D47.Z2 ICD-10-CM Code: Castleman disease

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for D47.Z2 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2026Effective: October 1, 2025

Notes without a marker are published on D47.Z2 itself; “inherited from” names the category or block whose note applies here.

Coder workflow for D47.Z2

MedCoder structured workflow — derived from this code’s own official record

Before you code D47.Z2

1. Confirm the documented site and behavior (this code’s block classifies uncertain behavior neoplasms), whether the site is primary or secondary, and whether the malignancy is current or a personal history: a previously excised malignancy with no further treatment and no evidence of disease is history, not current disease. Verify the site against the Table of Neoplasms. Neoplasm chapter guidelines (Guidelines I.C.2.a, I.C.2.d, I.C.2.m).

   See the Table of Neoplasms entries · Guide: Active cancer vs history of cancer →

2. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with D47.Z2. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).

   See the official tabular notes

Choose the right path

1. Does the documentation support a condition named in D47.Z2’s Excludes1 note?
   Yes → Do not simply proceed: review the excluded code. Both are reported only when the record shows the two conditions are unrelated.
   No → Continue.

   ReviewD49

Consider D47.Z2. Then review the Code Also note, and confirm the code is valid for the date of service in the Verify section.

Documentation check

The provider’s diagnostic statement

Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).

Anatomical site

At the most specific level the record states; a site the classification separates cannot be assumed from a procedure or a measurement.

Behavior and current-versus-history status

Malignant, in situ, benign or uncertain; primary or secondary; current disease or personal history (Guidelines I.C.2.m).

Official instructions as workflow

• Excludes1 — check before selecting D47.Z2(1 note)

  Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with D47.Z2: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

  CompareD49

  See the official tabular notes · Guidelines I.A.12.a

• Excludes2 — not part of D47.Z2(1 note)

  Coding workflow: The conditions named in this note are not included in D47.Z2. When the record documents both, both may be reported; the note is a boundary, not a prohibition.

  CompareC46

  See the official tabular notes · Guidelines I.A.12.b

• Code Also — related condition(1 note)

  Coding workflow: Review the related condition when both are documented and the instruction applies. A Code Also note does not fix sequencing; the order follows the circumstances of the encounter.

  ReviewB10.89

  See the official tabular notes · Guidelines I.A.17

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.

Indexed Clinical Terms (2)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

• Disease, diseased, Castleman (unicentric) (multicentric)
• Disease, diseased, Castleman (unicentric) (multicentric), HHV-8-associated

Don’t confuse D47.Z2 with…

MedCoder analysis — pairs from this code’s official notes, distinctions written by MedCoder

The codes most likely to be picked instead of D47.Z2 — named in its own official notes, or sharing its laterality or encounter family — with what separates them and which documentation moves the case. Each links to the full comparison.

• D49— Excludes1 conflict (Excludes1 — generally not reported together)

  Not coded here — the tabular list excludes this pair for the same encounter.

  Not reported together with this code, except where the two conditions are documented as unrelated.

  What moves you there: Documentation of neoplasms of unspecified behavior moves the case to D49; the two are not reported together unless documented as unrelated.

  Full comparison: D47.Z2 vs D49 →

• C46— Excludes2 relationship (Informational)

  Distinct conditions — both may be reported.

  A distinct condition — both may be reported when both are documented.

  What moves you there: Documentation of Kaposi's sarcoma adds C46; it is not part of this code and both may be reported when both are documented.

  Full comparison: D47.Z2 vs C46 →

Relationships are read from this code's own official notes, its laterality siblings and its encounter family; the wording of each distinction is MedCoder editorial, and the documentation named in each “what moves you there” line is the official note phrase or sibling title itself. Source: CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a.

Decision Points

The directives on this code's own record, as a pre-claim checklist.

1. 1 Code Also note — a second code may apply; the guidelines leave its sequencing to the circumstances of the encounter. See the Code Also notes
2. 1 Excludes1 entry — codes named there are generally not reported together with this code (Guidelines I.A.12.a). See the Excludes1 notes
3. 1 Excludes2 entry — those conditions are not part of this code and may be reported additionally when documented. See the Excludes2 notes

Checklist rows are derived from this code's own official directives; the wording of each check is MedCoder editorial. The official notes themselves are in the sections each row links to.

