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

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

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 2010-2012 approximately 550. Will fail edits if not reviewed and fixed.

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

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

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 9806-9809 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

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.

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

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

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.

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

Reminder: Diagnostic Confirmation Codes Microscopically Confirmed 1 Positive histology (tissue) 2 Positive cytology (cells) (rare for heme dx) 3 For histologies 9590-9992 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)

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

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.

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.

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)

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

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.

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

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

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

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.”

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.

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.

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

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

Reminder: Read the Notes in DB!

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

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.” 

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.” 

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

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

Any Questions?

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.

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