Hematopoietic Neoplasms Best Coding Practices Edition
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1 Hematopoietic Neoplasms Best Coding Practices Edition Presentation developed by April Fritz, RHIT, CTR Hematopoietic Neoplasms Coding Best Practices 1 Heme and Lymph Coding Rules Still causing issues with TCR 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 Work some heme practice cases Hematopoietic Neoplasms Coding Best Practices 2 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 Hematopoietic Neoplasms Coding Best Practices 3 Hematopoietic Neoplasms Coding Best Practices 1
2 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 USE 9650/3 Classical Hodgkin lymphoma 9664/3 Hodgkin lymphoma, nodular sclerosis, cellular phase USE 9663/3 Nodular sclerosis classical Hodgkin lymphoma 9665/3 Hodgkin lymphoma, nodular sclerosis, grade 1 USE 9663/3 Nodular sclerosis classical Hodgkin lymphoma 9667/3 Hodgkin lymphoma, nodular sclerosis, grade 2 USE 9663/3 Nodular sclerosis classical Hodgkin lymphoma 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 Hematopoietic Neoplasms Coding Best Practices 4 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 USE 9751/3 Langerhans cell histiocytosis 9753/1 Langerhans cell histiocytosis, multifocal USE 9751/3 Langerhans cell histiocytosis 9754/3 Langerhans cell histiocytosis, disseminated USE 9751/3 Langerhans cell histiocytosis Hematopoietic Neoplasms Coding Best Practices 5 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 USE 9811/3 B lymphoblastic leukemia/lymphoma, NOS 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 Hematopoietic Neoplasms Coding Best Practices 6 Hematopoietic Neoplasms Coding Best Practices 2
3 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 Hematopoietic Neoplasms Coding Best Practices 7 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 anticlotting 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 Hematopoietic Neoplasms Coding Best Practices 8 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 Hematopoietic Neoplasms Coding Best Practices 9 Hematopoietic Neoplasms Coding Best Practices 3
4 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 Hematopoietic Neoplasms Coding Best Practices 10 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 Hematopoietic Neoplasms Coding Best Practices 11 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) Hematopoietic Neoplasms Coding Best Practices 12 Hematopoietic Neoplasms Coding Best Practices 4
5 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 Hematopoietic Neoplasms Coding Best Practices 13 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 Hematopoietic Neoplasms Coding Best Practices 14 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. Hematopoietic Neoplasms Coding Best Practices 15 Hematopoietic Neoplasms Coding Best Practices 5
6 Using the Rules: Four Steps 1. Is it reportable? (Reportability Instructions) 2. How many primaries do I abstract? (M Rules) 3. How do I code the primary site and histology? (PH Rules) 4. How do I code the grade? (Grade Rules) Hematopoietic Neoplasms Coding Best Practices 16 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 Hematopoietic Neoplasms Coding Best Practices 17 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. Hematopoietic Neoplasms Coding Best Practices 18 Hematopoietic Neoplasms Coding Best Practices 6
7 Reminder: Distinguishing Acute vs. Chronic Neoplasms Follicular lymphoma (chronic) Diffuse large B-cell (acute) Hematopoietic Neoplasms Coding Best Practices 19 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 Hematopoietic Neoplasms Coding Best Practices 20 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 Hematopoietic Neoplasms Coding Best Practices 21 Hematopoietic Neoplasms Coding Best Practices 7
8 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. Hematopoietic Neoplasms Coding Best Practices 22 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. Hematopoietic Neoplasms Coding Best Practices 23 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. Hematopoietic Neoplasms Coding Best Practices 24 Hematopoietic Neoplasms Coding Best Practices 8
