Site specific - Haematological

In order to ensure that all the data items can be collected it is essential to discuss the process with clinicians responsible for treating the patients.

Note:

• for all haematological patients it is essential to record the ICD-O-3 Morphology Code (see Core data set)

ICD codes and WHO disease groups

Please refer to Appendix section of this user guide to find:

• appendix A and B for site specific registerable ICD codes for Haematological cancer patients
• appendix C for the full list of ICD10 codes which are applicable for Haematological diagnoses mapped against the relevant ICD-O-3 codes, as well as the data set which should be completed for each disease and the WHO Disease Group

Retired data items

The following data item has been retired from v10:

• Cancer Care Plan:
  • HA8210 - Splenomegaly Indicator (CLL)
  • HA8320 - Number of Abnormal Nodal Areas (Follicular)
  • HA8320 - Number of Abnormal Nodal Areas (DLBCL)
  • HA8330 - Primary Extranodal Site (Hodgkin Lymphoma)
  • HA8270 - Extramedullary Disease (ALL)
• Laboratory Results:
  • HA9200 - European Leukaemia Net (ELN) Genetic Risk (AML)
• Diagnosis:
  • CT7160 - FAB Classification
• Molecular and Biomarker:
  • CT6260 - ALK FUSION STATUS FOR ALCL

TNM Staging is not collected for Haematological cancers. However, the following staging data items are required for all relevant cases:

• CLL – Binet stage
• Myeloma – R-ISS stage
• All Lymphomas – Ann Arbor Stage
  • including if known, Ann Arbor Symptoms, Ann Arbor Extranodality, Ann Arbor Bulk and Ann Arbor Splenic Involvement

The following three data items are CTYA Only, to help complete the ‘Toronto Childhood Staging System’ international benchmarking project:

• Non Hodgkin Lymphoma – Murphy (St Jude) Stage
• Acute Lymphoblastic Leukaemia – Children’s Oncology Group (COG) Staging System Stage
• Acute Myeloid Leukaemia – Central Nervous System Involvement

Additionally, the following prognostic indicators are also required:

• CML – Sokal Index
• Myelodysplasia: IPSS-R
• Follicular lymphoma: FLIPI2 Index
• DLBCL – (R)IPI Index Score
• Nodal T-Cell Lymphoma – IPI Index Score
• Mantel Cell – MIPI Index Score
• Hodgkin Lymphoma – Hasenclever Index (Only applicable to advanced Stage 3 and 4 disease)

Lymphoblastic lymphoma and lymphoblastic leukaemia are now known to be the same entity. This is reflected in the latest ICD-O-3 coding update which assigns the same morphology code to both and uses the combined term 'lymphoblastic leukaemia/lymphoma'.

Historically different codes were assigned to lymphoblastic lymphoma and leukaemia and ICD10 coding still distinguishes between these 2 groups. The coding list below therefore retains the separate ICD10 codes (C83.5 and C91.0) but assigns the same ICD-O-3 codes to each pair of diseases. (Further detail can be provided if required).

The aim is to register patients presenting with symptoms referable to an underlying diagnosis of amyloidosis in the absence of a known, registerable plasma cell or lymphoid neoplasm.

Amyloidosis may be associated with plasma cell neoplasms such as multiple myeloma, other B cell neoplasms (such as lymphoplasmacytic lymphoma), or with paraproteinaemias (which are not associated with identified myeloma or lymphoma (for example MGUS).

If amyloidosis is identified in association with a registerable condition (such as multiple myeloma, plasmacytoma, lymphoplasmacytic lymphoma, Waldenstroms macroglobulinaemia), only the data for the associated registerable condition should be submitted through COSD and this will be registered as a new diagnosis by the cancer registries. Amyloidosis should not be submitted for COSD in these circumstances.

Amyloid deposition associated with chronic infection, medullary carcinoma of the thyroid, insulinoma, prolactinoma, Alzheimer disease, prion diseases and other non-AL types of amyloid, is considered to be secondary amyloidosis and should not be submitted for COSD.

If amyloidosis is identified in the absence of a registerable condition or before the identification of a registerable condition, then data for Primary Amyloidosis* should be submitted for COSD and this will be registered as a new diagnosis by the cancer registries.

