dipyridamole)
Plavix (Cloidrogel)
Pletal (Cilostazol)
Pradaxa (Dabigatran etexilate mesylate)
Reopro (Abciximab)
Ticlid (Ticlopidine)
Xarelto (Rivaroxaban)
Note: Not a complete list

10. First Course of Treatment for Hematopoietic Neoplasms
New section of rules
How to code treatment when disease transforms
Comments
‘Treatment’ refers to cancer-directed Tx (systemic or surgery), not passive Tx (supportive care or observation)
First course of treatment (FCOT) ends when treatment plan is completed—no time limit
Code Tx on both abstracts when multiple primaries and Tx given for one also affects/treats the other.
Donor leukocyte infusions
Abstract as bone marrow transplant when given, even if not listed in treatment section of database
Example of coding for both primaries: Patient is diagnosed in May 2014 with both multiple myeloma (9732/3) and mantle cell lymphoma (9673/3), which are separate primaries per rule M15. The oncologist states she began Velcade chemotherapy for the lymphoma. Velcade would affect both primaries, so it should be coded on both abstracts.

11. First Course of Treatment for Hematopoietic Neoplasms
Instructions
If only 1 neoplasm, code documented FCOT
Chronic  Acute transformation
Presence/absence of TX does not affect number of primaries
FCOT for chronic neoplasm may or may not be completed prior to transformation
Acute  Chronic transformation
Presence/absence of TX does affect number of primaries (M12, M13)
FCOT may not have been completed when chronic diagnosed
FCOT may have been completed and patient cancer free for interim when chronic neoplasm diagnosed
FCOT completed but patient not cancer free when chronic diagnosed

12. Reminder: Diagnostic Confirmation Codes
Microscopically Confirmed
1 Positive histology (tissue)
2 Positive cytology (cells) (rare for heme dx)
3 For histologies only: Positive histology PLUS Positive immunophenotyping AND/OR Positive genetic studies
4 Positive microscopic confirmation, method not spec.
“Cytochemistry” (Dx Conf code 3) includes
Immunophenotyping
Flow cytometry
CD antigens
Genetic studies
Chromosome studies
Cytogenetics
Karyotype analysis
In situ hybridization (FISH)

13. Reminder: Diagnostic Confirmation Codes
Use code 1 (Positive histology) when ONLY tissue, marrow or blood is used to diagnose a specific histology
Code 1 includes
For heme diseases, bone marrow aspiration or biopsy, lymph nodes, organs or other tissues
For leukemia only, complete blood count (CBC), white blood count (WBC)
Peripheral blood smear – can be used as histologic diagnosis for any heme diagnosis
Neoplasm microscopically confirmed AND no further testing done (immunophenotyping, genetic testing, JAK2) OR testing negative for disease being abstracted OR test not listed in Definitive Dx Methods
Historical cases not in registry database

14. Reminder Diagnostic Confirmation Codes
Do not use code 1 if diagnosis is based on immunophenotyping or genetic testing using tissue, bone marrow, or blood.
Code 3 Positive histology PLUS positive immunophenotyping or positive genetic testing
Use when special testing (immunophenotyping, genetic testing or JAK-2)
Confirms diagnosis OR
Identifies more specific histology
Do not use when
Testing identifies more specific histology but uses ambiguous terminology OR
Test result preceded by “patchy weak staining”
Genetics and/or immunophenotyping information added to data base for all applicable diseases
Angst about their "ambiguous" term and how you cannot use it to code. If they talked to doctors who treat daily, they would know that when a pathologist uses a term like "most likely", that is what the oncologist believes.

15. Reminder Diagnostic Confirmation Codes
Code 3 Examples
Example 1: Bone marrow biopsy shows acute myeloid leukemia (9861/3). Immunophenotyping shows AML with inv(16)(p13.1q22) (9871/3; use this morphology code)
Example 2: CBC “suggestive of” acute leukemia. Bone marrow biopsy confirms monosomy but not acute leukemia. Final diagnosis: RAEB2 based on bone marrow and cytogenetics.
Example 3: Bone marrow bx: plasma cell dyscrasia (9765/1). Peripheral blood smear compatible with plasma cell leukemia. FISH and chromosome analysis show plasma cell myeloma. Use code 3 because FISH confirms multiple myeloma/plasma cell myeloma.

