2020 Changes
Released September 1, 2020

In this section:

Histology Changes

New histologies. These histologies can only be used for cases diagnosed 2021+:

  • 9715/3: Anaplastic large cell lymphoma, ALK-negative/ Breast implant-associated anaplastic large cell lymphoma
  • 9749/3: Erdheim-Chester Disease
  • 9766/3: Lymphomatoid granulomatosis grade 3
  • 9819/3: B-lymphoblastic leukemia/lymphoma, BCR-ALB1 like
  • 9877/3: Acute myeloid leukemia with mutated NPM1
  • 9878/3: Acute myeloid leukemia with biallelic mutation of CEBPA
  • 9879/3: Acute myeloid leukemia with mutated RUNX1
  • 9912/3: Acute myeloid leukemia with BCR-ABL1
  • 9968/3: Myeloid/lymphoid neoplasm with PCM1-JAK2
  • 9993/3: Myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia

The following histologies are new, but are /1 and not reportable. They have been included in the Hematopoietic Database for informational purposes:

  • 9591/1: Monoclonal B-cell lymphocytosis, non-CLL type
  • 9673/1: In situ mantle cell neoplasia
  • 9680/1: EBV-positive mucocutaneous ulcer
  • 9695/1: In situ follicular neoplasia
  • 9702/1: Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract
  • 9709/1: Primary cutaneous CD4-positive small/medium T-cell lymphoproliferative disorder (previously listed as an alternate name in 9709/3)
  • 9738/1: HHV8-positive germinotropic lymphoproliferative disorder
  • 9761/1: IgM monoclonal gammopathy of undetermined significance
  • 9823/1: Monoclonal B-cell lymphocytosis, CLL-type

Changes in Reportability

  1. The following histologies are now a /1 (instead of a /3) and are no longer reportable starting with 2021 diagnoses:
    • 9725/3: Hydroa vacciniforme-like lymphoma (New preferred name: Hydroa vacciniforme-like lymphoproliferative disorder)
      • Note: See 9725/1 for 2021+
    • 9971/3: Post-transplant lymphoproliferative disorder (PTLD)
      • Note: See 9971/1 for 2021+
  2. The following histology codes and terms are obsolete and have a new code starting with 2021 diagnoses:
    • 9826/3: Burkitt Leukemia (for diagnosis 2021+, coded as 9687/3 Burkitt lymphoma with primary site C421)
    • 99991/3: Refractory neutropenia (for diagnosis 2021+, coded as 9980: Myelodysplastic syndrome with single lineage dysplasia)
    • 99992/3: Refractory thrombocytopenia (for diagnosis 2021+, coded as 9980: Myelodysplastic syndrome with single lineage dysplasia)
  3. Change in histology 9751/3
    • Only Langerhans cell histiocytosis, disseminated is a /3 for 2021+ diagnoses. All other terminology, including Langerhans cell histiocytosis, NOS, is now a /1 (see updated alternate names list when "help me code for diagnosis" is 2021)

Coding Diagnostic Confirmation

  • Code 1: Added "includes peripheral blood smear"
  • Code 3: Added "includes peripheral blood smear followed by flow cytometry"
  • Code 5: Added Note 2: This does not include cases where a peripheral blood smear is done (code 1) and peripheral blood smear followed by flow cytometry (code 3)

Diagnostic Confirmation Coding Instructions for Hematopoietic and Lymphoid Neoplasms (9590/3-9993/3)

Code 1: Positive histology

4b: White blood count (WBC)

Note: A registrar may not abstract a hematopoietic neoplasm based on a CBC or WBC with abnormal counts alone. There must be a diagnosis of a reportable Heme neoplasm on the CBC or WBC report or a subsequent physician diagnosis based on the WBC or CBC.

Code 3: Positive histology PLUS positive immunophenotyping or genetic testing

Added 1c: Peripheral blood smear followed by flow cytometry (most commonly done with CLL/SLL, 9823/3)

Note: Flow cytometry studies are normally done based on an abnormal blood smear. If unable to find documentation that a peripheral blood smear was done first, assume that it was and code 3

Example: Peripheral blood flow cytometry report: Flow cytometry express HLA-DR, CD5, CD19, moderate CD20, CD22, bright CD45, bridght CD200 and exhibit lambda immunoglobin light chain restruction by intracellular staging. These cells lack expression of CD38. Taken together, these results demonstrate the presence of a clonal population of B-cell, immonphenotypically diagnostic of CLL/SLL

Code 5: Positive laboratory test/marker study

Note: Do not use this code when a peripheral blood smear is done (which qualifies for a code 1) or a peripheral blood smear followed by flow cytometry (which qualifies for a code 3). Flow cytometry studies are normally done based on an abnormal peripheral blood smear. If unable to find documentation that a peripheral blood smear was done first, assume that it was and code 3

New Section in 2021

  • Appendix D (New): Introduction to Genetic Nomenclature.