Verify Before Coding

• CC as a secondary diagnosis (FY2026). Can raise the stay's MS-DRG severity tier.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder structured relationships — computed from published CMS and AHRQ datasets

MS-DRG Grouper Relationships (FY2026)

Potential MS-DRG participation — not a DRG assignment.

FY2026 MS-DRG: CC — Complication or Comorbidity. Reported as a secondary diagnosis, this code raises the stay's MS-DRG severity tier — except when the principal diagnosis is one of 133 clinically related codes on its CMS exclusion list.

Named in the grouper logic of 9 MS-DRGs: DRG 820 (MDC 17), DRG 821 (MDC 17), DRG 822 (MDC 17), DRG 823 (MDC 17), DRG 824 (MDC 17), DRG 825 (MDC 17), DRG 840 (MDC 17), DRG 841 (MDC 17), DRG 842 (MDC 17).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):NEO072 — Neoplasms of unspecified nature or uncertain behavior (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same clinical process (MS-DRG)

Acts as CC — raises the severity of other admissions. CMS groups these diagnoses into one MS-DRG principal-diagnosis exclusion process — a CC/MCC on this list never raises severity when the principal diagnosis is also on it.

D46.0 — Refractory anemia without ring sideroblasts, so stated, D46.1 — Refractory anemia with ring sideroblasts, D46.20 — Refractory anemia with excess of blasts, unspecified, D46.21 — Refractory anemia with excess of blasts 1, D46.22 — Refractory anemia with excess of blasts 2, D46.4 — Refractory anemia, unspecified, D46.9 — Myelodysplastic syndrome, unspecified, D46.A — Refractory cytopenia with multilineage dysplasia, D46.B — Refractory cytopenia with multilineage dysplasia and ring sideroblasts, D46.C — Myelodysplastic syndrome with isolated del(5q) chromosomal abnormality, D46.Z — Other myelodysplastic syndromes, D47.01 — Cutaneous mastocytosis, D47.02 — Systemic mastocytosis, D47.09 — Other mast cell neoplasms of uncertain behavior, D47.1 — Chronic myeloproliferative disease, D47.3 — Essential (hemorrhagic) thrombocythemia, D47.9 — Neoplasm of uncertain behavior of lymphoid, hematopoietic and related tissue, unspecified, D47.Z9 — Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue, D48.7 — Neoplasm of uncertain behavior of other specified sites, D48.9 — Neoplasm of uncertain behavior, unspecified, +112 more

Same CMS-HCC risk category (V28)

CMS maps these diagnoses to the same Hierarchical Condition Category (Lymphoma and Other Cancers) for risk-adjusted payment.

C88.41 — Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma], in remission, C88.80 — Other malignant immunoproliferative diseases not having achieved remission, C88.81 — Other malignant immunoproliferative diseases, in remission, C88.90 — Malignant immunoproliferative disease, unspecified not having achieved remission, C88.91 — Malignant immunoproliferative disease, unspecified, in remission, C96.0 — Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis, C96.20 — Malignant mast cell neoplasm, unspecified, C96.21 — Aggressive systemic mastocytosis, C96.22 — Mast cell sarcoma, C96.29 — Other malignant mast cell neoplasm, C96.4 — Sarcoma of dendritic cells (accessory cells), C96.5 — Multifocal and unisystemic Langerhans-cell histiocytosis, C96.6 — Unifocal Langerhans-cell histiocytosis, C96.9 — Malignant neoplasm of lymphoid, hematopoietic and related tissue, unspecified, C96.A — Histiocytic sarcoma, C96.Z — Other specified malignant neoplasms of lymphoid, hematopoietic and related tissue, D47.1 — Chronic myeloproliferative disease, D47.9 — Neoplasm of uncertain behavior of lymphoid, hematopoietic and related tissue, unspecified, D47.Z1 — Post-transplant lymphoproliferative disorder (PTLD), D47.Z9 — Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue, +442 more

Related risk categories

These categories interact through CMS's HCC hierarchy — one can suppress the other's risk-adjustment weight when both are present on a claim.