16. Using the Rules: Four Steps
Is it reportable? (Reportability Instructions)
How many primaries do I abstract? (M Rules)
How do I code the primary site and histology? (PH Rules)
How do I code the grade? (Grade Rules)

17. Case Reportability Instructions
No changes from previous versions
Not ‘rules’—not hierarchical
Text format only
If diagnosis uses ambiguous term, review list
Do not use ambiguous terms for assigning morphology code or primary site
Follow back to physician office if indicated to confirm diagnosis; avoid under-reporting
For heme diseases, special attention should be paid to information in physician offices

18. M Instructions and Rules
No additions or deletions for M rules for 2015 diagnoses and later
Changes to rules are minor and address clarifications
New instruction: Use the M rule references in the Heme DB as a guide only. Start with M1 for each case, move through the rules and stop at the first rule that applies.

19. Reminder: Distinguishing Acute vs. Chronic Neoplasms
Follicular lymphoma (chronic)
Diffuse large B-cell (acute)

20. PH Instructions and Rules
No additions or deletions for PH rules for 2015 diagnoses and later
Changes to rules are minor and address clarifications
Primary Site Coding Instructions
New section added to 2015 manual to further define how to code primary site
Primary source for primary site assignment for hematopoietic neoplasms.
Matches what is ALREADY in the database
New field included in Primary Sites section
Primary site guidelines moved from Abstractor Notes
Additional requirements included here
Example: “Primary site must be bone marrow” for leukemias

21. PH Instructions and Rules
Primary Site Coding Instructions, continued
Detailed instructions for certain site-histology combinations
9679/3 Primary mediastinal (thymic) lymphoma  thymus or mediastinum as appropriate
9731/3 Solitary plasmacytoma of bone  bone
9761/3 Waldenstrom macroglobulinemia  blood
Histologies coded to bone marrow
Histologies coded to spleen
Histologies coded to skin
Histologies coded to lymph nodes (in most circumstances)
Primary sites for lymphoma/leukemias
Other hematopoietic histologies (Module 7)
New note: More than one PH rule will be required to code the primary site for some cases

22. PH Rules – Module 7 Histologies Included
Histologies without a default primary site
9590/3-9729/3 Malignant Lymphomas
9734/3 Extraosseous plasmacytoma
9740/3 Mast cell sarcoma
9755/3-9759/3 Histiocytic and Dendritic cell neoplasms
9762/3 Heavy chain disease
9930/3 Myeloid sarcoma
9971/3 Post-transplant lymphoproliferative disease
Rule PH22 (If site unknown, code to C77.9) – Missing statement from 2014 update restored
Last bullet “Lymph node(s) are involved and no primary site/particular lymph node region is identified.”

23. Histology Coding Instructions
New section added to PH rules in 2015 manual to further define how to code histology
Definitive diagnostic methods defined
When Definitive Diagnostic Method tests or reports are not available, code from (in priority)
1. Original scans, testing, or pathology reports
2. Consultations referring to histology
3. Death certificate (central/regional registries only)
Clarification developed for rules regarding NOS histologies:
When test or report lists a specific histology with ambiguous term(s) and an “NOS” histology, code the NOS histology.
For incidence reporting, this prevents coding a temporary/ provisional histology that could change with further testing that may not appear in the patient’s chart.

24. Histology Coding Instructions
New Notes
A specific histology can be assigned if there is documentation that the physician is treating the patient for the specific disease.
If there is only one histology available and it is preceded by ambiguous terminology, review Abstractor Notes in Heme DB to see if other information can be used to confirm the diagnosis
If the Abstractor Notes, immunophenotyping or genetics information and the only histology available is preceded by ambiguous terminology, code the ambiguous histology so that the case can be reported for incidence. 
Angst about their "ambiguous" term and how you cannot use it to code. If they talked to doctors who treat daily, they would know that when a pathologist uses a term like "most likely", that is what the oncologist believes.

25. G Rules No changes from previous version Reminders
Do not code well-, moderately-, or poorly differentiated statements
Do not code low grade, intermediate grade, or high grade statements
Do not code grade 1, grade 2 or grade 3 for follicular lymphoma
Automated conversion may change some phenotype (grade/6th digit) codes

26. Reminder: Appendix C Lymph Node Chain Reference Table
Alphabetic order
ICD-O-3 code
ICD-O-3 lymph node region
AJCC lymph node region
Use with PH rules to determine whether involved nodes are in a single or multiple regions
Term
ICD-O
Code
ICD-O-3 LN Region
AJCC LN Region
Cloquet’s node
C77.4
Inguinal region or leg
Inguino-femoral, right and left*
Colic NOS, ileocolic, mesocolic, middle (right)
C77.2
Intra-abdominal
Mesenteric
Common bile duct
Para-aortic
Cubital
C77.3
Axilla or arm
Axillary, right and left*
Cystic duct