2019 Changes
Released January 22, 2019

  • Hematopoietic manual - histologies fixed in Module 6 and 7
  • User’s manual updated

2018 Changes
Released May 1, 2018

This list provides a brief summary of the major changes in the revised Hematopoietic and Lymphoid Neoplasm Coding Manual and Database released in May 2018.

In this section:

Hematopoietic and Lymphoid Neoplasm Coding Manual

Hematopoietic Database

  • Stand-alone version is no longer available.
  • Note added regarding Multiple Primaries Calculator (MPC):
    • The multiple primaries calculator (MPC) is to be used only when the rules instruct you to do so. Use of the MPC without applying the rules first may result in an incorrect number of primaries and/or histologies.

Diagnostic Confirmation Table

  • Following note added:
    • Note: Includes cases with positive immunophenotyping or genetic studies and no histological confirmation.

Diagnostic Confirmation Coding Instructions

  • Code 1: New Note 6: IHC studies are done, but the patient has a provisional (NOS) diagnosis or one or more provisional diagnoses.
  • Code 5: Note updated: Assign code 5 when the diagnosis of cancer is based on laboratory tests, tumor marker studies, genetics or immunophenotyping that are clinically diagnostic for that specific cancer. Laboratory tests are listed under Definitive Diagnostic Methods in the Hematopoietic Database. Do not assign code 5 when there is histologic confirmation (see code 3).

M Rules

  • M2: An exception has been added regarding MALT lymphomas.
    • Exception: Abstract multiple primaries when a nodal MALT (C770-779, 9699/3) occurs before or after an extranodal MALT (all other sites, 9699/3).
    • Note: These are two distinct lymphomas that have the same histology code.
      • Example: Marginal zone lymphoma (MALT) of right inguinal node (C774) diagnosed in 2013. Stage I with no recurrence. In March 2018, diagnosed with Stage III ocular marginal zone lymphoma. Abstract a new primary.
    • An exception has been added regarding Plasmacytomas and Plasma Cell Myeloma.
      • Exception for plasmacytoma (9731, 9734) and plasma cell myeloma (9732): This rule does not apply. The presence of the plasmacytomas and a diagnosis of plasma cell myeloma diagnosed at the same time (simultaneously) or during the initial workup, is evidence of advanced disease. Abstract one primary, plasma cell myeloma, 9732/3.
    • See M11 change above.

Primary Site Coding Instructions

  • C: Moved to D for cases diagnosed 1/1/2018 and forward.
    • Waldenstrom Macroglobulinemia (9761/3) now has primary site of C421 (bone marrow).

Primary Site and Histology Coding Rules (PH Rules)

  • Rule PH2

    • Multiple extraosseous (extramedullary) plasmacytomas and Multiple plasmacytomas removed.
    • New note 1 indicating the presence of multiple plasmacytomas is diagnostic of plasma cell myeloma (9732/3) and not plasmacytoma (9731, 9734).
      • Note 1: References to multiple areas of ICD-O-involvement has been removed from this rule. Per the WHO classification of Tumours of Haematopoietic and Lymphoid Tissues (October 2017), the presence of multiple plasmacytomas is diagnostic of plasma cell myeloma (9732/3).
  • Rule PH 3

    • Multiple medullary plasmacytomas, Multiple plasmacytomas, Multiple plasmacytomas of bone removed.
    • New note 1 indicating the presence of multiple plasmacytomas is diagnostic of plasma cell myeloma (9732/3) and not plasmacytoma (9731, 9734).
      • Note 1: References to multiple areas of ICD-O-involvement has been removed from this rule. Per the WHO classification of Tumours of Haematopoietic and Lymphoid Tissues (October 2017), the presence of multiple plasmacytomas is diagnostic of plasma cell myeloma (9732/3).
  • Rule PH 4

    • New note 1 indicating the presence of multiple plasmacytomas is diagnostic of plasma cell myeloma (9732/3) and not plasmacytoma (9731, 9734).
      • No change in histologies covered in this module.
      • Order of histologies included changed.
  • Module 6