Cancer Metastatic to Lung, Liver, Brain, and Other Organs; Acute Myeloid Leukemia Except Promyelocytic, Cancer Metastatic to Bone, Other and Unspecified Metastatic Cancer; Acute Leukemia Except Myeloid, Myelodysplastic Syndromes, Multiple Myeloma, and Other Cancers, Lung and Other Severe Cancers, Bladder, Colorectal, and Other Cancers, Prostate, Breast, and Other Cancers and Tumors

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Neoplasms of unspecified nature or uncertain behavior).

D47.0 — Mast cell neoplasms of uncertain behavior, D47.01 — Cutaneous mastocytosis, D47.02 — Systemic mastocytosis, D47.09 — Other mast cell neoplasms of uncertain behavior, D47.1 — Chronic myeloproliferative disease, D47.2 — Monoclonal gammopathy, D47.3 — Essential (hemorrhagic) thrombocythemia, D47.4 — Osteomyelofibrosis, D47.9 — Neoplasm of uncertain behavior of lymphoid, hematopoietic and related tissue, unspecified, D47.Z1 — Post-transplant lymphoproliferative disorder (PTLD), D47.Z9 — Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue, D48.0 — Neoplasm of uncertain behavior of bone and articular cartilage, D48.1 — Neoplasm of uncertain behavior of connective and other soft tissue, D48.110 — Desmoid tumor of head and neck, D48.111 — Desmoid tumor of chest wall, D48.112 — Desmoid tumor, intrathoracic, D48.113 — Desmoid tumor of abdominal wall, D48.114 — Desmoid tumor, intraabdominal, D48.115 — Desmoid tumor of upper extremity and shoulder girdle, D48.116 — Desmoid tumor of lower extremity and pelvic girdle, +100 more

Lab tests where this diagnosis supports Medicare coverage (NCD)

Medicare's National Coverage Determination (NCD) program lists this diagnosis as medical justification for these lab tests.

Gamma-glutamyl Transferase (GGT) Test, HIV Screening Test, Iron Panel, Partial Thromboplastin Time (PTT) Test

Contextual Map

Every relationship of D47.Z2 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run D47.Z2 with these 2 related codes in Claim Check

Hierarchy

• C00-D49 — Chapter 2: Neoplasms (C00-D49) (C00-D49)[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• D37-D48 — Neoplasms of uncertain behavior, polycythemia vera and myelodysplastic syndromes[Hierarchy]— CMS ICD-10-CM tabular structure · icd10cm-fy2026

Excludes2

• C46 — Kaposi's sarcoma[Excludes2]: “Kaposi's sarcoma (C46.-)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026

Code Also

• B10.89 — Other human herpesvirus infection[Code Also]: “, if applicable, human herpesvirus 8 infection (B10.89)”— CMS ICD-10-CM tabular instructional notes · icd10cm-fy2026

Clinical classification (CCSR)

• NEO072 — Neoplasms of unspecified nature or uncertain behavior[CCSR]— AHRQ CCSR for ICD-10-CM Diagnoses (HCUP)

Risk adjustment (CMS-HCC)

• HCC 21 — Lymphoma and Other Cancers [CMS-HCC]: “Lymphoma and Other Cancers — supersedes HCC 22 (Bladder, Colorectal, and Other Cancers), HCC 23 (Prostate, Breast, and Other Cancers and Tumors)”— CMS-HCC V28 · 2026

MS-DRG Grouper (9)