27. Reminder: Read the Notes in DB!

28. Non-Reportable Conditions Note: Local guidelines may make these reportable-by-agreement.
Listed in Appendix F
Examples – DO NOT REPORT
Acquired hypogammaglobulinemia
Amyloidosis [by itself]
Anemia
Aplastic, macrocytic, hemolytic anemias
Anemia of chronic disorders
Anemia in neoplastic disease
Castleman’s disease [by itself]
Hemophagocytic syndrome
Hemophagocytic lymphohistiocytosis
Idiopathic thrombocytic purpura (ITP)
Not the same as refractory thrombocytopenia
Light chain disease

29. Non-Reportable Conditions Note: Local guidelines may make these reportable-by-agreement.
Examples – DO NOT REPORT
Lymphocytosis
Mast cell activation syndrome (MCAS)
Monoclonal B lymphocytosis of uncertain significance (MLUS)
Myelodysplasia [by itself]
Myelofibrosis [NOS]
Plasma cell dyscrasia
Plasma cell neoplasm [by itself]
Polycythemia [by itself]
Polycythemia vera secondary to volume depletion
Sclerosing extramedullary myeloid tumor
Report underlying myeloproliferative neoplasm if known
Secondary myelofibrosis
The diagnosis of “myelodysplasia” is not reportable because the term includes both non-malignancies and malignancies. Follow-back to the physician is necessary to determine whether or not a particular case represents a malignancy.  The following definition will be added to the glossary in the Hematopoietic and Lymphoid Neoplasm manual. “The term myelodysplasia covers a group of disorders that result in the inability to produce enough healthy mature blood cells. Those disorders include: Anemia, leukopenia, thrombocytopenia, MDS, refractory anemia, refractory anemia with excess blasts in transformation, refractory anemia with ring sideroblasts, refractory anemia with excess blasts, chronic myelomonocytic leukemia, acute myeloid leukemia.” 

30. Non-Reportable Conditions Note: Local guidelines may make these reportable-by-agreement.
Examples – DO NOT REPORT
Thrombocythemia [by itself]
Thrombocytosis [by itself]
Reactive thrombocytosis
Thrombocytopenia
Essential thrombocytopenia
Autoimmune thrombocytopenia
Thrombocytopenia of unknown etiology
The diagnosis of “myelodysplasia” is not reportable because the term includes both non-malignancies and malignancies. Follow-back to the physician is necessary to determine whether or not a particular case represents a malignancy.  The following definition will be added to the glossary in the Hematopoietic and Lymphoid Neoplasm manual. “The term myelodysplasia covers a group of disorders that result in the inability to produce enough healthy mature blood cells. Those disorders include: Anemia, leukopenia, thrombocytopenia, MDS, refractory anemia, refractory anemia with excess blasts in transformation, refractory anemia with ring sideroblasts, refractory anemia with excess blasts, chronic myelomonocytic leukemia, acute myeloid leukemia.” 

31. Other Non-Reportable Conditions Note: Local guidelines may make these reportable-by-agreement.
Serum hyperviscosity syndrome
Reactive lymphoid hyperplasia
Mastocytosis (not stated as malignant or systemic)
Lymphomatoid/lymphoid granulomatosis
Angiocentric immunoproliferative lesion (AIL)
Angioimmunoblastic lymphadenopathy
Plasmablastic hyperplasia (new code 9971/1)
Graft versus Host disease (GVHD)
Immunoglobulin deposition disease
Primary amyloidosis
Systemic light chain disease
Heavy chain
Monoclonal
Chemotherapeutic atypia
Hemophagocytic lymphohistiocytosis

32. Best Coding Practices Summary
Use Heme DB to determine number of primaries
Enter diagnostic term in DB
Check alternate names section for other terms
Do not use ambiguous terms to code histology
Go to Heme Manual M rules
Review rules in order
Stop at first rule that fits the case
Go back to Heme DB to determine primary site(s) and histology code(s)
Check Abstractor Notes for additional information
If indicated in Heme DB, use Multiple Primaries Calculator to determine correct histology
Use G rules to code grade

33. Any Questions?

34. Final Thoughts
Before I came here I was confused about this subject. Having listened to your lecture I am still confused. But on a higher level.
—Enrico Fermi
Thank you for your attention.

37. Follicular Lymphoma In Situ Note: Local guidelines may make FLIS reportable-by-agreement.
Not reportable
Recently identified pathologic entity
Still some controversy regarding criteria for identifying in situ lymphoma
Experts recommend to wait until guidelines had been established for pathologists
Probably will collect FLIS in the future