Transcript Hematopoietic Diseases - University of Kentucky

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CSv2 for the Hematopoietic
Neoplasms
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This includes five schemas ….
• Hematopoietic, Reticuloendothelial,
Immunopro-liferative and Myeloproliferative
neoplasms
• Myeloma and Plasma Cell Disorders
• Lymphoma
• Lymphoma, Ocular Adnexa
• Mycosis fungoides and Sézary syndrome
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CS Data Items new in 2010 are NA
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Data Item
Code
Explanation
Grade Path Value
Blank
Not applicable
Grade Path System
Blank
Not applicable
Lymph vascular invasion
(LVI)
Mets at Dx-Bone
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Not applicable
8
Not applicable
Mets at Dx-Lung
8
Not applicable
Mets at Dx-Brain
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Not applicable
Mets at Dx-Liver
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Not applicable
Hematopoietic /
Reticuloendothelial Neoplasms
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Based primarily on histology
• 9733, 9740-9742, 9750-9758, 9760-9762, 9764-9769,
9800-9801, 9805-9809, 9811-9818, 9820, 9823 [C420,
C421, or C424 ONLY], 9826, 9827 [C420, C421, or
C424 ONLY], 9831-9837, 9840, 9860-9861, 9863,
9865-9867, 9869-9876, 9891, 9895-9898, 9910, 9920,
9930-9931, 9940, 9945-9946, 9948, 9950, 9960-9967,
9970, 9971, 9975, 9980, 9982-9987, 9989, 9991-9992
• Note: AJCC does not define TNM staging for this site.
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Heme Retic Schema
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CS Extension
Description
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Localized disease: (single/solitary/unifocal/isolated)
may be coded for:
Mast cell sarcoma (9740)
Malignant histiocytosis (9750)
Langerhans cell histiocytosis (9751)
Histiocytic sarcoma (9755)
Langerhans cell sarcoma (9756)
Dendritic cell sarcoma (9757, 9758)
Myeloid sarcoma (9930)
800
Systemic disease
All histologies including those in 100
999
Unknown, cannot be assessed, not documented in record
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Remaining CS Data Elements
• All these fields are “not applicable”
▫ Tumor size=988
▫ CS Lymph nodes=988
▫ Reg LNs positive and Reg LNs examined=99
▫ CS Mets at Dx=98
▫ CS Eval fields (TS/Ext, Reg Nodes, Mets)=9
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CS Site Specific Factor 1 - JAK-2
Code
Description
000
JAK-2 test result stated as negative
010
JAK2 test performed, positive for mutation V617F in exon 14
020
JAK2 test performed, positive for mutation of exon 12
800
JAK2 test performed, positive for other specified mutation
810
JAK2 test performed, positive for more than one mutation
850
JAK2 test performed, positive NOS; specific mutation(s) not stated
888
OBSOLETE DATA CONVERTED V0200
See code 988: Not applicable for this schema
988
Not applicable: Information not collected for this case
(May include cases converted from code 888 used in CSv1. If this
item is required to derive T, N, M, or any stage, use of code 988 may
result in an error.)
997
Test ordered, results not in chart
998
Test not done
999
Unknown, Insufficient information, Not documented in record
Myeloma / Plasma Cell Disorders
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MyelomaPlasmaCell - Extension
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Myeloma – Lymph Nodes
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Myeloma – CS Mets at Dx – use 98
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Other CS fields Not applicable
• Tumor size/Extent Eval = 9
• CS Lymph Nodes Eval = 9
• CS Mets Eval
=9
• Regional Nodes Positive = 99
• Regional Nodes Examined = 99
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Myeloma – SSF1 JAK2 –Obsolete
Use only code 988
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Myeloma SSF2- Durie-Salmon Stage
• Note 1: Durie-Salmon staging applies to multiple
myeloma (9732) only. For plasmacytomas, (9731 and
9734), use code 987 for not applicable.
• Note 2: The A and B subclassifications reflect the
serum creatinine levels at diagnosis. If the A or B
subclassification is not noted, use the NOS codes.
• Note 3: Only code the Durie-Salmon stage when the
stage is documented in the medical record.
• Note 4: Code the stage as Durie-Salmon when the
record states the stage, but does not identify the
staging system.
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New: Myeloma – SSF2
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Myeloma SSF 3- MM Terminology
• Note 1: Descriptors for multiple myeloma (9732) only are
collected. Code 987, not applicable, for histologies 9731 and
9734.
• Note 2: A number of terms are used to characterize early
multiple myeloma at time of diagnosis. All these terms are
reportable according to the new Hematopoietic and
Lymphoid Neoplasms rules effective for cases diagnosed
January 1, 2010 and later.
• Note 3: Code the terminology used by a physician at the
time of diagnosis. The terminology may be taken from
sections of the record other than a pathology report. Do
not change the code in this Site Specific Factor if a term
used at a later time indicates progression to a more
aggressive disease course.