Note:

• for the purpose of COSD, MGUS (monoclonal gammopathy of unknown significance) is not a registerable disease and therefore amyloidosis associated with a paraprotein/MGUS should be submitted for COSD and will be registered as a new diagnosis

Amyloidosis as identified above should be recorded for COSD and coded as follows:

• ICD10 code: E85.9 (Amyloidosis unspecified)
• ICD-O-3 morphology code: M9769/1

Primary Amyloidosis is composed of abnormal immunoglobulin light chains (or rarely heavy chains) which deposit (either intact or in fragments) in various tissues. These form B-pleated sheets (AL amyloid) that bind Congo Red dye with characteristic birefringence.

In Appendix I, you will find a proforma that shows which of the site specific data items are applicable to each haematological diagnosis group.

This can be used as a tool (by the clinical team) during MDT, to ensure capture of all relevant data items and to help the MDT coordinator input the clinically agreed data.

This proforma in PDF format, as well as an associated guidance document, is available for download from the Appendix section of this user guide. 

Notes:

• this data set has been separated into 2 sub sections ‘Haematology’ and ‘CTYA’
• this will make allocating and recording data on both subgroups easier

This section contains all the data items that would be expected to be available at the MDT, where the care plan was agreed. This is a child of 'Core – Cancer Care Plan'.

Cautionary note:

• the choice order and selections within each choice may have changed between v9 to v10

Haematological - Cancer care plan choice - Choice 0..1

Haematological - Cancer care plan choice - Choice 1

End of haematological - Cancer care plan choice - Choice 1

Haematological - Cancer care plan choice - Choice 2

End of haematological - Cancer care plan choice - Choice 2

Haematological - Cancer care plan choice - Choice 3

End of haematological - Cancer care plan choice - Choice 3

Haematological - Cancer care plan choice - Choice 4

End of haematological - Cancer care plan choice - Choice 4

Haematological - Cancer care plan choice - Choice 5

End of haematological - Cancer care plan choice - Choice 5

Haematological - Cancer care plan choice - Choice 6

End of haematological - Cancer care plan choice - Choice 6

Haematological - Cancer care plan choice - Choice 7

End of haematological - Cancer care plan choice - Choice 7

End of Haematological - Cancer Care Plan Choice

Sokal Index (Chronic Myeloid Leukaemia)

Where applicable, this is a mandatory data item Index derived from age, spleen size, platelet count, myeloblasts %.

Note:

• You can use an online version of the Sokal Index Calculator.

IPSS-R (Myelodysplasia)

Where applicable, this is a mandatory data item. The Revised International Prognostic Scoring System (IPSS-R) for Myelodysplastic Syndromes Risk Assessment Calculator is derived from Haemoglobin, Absolute Neutrophil Count, Platelets and Bone Marrow Blasts as:

• Haemoglobin (g/dL) [4-20] – A possible conversion for Hb values:
  10 g/dL= 6.2 mmol/L, 8 g/dL= 5.0 mmol/L
• Absolute Neutrophil Count (x109/L) [0-15]
• Platelets (x109/L) [0-2000]
• Bone Marrow Blasts (percent) [0-30]
• Cytogenetic Category

Notes:

• You can use an online version of the IPSS-R scoring system
• format has changed from n1.n1 to n2.n1 in v10

FLIPI 2 Index Score

Where applicable, this is now a mandatory data item in v10. Follicular Lymphoma International Prognostic Index 2 Score (FLIPI2), derived from age, Serum beta 2 microglobulin, bone marrow involvement, longest diameter of largest involved node and Haemoglobin.

Note:

• You can use an online version of the Follicular Lymphoma Prognostic Index 2 Score (FLIPI2) calculator.

Primary Extranodal Site

Site of origin of lymphoma if believed to be outside lymph nodes as agreed by MDT based on clinical and radiological findings. This is only required for the following 2 types: DLBCL.

Number Of Extranodal Sites Code

Number of sites with Lymphoma outside lymph nodes (clinical assessment).

(R)IPI Index Score

Revised International Prognostic Index Score, derived from Age, performance status (0-1 v 2-4), LDH, extranodal sites, Ann Arbor Stage.