• CC — Complication or Comorbidity [MS-DRG severity]: “As a secondary diagnosis this code can raise the stay's MS-DRG severity tier (CC).”— CMS MS-DRG Definitions Manual (Appendix C) · FY2026
• DRG 820 — LYMPHOMA AND LEUKEMIA WITH MAJOR O.R. PROCEDURES WITH MCC[MS-DRG]: “LYMPHOMA AND LEUKEMIA WITH MAJOR O.R. PROCEDURES WITH MCC (MDC 17)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• DRG 821 — LYMPHOMA AND LEUKEMIA WITH MAJOR O.R. PROCEDURES WITH CC[MS-DRG]: “LYMPHOMA AND LEUKEMIA WITH MAJOR O.R. PROCEDURES WITH CC (MDC 17)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• DRG 822 — LYMPHOMA AND LEUKEMIA WITH MAJOR O.R. PROCEDURES WITHOUT CC/MCC[MS-DRG]: “LYMPHOMA AND LEUKEMIA WITH MAJOR O.R. PROCEDURES WITHOUT CC/MCC (MDC 17)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• DRG 823 — LYMPHOMA AND NON-ACUTE LEUKEMIA WITH OTHER PROCEDURES WITH MCC[MS-DRG]: “LYMPHOMA AND NON-ACUTE LEUKEMIA WITH OTHER PROCEDURES WITH MCC (MDC 17)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• DRG 824 — LYMPHOMA AND NON-ACUTE LEUKEMIA WITH OTHER PROCEDURES WITH CC[MS-DRG]: “LYMPHOMA AND NON-ACUTE LEUKEMIA WITH OTHER PROCEDURES WITH CC (MDC 17)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• DRG 825 — LYMPHOMA AND NON-ACUTE LEUKEMIA WITH OTHER PROCEDURES WITHOUT CC/MCC[MS-DRG]: “LYMPHOMA AND NON-ACUTE LEUKEMIA WITH OTHER PROCEDURES WITHOUT CC/MCC (MDC 17)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• DRG 840 — LYMPHOMA AND NON-ACUTE LEUKEMIA WITH MCC[MS-DRG]: “LYMPHOMA AND NON-ACUTE LEUKEMIA WITH MCC (MDC 17)”— CMS MS-DRG Definitions Manual (Appendix B) · FY2026
• and 2 more

MDC crossing

• MDC 17 — Myeloproliferative Diseases and Disorders, Poorly Differentiated Neoplasms[MDC crossing]: “Myeloproliferative Diseases and Disorders, Poorly Differentiated Neoplasms — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 4,730 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2026

Index entries

• Disease, diseased, Castleman (unicentric) (multicentric)[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
• Disease, diseased, Castleman (unicentric) (multicentric), HHV-8-associated[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026

Nearest codes (13)

• D47 — Other neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• D47.0 — Mast cell neoplasms of uncertain behavior[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• D47.01 — Cutaneous mastocytosis[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• D47.02 — Systemic mastocytosis[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• D47.09 — Other mast cell neoplasms of uncertain behavior[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• D47.1 — Chronic myeloproliferative disease[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• D47.2 — Monoclonal gammopathy[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• D47.3 — Essential (hemorrhagic) thrombocythemia[Sibling]— CMS ICD-10-CM tabular structure · icd10cm-fy2026
• and 5 more

Change history

• FY2017 — Added to the code set[Change history]— CMS release files (code change ledger) · icd10cm-fy2017

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data

CMS/CDC ICD-10-CM FY2026 tabular list, index and tables, effective October 1, 2025 Release, file and checksum · Publisher’s page

Claim edits Official source data

CMS Definitions of Medicare Code Edits — v43.1 (April 2026) Release, file and checksum · Publisher’s page

Inpatient payment groups Official source data

CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v43 Release, file and checksum · Publisher’s page

Risk adjustment Official source data

2026 Mid-Year Final ICD-10 Mappings + Model Software (cms.gov/files/zip/2026-midyear-final-icd-10-mappings.zip, 2026-midyear-final-model-software.zip) — CMS-HCC V28, PY2026 mid-year final release Release, file and checksum · Publisher’s page

Change history and date-of-service validity Official source data

CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page

Comparisons, relationships and the contextual map MedCoder structured relationship

Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears

Summary and FAQ answers MedCoder explanation

Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Labels on this page: Official source data · MedCoder structured relationship · MedCoder explanation. How to read the labels · All data sources and release dates

Nearest Codes in This Family

Official ICD-10-CM classifications closest to D47.Z2 in its code family, with their registry titles.

• D47.02 — Systemic mastocytosis
• D47.09 — Other mast cell neoplasms of uncertain behavior
• D47.1 — Chronic myeloproliferative disease
• D47.2 — Monoclonal gammopathy
• D47.3 — Essential (hemorrhagic) thrombocythemia
• D47.4 — Osteomyelofibrosis
• D47.9 — Neoplasm of uncertain behavior of lymphoid, hematopoietic and related tissue, unspecified
• D47.Z — Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue
• D47.Z1 — Post-transplant lymphoproliferative disorder (PTLD)
• D47.Z9 — Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue

View all codes in the D47 family