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New: Myeloma – SSF 3
Hodgkin and Non-Hodgkin
Lymphomas
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Lymphatic vs Extralymphatic sites
Lymphatic
Extralymphatic
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•
•
•
•
•
•
•
•
•
•
Lymph nodes (C77._)
Thymus (C37.9)
Spleen (C42.2)
Peyer’s patches (C17.2)
Nodules in the Appendix
(C18.1)
• Waldeyer’s Ring (C14.2)
▫ Pharyngeal tonsil
▫ Tubal tonsils
▫ Palatine tonsils
▫ Lingual tonsils
Stomach
Small Intestine
Gastrointestinal tract
Brain
Lung
Any organ can be involved by
lymphoma
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The Importance of the Diaphragm
• The diaphragm’s role in staging:
▫ The muscle that separates the chest (thoracic)
cavity from the abdomen.
▫ Is the dividing point for staging in lymphomas
(below and above, on both sides)
• The diaphragm is used in defining stage:
▫ Same side of the diaphragm
▫ Opposite side of the diaphragm
▫ Both sides of the diaphragm
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CS Extension Summary table
Side of
Diaphragm
Nodal/
Lymphatic
Extranodal/
Extralymphatic
Spleen
(involvement)
Same
100
110
120*
Same
200
210
220, 230
Both
300
310
320, 330
Both,
metastatic
800
800
800
Unknown
999
999
999
*Primary site=Spleen (C42.2), no other organs or lymph nodes involved
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CS Extension-Lymphatic
• 100 (Stage I)
▫ Primary site: Lymphatic
▫ Involvement of single lymph node region
• 200 (Stage II)
▫ Primary site: Lymphatic
▫ Two or more lymph node regions involved on
same side of diaphragm
• 300 (Stage III)
▫ Primary site: Lymphatic
▫ Lymph node regions involvement on both sides of
the diaphragm
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CS Extension-Extralymphatic
• 110 (Stage IE)
▫ Primary site: Extralymphatic organ
▫ Involvement of extralymphatic organ only
• 210 (Stage IIE)
▫ Primary site: Extralymphatic organ
▫ Lymph node involvement on same side of
diaphragm
• 310 (Stage IIIE)
▫ Primary site: Extralymphatic organ
▫ Lymph node involvement on both sides of
diaphragm
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CS Extension-Spleen
• 120 (Stage IS)
▫ Primary site: Spleen ONLY
• 220 (Stage IIS)
▫ Primary site: Spleen or lymphatic structure
▫ Spleen involved
▫ Lymph nodes below the diaphragm involved
• 230 (Stage IIES)
▫ Primary site: Extralymphatic organ
▫ Spleen involved
▫ Extralymphatic organ below the diaphragm
involved
▫ Lymph nodes below the diaphragm involved/not
involved
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CS Extension-Spleen
• 320 (Stage IIIS)
▫ Primary site: Spleen or lymphatic structure
▫ Spleen involved
▫ Lymph nodes above diaphragm or on both sides
of diaphragm involved
• 330 (Stage IIIES)
▫ Primary site: Extralymphatic organ
▫ Spleen involved
▫ Lymph nodes involved on opposite or both sides
of the diaphragm
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CS Extension
• 800 (Stage IV)
▫ Lymphatic sites (including Spleen)
 Metastatic sites (Bone marrow, Liver, Lung)
▫ Extralymphatic sites
 Diffuse or disseminated (multifocal) involvement of
one or more extralymphatic sites with or without
lymph node involvement
 Involvement of isolated extralymphatic organ in
absence of involvement of adjacent lymph nodes, but
in conjunction with disease in distant sites
 Metastatic sites (Bone marrow, Liver, Lung)
• 999 (Unknown)
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CS TS/Ext Eval
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Remaining CS Data Elements
• All these fields are “not applicable”
▫ Tumor size=988
▫ CS Eval fields (Reg Nodes, Mets)=9
▫ CS Lymph nodes=988
▫ Reg LNs positive and Reg LNs examined=99
▫ CS Mets at Dx=98
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New Data Items
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Data Item
Code
Explanation
Grade Path Value
Blank
Not applicable
Grade Path System
Blank
Not applicable
Lymph vascular invasion
(LVI)
Mets at Dx-Bone
8
Not applicable
8
Not applicable
Mets at Dx-Lung
8
Not applicable
Mets at Dx-Brain
8
Not applicable
Mets at Dx-Liver
8
Not applicable
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SSF 1 and SSF 2
• SSF 1 Associated with HIV/AIDS
▫ If there is no mention of AIDS in the chart and no
evidence of HIV testing, code 999 for unknown
• SSF 2 Systemic symptoms at diagnosis
▫ Coding “none” if H&P, progress notes and
consults make no mention of B symptoms
▫ B symptoms
 Drenching Night sweats
 Unexplained fever (above 38 degrees C)
 Unexplained weight loss (greater than 10% loss of
body weight in last six months before admission)
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▫ Pruritis (alone it does not mean B symptoms)
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SSF 3-5
• SSF 3 International Prognostic Index (IPI)
▫ This SSF applies to B-cell lymphomas
▫ Additional categories added (stated as “low” risk,
stated as “intermediate” risk, etc.)