Notes:

• You can use an online version of the (R)IPI Index for DLBCL Score calculator
• this data item has moved to form a new choice section for all the international prognostic score index data items
• updated data item name, previously ‘(R)IPI Index for DLBCL Score’

Number of Abnormal Nodal Areas

Number of abnormal nodal areas detected clinically and radiologically; this is only required for early stage Hodgkin Lymphoma.

Hasenclever Index

Hasenclever Index is only required for lymphomas with Ann Arbor Stage 3 or 4. Index derived from age, gender, Hb, Albumin, white blood count, Lymphocyte count, Ann Arbor.

Note:

• You can use an online version of the Hasenclever Index calculator.

IPI Index Score

This is a new data item for v10. The International Prognostic Index Score (IPI) for nodal T-cell lymphoma (PTCL-NOS, angioimmunoblastic and anaplastic large cell lymphoma [both ALK+ and ALK-) derived from Age, performance status (0-2 v 3-4), LDH, extranodal sites, Ann Arbor Stage

Note:

• You can use an online version of the IPI Index calculator

MIPI Index Score

This is a new data item for v10. The Mantle Cell International Prognostic Index Score (MIPI), derived from Age, ECOG performance status, LDH, and total white cell count.

Note:

• You can use an online version of the MIPI Index score calculator

This section contains site specific stage data items that would be expected to be available at the MDT. All site specific staging fields are mandatory and a child of ‘CORE – Site Specific Staging’ section, and together mandates the collection of:

• the date the sample was taken which provided a positive site specific stage outcome or the MDT where the stage was agreed
• the organisation who carried out the stage
  • this is a ‘required’ data item from v10, but important to collect if known
• the stage itself

Cautionary notes:

• the choice order and selections within each choice have changed between v9 to v10
• the following data items form a 6-choice menu and if applicable, one of the following ‘Site Specific Staging’ Sections MUST be provided per submission

Haematological - Site Specific Staging Choice 1..1
Haematological - Site Specific Staging - Choice 1

End of Haematological - Site Specific Staging - Choice 1
Haematological - Site Specific Staging - Choice 2

End of Haematological - Site Specific Staging - Choice 2

Haematological - Site Specific Staging - Choice 3

End of Haematological - Site Specific Staging - Choice 3
Haematological - Site Specific Staging - Choice 4

End of Haematological - Site Specific Staging - Choice 4

Haematological - Site Specific Staging - Choice 5

End of Haematological - Site Specific Staging - Choice 5

Haematological - Site Specific Staging - Choice 6

End of Haematological - Site Specific Staging - Choice 6

End of Haematological - Site Specific Staging Choice

Ann Arbor Stage

Where applicable, this is a mandatory data item. Staging is based on location of detected disease.

Binet Stage

Where applicable, this is a mandatory data item. Applicable to Chronic Lymphocytic Leukaemia (CLL). Prognostic index derived from platelet count, Hb, lymphadenopathy, hepatomegaly, and splenomegaly. Note that immune cytopenias are not included when calculating the Stage (such as if Platelet count is below 100 and/or Haemoglobin levels are below 110 as a result of immune cytopenia). Also, please see note on calculations below.*

Binet Stage “solely rely on physical examination and standard laboratory tests, and do not require ultrasound, computed tomography, or magnetic resonance imaging.”

Notes on Binet Stage calculations*:

• Platelet count >99 is more fully described as Platelet count >99x109/l
• Hb >99 is more fully described as Hb>99 g/L
• you can use the online version of the BINET calculator for CLL

R-ISS Stage for Myeloma

Where applicable, this is a mandatory data item. The Revised International Staging System (R-ISS) includes variables included in the original ISS (serum beta-2 microglobulin and serum albumin), while also including the additional prognostic information obtained from serum LDH and high-risk chromosomal abnormalities detected by interphase fluorescent in situ hybridization (iFISH) after CD138 plasma cell purification.

The revised (R-ISS for Myeloma) stages are as follows:

You can use an online version of the R-ISS calculator.

Murphy (St Jude) Stage

Where applicable, this is a mandatory data item. The St. Jude Children's Research Hospital model (Murphy Staging), which separates patients on the basis of limited versus extensive disease. More details are available on the National Cancer Institute website.

It is essential to record the disease specific stage for this group of patients. This information should be available to the MDT.

The following definitions are used:

Stage 1 – disease is limited to a single tumour or to one lymph node group (for example, neck, axilla, groin) outside of the abdomen or mediastinum.