9 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/6 th digit) codes Hematopoietic Neoplasms Coding Best Practices 25 Reminder: Appendix C Lymph Node Chain Reference Table Alphabetic order ICD-O-3 code ICD-O-3 lymph node region AJCC lymph node region Term ICD-O Code Cloquet s node C77.4 Inguinal region or leg Colic NOS, ileocolic, mesocolic, middle (right) Hematopoietic Neoplasms Coding Best Practices 26 Use with PH rules to determine whether involved nodes are in a single or multiple regions ICD-O-3 LN Region AJCC LN Region Inguino-femoral, right and left* C77.2 Intra-abdominal Mesenteric Common bile duct C77.2 Intra-abdominal Para-aortic Cubital C77.3 Axilla or arm Axillary, right and left* Cystic duct C77.2 Intra-abdominal Para-aortic Reminder: Read the Notes in DB! Hematopoietic Neoplasms Coding Best Practices 27 Hematopoietic Neoplasms Coding Best Practices 9
10 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 Hematopoietic Neoplasms Coding Best Practices 28 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 Hematopoietic Neoplasms Coding Best Practices 29 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 Hematopoietic Neoplasms Coding Best Practices 30 Hematopoietic Neoplasms Coding Best Practices 10
11 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 Hematopoietic Neoplasms Coding Best Practices 31 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 Hematopoietic Neoplasms Coding Best Practices 32 Heme Coding Case MRI bone - susp mets in L5 vert body w/ path fracture; abnormal soft tiss enhancement in epidural space from S1 through L4 ; bone biopsy- L5 - atypical lymphoid infilt; Case submitted to TCR as primary unk, no micro confirm, malig nos Hematopoietic Neoplasms Coding Best Practices 33 Hematopoietic Neoplasms Coding Best Practices 11
12 Heme Coding Case 1 9/15/15 65yowf found to have elevated globulins 9/15/15 Bone bx L5 atypical lymphoid infiltrate 9/19/15 BM bx monoclonal plasma cell population, lambda restricted plasma cells c/w plasma cell neoplasm Comment: Most consistent with Multiple Myeloma 9/19/15 CT c/a/p lymphadenopathy L axilla, R hilum, retroperitoneum, and pelv How many primaries? Primary site #1 Primary site #2 Histology #1 Histology #2 Dx Confirm Hematopoietic Neoplasms Coding Best Practices 34 Heme Coding Case 1 Histology: Multiple Myeloma Rx: 10/1/15-10/2/15 Bendamustine; 10/1/15 Rituxan; 10/1/15-10/2/15 Dexamethasone 10/29/15 Vincristine Chemo code Hormone code Immuno code Other Rx code Hematopoietic Neoplasms Coding Best Practices 35 Heme Coding Case Bilateral br US: no susp les in either br; 2 lym nodes ID in R axilla, larger measures 7 x 13 x 16 mm. Total effacement of central echogenic fat slightly susp. Sampling advised. No axil adenopathy on l. 4/11/15 66 WDF prs w/lump in the axilla. She has a remote hx of br ca about 18 yrs ago. She has a chronic L axillary node that has been previously noted on US and found to be fatty replaced R axil LN, US core bx: chronic lymphocytic leukemia/sm lymphocytic lymphoma; no areas of lg cell transformation ID Hematopoietic Neoplasms Coding Best Practices 36 Hematopoietic Neoplasms Coding Best Practices 12
13 Heme Coding Case 2 Case submitted to TCR as prim site: R axillary LN (C779); Histology: sm lymphocytic lymphoma (9670/3); Staging: Not stagable Chemo: None here - pt went elsewhere How many primaries? Primary site #1 Primary site #2 Histology #1 Histology #2 Dx Confirm Hematopoietic Neoplasms Coding Best Practices 37 Heme Coding Case 3 2/12/ Oncology Note: Clinical diagnosis is myeloproliferative neoplasm, possibly polycythemia vera, essential thrombocythemia or primary myelofibrosis. 02/05/ Bone marrow biopsy: Final Diagnosis: Probable myeloproliferative neoplasm, polycythemia vera, essential thrombocythemia, or primary myelofibrosis. Addendum Diagnosis: JAK 2 Negative. How many primaries? Primary site #1 Primary site #2 Histology #1 Histology #2 Dx Confirm Hematopoietic Neoplasms Coding Best Practices 38 Case source: SEER*Educate Heme Coding Case 4 01/01/ Left posterior iliac bone marrow aspiration: Final Diagnosis: Acute lymphoblastic leukemia. Flow cytometry: Precursor B-cell lymphoblastic leukemia (ALL). Blasts mark CD10, CD20 and CD34 positive. Cytogenetics: Abnormal results: 46,XX,t(9;22)(q34;q11.2)[4]/46,XX[16]. How many primaries? Primary site #1 Primary site #2 Histology #1 Histology #2 Dx Confirm Hematopoietic Neoplasms Coding Best Practices 39 Case source: SEER*Educate Hematopoietic Neoplasms Coding Best Practices 13
14 Any Questions? Hematopoietic Neoplasms Coding Best Practices 40 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. Hematopoietic Neoplasms Coding Best Practices 41 Hematopoietic Neoplasms Coding Best Practices 14
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