  • Rule PH14

  • Rule PH16

  • Module 7

    • Title of Module changed to "Coding Primary Site."
    • No change in histologies covered in this rule.
    • Order of histologies included changed.
    • Note added regarding secondary involvement of lymphomas: Secondary involvement of distant lymph nodes (for an extranodal lymphoma), bone marrow, liver, multifocal lung, spleen or CNS are included in the stage fields only. This secondary involvement excludes rare primary lymphoid neoplasms of spleen, lung involvement, liver or CNS (see PH Rules). Secondary involvement of distant site(s) is disregarded for the purpose of coding primary site. For lymphoid neoplasms, this secondary or distant involvement is akin to metastasis for solid tumors and does not alter the primary site assigned by the physician or determined using the PH Rules.
  • Rule PH18

    • Note indicating that other types of "mass" are not included in this rule, only those that are listed.
      • Note 1: This rule does not apply to other descriptions of "mass." For example, a "mass" in the neck is likely describing cervical lymph node involvement and does not meet the criteria for this rule.
  • Rule PH22

    • Clarification added for this rule: there is only distant lymph node involvement, no presence of regional lymph node involvement.
    • An organ and lymph nodes that are not regional (distant lymph nodes only, no regional lymph node involvement) for that organ and the origin of the lymphoma cannot be determined even after consulting the physician OR
    • Note added: Do not use this rule for extraosseous plasmacytomas (9734/3).
    • Reference to Collaborative Stage removed.
    • Clarifications added to bulleted notes:
      • Multiple organs and the regional nodes for all involved organs OR
        • Note: Does not include distant involvement (e.g., bone marrow involvement).
      • Multiple organs and some combination of regional and distant nodes for the involved organs OR
      • Lymph node(s) and involved organ(s) and no primary site/particular lymph node region is identified.
        • Note: Use for history only or path only cases.
      • Lymph node(s) and no primary site/particular lymph node region is identified.
        • Note: Use when no other information is available.
  • Rule PH27

    • Note 1: If lymph nodes are involved, see Rule PH22.
    • Note 2: For Langerhans cell histiocytosis (9751/3), if there is no information available, assign primary site to bone, NOS (C419).

Grade of Tumor Rules

  • Starting with diagnosis date January 1, 2018, Grade is no longer applicable for Hematopoietic neoplasms.
  • Grade section is still applicable for cases diagnosed 2010-2017.

Appendices

  • Obsolete Terms as Defined in ICD-O-3:
    • Hematopoietic and Lymphoid Neoplasm
    • This section has been removed from the Hematopoietic manual. For historical cases only. Obsolete terms are included in the Hematopoietic Database.
  • Appendix B: WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues
  • Appendix C: Lymph Node/Lymph Node Chain Reference Table
    • Histology Lineage
    • This appendix also included in the Summary Stage 2018 manual.
  • Appendix D: New Histology Terms and Codes Hematopoietic and Lymphoid Neoplasms
    • This section has been removed. It was originally written for the 2010 changes, which are no longer new codes.
  • Appendix E: Obsolete Hematopoietic Neoplasm Codes
    • This section has been removed. Information is included in the Hematopoietic Database.
    • In 2015, all obsolete codes used for cases diagnosed 2010 and later, were converted to the current code.
    • Edits in place that will not allow use of the obsolete codes for cases diagnosed 2010 and forward.

Hematopoietic and Lymphoid Neoplasm Database

  1. Applicable ICD-O-1 and ICD-O-2 codes added.
  2. The following changes were made to various histologies based on the WHO Classification of Tumours of Haematopoietic and Lymphoid Neoplasms, Revised 4th Edition (2016).
    1. Changes in preferred name
    2. Alternate names added
    3. Updates to genetic and immunophenotyping
  3. Sources: Added to all histologies. Primary sources are the revised WHO blue book, ICD-O-3 and NCI website.
  4. Changes in same primaries:
    1. Many changes were done to same primaries (multiple primary calculator). These are due to errors found in the existing database.
  5. Grade: For diagnosis year of 2018, shown as "not applicable."
    1. Exception: For follicular lymphomas (9690, 9691, 99695, 9698) with ocular primary sites (C441, C690, C695-C696), grade is still applicable.
    2. See the SSDI Grade Manual for instructions.
    3. Based on AJCC 8th Edition: Chapter 71: Lymphoma Ocular Adnexa.
    4. Note: Grade is still applicable for cases diagnosed 2010-2017. Make sure correct diagnosis year is chosen.

Diagnosis Year Box and Grade Field for 2018

image displaying a select box with year 2018 selected

Diagnosis Year Box and Grade Field for 2017

image displaying a select box with year 2017 selected