Stage 2 – disease is limited to one tumour with local lymph node involvement, to 2 or more tumours or lymph node groups on the same side of the diaphragm, or to a completely resected primary tumour of the gastrointestinal tract with/without involvement of local lymph nodes

Stage 3 – disease includes tumours or lymph node groups involved on both sides of the diaphragm, any primary intrathoracic tumour (mediastinal, pleural or thymic disease), or extensive NHL within the abdomen; or any paraspinal or epidural tumours.

Stage 4 – disease involves the bone marrow and / or central nervous system (CNS), with/without other sites of involvement. Bone marrow involvement in NHL is defined as >5% - <25% malignant cells in an otherwise normal bone marrow. (> 25% malignant cells in the bone marrow is defined as leukaemia).

Children’s Oncology Group (COG) staging system stage

This is a new data item for v10. Where applicable, this is a mandatory data item and has been included in COSD to help complete the ‘Toronto Childhood Staging System’ international benchmarking project.

Additional national code definition notes:

• CNS1:
  • no clinical signs of CNS involvement and no blasts in CSF
• CNS2:
  • no clinical signs of CNS involvement and blasts in CSF and either: WBC <5/μL CSF or WBC ≥5/μL CSF and RBC ≥10/μL CSF and WBC/RBC in CSF ≤2x WBC/RBC in blood
• CNS3:
  • clinical signs of CNS involvement or Blasts in CSF and WBC ≥5/μL CSF and either: RBC <10/μL CSF or RBC ≥10/μL CSF and WBC/RBC in CSF >2x WBC/RBC in blood

Explanatory abbreviation notes:

• CNS    Central Nervous System
• CSF     Cerebrospinal fluid
• WBC   White Blood Cells
• RBC    Red Blood Cells

Central Nervous System Involvement

This is a new data item for v10. Where applicable, this is a mandatory data item and has been included in COSD to help complete the ‘Toronto Childhood Staging System’ international benchmarking project.

Additional national code definition notes:

• CNS Negative
  • lumbar puncture nontraumatic and no blasts in CSF and no clinical signs of CNS involvement
• CNS Positive
  • lumbar puncture traumatic or lumbar puncture nontraumatic and blasts in CSF or clinical signs of CNS involvement

These data are expected to be collected to support Ann Arbor Stage, although maybe submitted independently of the stage itself.
May be up to one occurrence per Primary Cancer Pathway (0..1)

Ann Arbor Symptoms

Additional stage designation based on presence or absence of specific symptoms.

Ann Arbor Extranodality

Additional staging designation based on extranodal involvement.

Additional notes:

• for Primary Nodal lymphoma, code "E" if there is involvement of a single extranodal site by contiguous spread (i.e. directly adjoining) from the known nodal group
• for Primary Extranodal lymphoma, code “E” if there is a single extranodal lesion with or without lymphatic involvement in the draining area (for example, a thyroid lymphoma with draining cervical lymph node involvement = “llE”)
• the designation of Stage 4 for nodal disease implies disseminated disease involving (distant) extranodal sites
• multiple extranodal deposits should be considered Stage lV and “E” should not be used - however, by convention, involvement of the bone marrow, liver, lung, pleura and CSF are always considered Stage 4 even if the disease is isolated to that organ

Ann Arbor Bulk

Additional staging designation based on presence of bulky disease for DLBCL and Hodgkin disease only.

Notes:

• DLBCL code X - if there are 1 or more lymph node masses >7.5cm
• Hodgkin lymphoma code “X” - if there is presence of bulky disease, that is, a nodal mass whose greatest dimension is more than 10 centimetres in size, and/or a widening of the mediastinum (middle chest) by more than one-third
• Speech marks “ ” have been removed from the description on the request of the NHS England, Data Model and Dictionary Service

Ann Arbor Splenic Involvement

Additional staging designation based on splenomegaly or normal spleen size with confirmed disease involvement.
Code "S" if either is true.

All data sets for Acute Lymphoblastic Leukaemia (ALL) now become age agnostic - you can duplicate them in a CTYA section. Adult and paediatric colleagues have agreed this collaboratively.