▫ Criteria have been listed, DO NOT figure out index,
only enter what has been documented
• SSF 4 Follicular Lymphoma International
Prognostic Index (FLIPI)
• SSF 5 International Prognostic Score (IPS)
▫ Used with Hodgkin Lymphoma
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Lymphoma, Ocular Adnexa
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Mycosis fungoides
Sézary syndrome
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Histology
• Mycosis Fungoides (9700) is the most common
form of Cutaneous T cell lymphoma (CTCL)
• Sezary Syndrome (9701) is an aggressive leukemic
form of CTCL
• Different staging system from other CTCL due to
the prognosis of these diseases
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CS Extension
• 100 OBSOLETE (see codes 110-130)
• 110 Patches only with less than 10% of skin
surface involved
• 120 Plaques, with or without patches, with less
than 10% of skin surface involved
• 130 Plaques, papules, or eythematous patches
(“plaque stage”); Limited plaques/patches; MFCG
Stage I; Stated as T1
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CS Extension
• 200 OBSOLETE (see codes 210-240)
• 210 Patches only with more than 10% of skin
surface involved
• 220 Plaques, with or without patches, with more
than 10% of skin surface involved
• 230 Plaques, papules, or erythematous patches
(“plaque stage”); Generalized plaques/patches;
MFCG Stage II; Stated as T2
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CS Extension
• 250 Plaques, papules, or erythematous patches
(“plaque stage”); body surface not stated
• 300 Skin involvement, NOS
• 500 OBSOLETE
• 550 One or more tumors (tumor stage) less than 1
cm, cutaneous tumors, size not stated
• 600 One or more tumors (tumor stage) greater
than 1 cm
• 650 Stated as T3
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CS Extension
• 700 OBSOLETE
• 730 Generalized erythroderma (greater than 50%
and less than 80%)
• 750 Generalized erythroderma (greater than or
equal to 80%)
• 800 Stated as T4
• 950 No evidence of primary tumor
• 999 Unknown
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CS Lymph Nodes
• Lymph node staging is based on clinical and
pathologic factors
• Lymph node biopsies only recommended on
clinically abnormal nodes greater than 1.5 cm
• Clonality (negative, positive or unknown) will
determine a, b or NOS
• Dutch grade system and NCI LN grade system are
also included in lymph node definitions
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CS Mets at Dx
• Referred to as “Visceral Organ Involvement”
instead of mets
• 00 No visceral organ involvement
• 05 Clinical confirmation only of Visceral (non
cutaneous, extra nodal) involvement (except for
Liver and Spleen imaging, see code 10)
• 10 Imaging confirmation of Spleen or Liver
metastases
• 40 OBSOLETE
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CS Mets at Dx
• 45 Visceral (non cutaneous, extra nodal)
involvement, pathologically confirmed,
involvement by at least one organ outside the skin,
nodes or blood or bone marrow
• 60 Carcinomatosis; Distant mets, NOS; MFCG
Stage IV; Unknown if clinical or pathologic
confirmation
• 70 Stated as M1
• 99 Unknown
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New Data Items
Data Item
Code
Explanation
Grade Path Value
Blank
Not applicable
Grade Path System
Blank
Not applicable
8
Not applicable
Mets at Dx-Bone
0,1,9
Applicable
Mets at Dx-Lung
0,1,9
Applicable
Mets at Dx-Brain
0,1,9
Applicable
Mets at Dx-Liver
0,1,9
Applicable
Lymph vascular invasion (LVI)
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SSF1 Peripheral blood involvement
• A peripheral blood smear must be done to detect peripheral blood
involvement
• The categories for peripheral blood involvement are:
▫ B0 – no significant blood involvement
▫ B1 – Low blood tumor burden
▫ B2 – High blood tumor burden
• Clonality may also be assessed on blood samples.
• Record the B rating as stated by the physician. If counts or
percentages of neoplastic cells, flow cytometry test results, and/or
clonality test results are performed but a B rating is not stated by the
physician, use code 090.
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Summary remarks for this schema
• Mycosis fungoides/ Sézary syndrome
▫ Extension divides T1, T2 into ‘a’ and ‘b’ categories
based on whether you have patches or plaques
▫ Lymph nodes are divided into “a” and “b” categories
for N1, N2 based on clonality (negative vs positive)
▫ Peripheral blood involvement (SSF1) now a part of
TNM stage group and is further divided into “a” and
“b” categories for B0 and B1 based on clonality
(negative vs positive)
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