This section contains laboratory results data items that would be expected to be available at the MDT. This group is a child of CORE – Laboratory Results, and will mandate:

• the date the sample was reported
• the organisation who processed the sample

Cautionary note:

• the choice order and selections within each choice have changed between v9 to v10
• the following data items form a 4-choice menu and if applicable, one of the following sections MUST be provided per submission

Haematological - Laboratory Results Choice 0..1
Haematological - Laboratory Results - Choice 1

End of Haematological - Laboratory Results - Choice 1
Haematological - Laboratory Results - Choice 2

End of Haematological - Laboratory Results - Choice 2
Haematological - Laboratory Results - Choice 3

End of Haematological - Laboratory Results - Choice 3

Haematological - Laboratory Results - Choice 4

End of Haematological - Laboratory Results - Choice 4

End of Haematological - Laboratory Results Choice

Bone Marrow Blasts

Blast cells in bone marrow aspirate as percentage of all nucleated cells. Normally taken from laboratory report on diagnostic bone marrow.
(%) Range 0 - 100

Cytogenetics Subsidiary Comment

Description of cytogenetic findings.

Cellularity

Percentage value of Cellularity, (%) Range 0 to 100.

DEB Test

Record the outcome of DEB Test.

Dysplastic Haemopoiesis

Record if the bone marrow produced (Haemopoiesis) is Unilineage, Bilineage or Trilineages dysplastic.

White Blood Cell Count (Highest Pretreatment)

Highest White blood cell count pre-treatment (x10^₉ per litre). Normally provided by Haematological labs before transfusion/treatment.

Notes:

• range 0.0 to 999.9 (to 1dp)
• this has moved into new choice and has a new data item number as only required now for CTYA cases

Post Induction MRD

Where applicable, this is a mandatory data item. Percentage of leukaemic cells present at the end of Minimal Residual Disease (MRD) induction.

Note:

• a range has been added to the national code definitions of 2, 3, 4 and 5, to improve data quality and accuracy of reported data

These group of data items are specific to the CTYA diagnosis and should be available through the MDT, although the advice is to get clinical clarification and support if unsure.

Must be one occurrence if chosen per CORE - Diagnosis (1..1)
Haematological - Diagnosis – Choice 0..1
Haematological - Diagnosis - Choice 1
Start of Repeating Item - Mixed Phenotype Symptoms (At Diagnosis)

End of repeating item - Mixed Phenotype Symptoms (at Diagnosis)

End of Haematological - Diagnosis - Choice 1

Haematological - Diagnosis - Choice 2

End of Haematological - Diagnosis - Choice 2
Haematological - Diagnosis - Choice 3
Start of Repeating Item - Paediatric Myelodysplasia

End of Repeating Item - Paediatric Myelodysplasia
Start of Repeating Item - Underlying Disease Associated with MDS

End of Repeating Item - Underlying Disease Associated With MDS

Start of Repeating Item - Myelodysplasia Symptoms at Diagnosis

End of Repeating Item - Myelodysplasia Symptoms at Diagnosis
End of Haematological - Diagnosis - Choice 3
End of Haematological - Diagnosis - Choice

Mixed Phenotype Symptoms (at Diagnosis)

Record if any of the associated symptoms were present at Diagnosis, multiple symptoms can be submitted.

EGIL Score

The EGIL Score (European Group for the Immunological Classification of Leukaemia) assigns score points to major antigens to determine if certain lineage is present.

Paediatric Cytogenetic / Molecular Genetic Risk Group

Risk groups for ages 0 to 18 – cytogenetic and molecular genetic abnormalities.

AML Risk Factors

Record if any of these risk factors are present in a patient at diagnosis.

Paediatric Myelodysplasia

Record the Paediatric Myelodysplasia clinical findings at Diagnosis, multiple findings can be submitted.

Underlying Disease Associated with MDS

Record any underlying disease associated with MDS present at diagnosis, multiple underlying diseases can be submitted.

Congenital Anomalies

Record any Congenital Anomalies associated with the MDS at Diagnosis, multiple congenital anomalies can be submitted.

Myelodysplasia Symptoms at Diagnosis

Record any other Myelodysplasia symptoms present at diagnosis, multiple symptoms can be submitted.

May be up to one occurrence per Record (0..1)

Primary Induction Failure

Did the patient fail to achieve morphological remission after induction chemotherapy? This is a Haematological CYTA required data item.