ICD 11

Mature T-Cell Or NK-Cell Lymphomas And Lymphoproliferative Disorders Primary Cutaneous Specified Types – Definitions & ICD 11 Codes


Definition of Mature T-Cell Or NK-Cell Lymphomas And Lymphoproliferative Disorders Primary Cutaneous Specified Types: Primary cutaneous T-cell lymphomas (CTCL) and NK-cell lymphomas are malignant lymphoproliferative diseases of unknown cause that are thought to originate from T-lymphocytes in the lymphoid tissue of the skin and by definition are confined to the skin at initial diagnosis. Included in the class is also a small number of lymphoma-like primary cutaneous lymphoproliferative disorders which are not considered to be truly malignant.

Inclusions:


  • Primary cutaneous peripheral T-cell lymphoma

Exclusions:

  • Skin infiltration by nodal or non-cutaneous extranodal lymphoma (2E08)

Subcutaneous panniculitis-like T-cell lymphoma

Definition of Subcutaneous panniculitis-like T-cell lymphoma: Subcutaneous panniculitis-like T-cell lymphoma is a neoplasm of alpha/beta, usually CD8+ T-cells, mainly confined to the subcutis, presenting clinically as subcutaneous nodules which are usually not ulcerated.

ICD 11 Codes For Subcutaneous panniculitis-like T-cell lymphoma

  2B00  Subcutaneous panniculitis-like T-cell lymphoma

Mycosis fungoides

Definition of Mycosis fungoides: A peripheral (mature) T-cell lymphoma presenting in the skin with patches/plaques or less commonly with tumours or erythroderma. It is characterised by epidermal and dermal infiltration of small to medium-sized T-cells with cerebriform nuclei.

ICD 11 Codes For Mycosis fungoides

  2B01  Mycosis fungoides

Sézary syndrome

Definition of Sézary syndrome: A generalised peripheral (mature) T-cell neoplasm characterised by the presence of erythroderma, lymphadenopathy, and neoplastic, cerebriform T-lymphocytes in the blood. Sézary syndrome is an aggressive disease.

ICD 11 Codes For Sézary syndrome

  2B02  Sézary syndrome

Primary cutaneous CD-30 positive T-cell lymphoproliferative disorders

Definition of Primary cutaneous CD-30 positive T-cell lymphoproliferative disorders: Primary skin disorders characterised immunohistologically by infiltration by neoplastic CD30+ lymphocytes.

ICD 11 Codes For Primary cutaneous CD-30 positive T-cell lymphoproliferative disorders

  2B03  Primary cutaneous CD-30 positive T-cell lymphoproliferative disorders

Inclusions:

  • Primary cutaneous CD30+large T-cell lymphoma

2B03.0 Primary cutaneous CD30 positive anaplastic large cell lymphoma

Definition of Primary cutaneous CD30 positive anaplastic large cell lymphoma: An anaplastic large cell lymphoma limited to the skin at the time of diagnosis. Most patients present with solitary or localised skin lesions, which may be tumours, nodules or papules. The t(2;5) translocation that is present in many cases of systemic anaplastic large cell lymphoma, is not found in this disease.

2B03.1 Lymphomatoid papulosis

Definition of Lymphomatoid papulosis: Lymphomatoid papulosis is a proliferation of T-cells, often clonal, characterised clinically by the appearance of crops of dome-shaped papules and nodules which tend to ulcerate and then heal with scarring.

Other specified primary cutaneous mature T-cell or NK-cell lymphomas and lymphoproliferative disorders

ICD 11 Codes For Other specified primary cutaneous mature T-cell or NK-cell lymphomas and lymphoproliferative disorders

  2B0Y  Other specified primary cutaneous mature T-cell or NK-cell lymphomas and lymphoproliferative disorders

Primary cutaneous T-cell lymphoma of undetermined or unspecified type

ICD 11 Codes For Primary cutaneous T-cell lymphoma of undetermined or unspecified type

  2B0Z  Primary cutaneous T-cell lymphoma of undetermined or unspecified type

Other specified mature T-cell or NK-cell neoplasms

ICD 11 Codes For Other specified mature T-cell or NK-cell neoplasms

  2B2Y  Other specified mature T-cell or NK-cell neoplasms

Mature T-cell or NK-cell neoplasms unspecified

Definition of Mature T-cell or NK-cell neoplasms unspecified: Malignant lymphomas, previously known as Hodgkin’s disease, characterised by the presence of large tumour cells in an abundant admixture of nonneoplastic cells. There are two distinct subtypes: nodular lymphocyte predominant Hodgkin lymphoma and classical Hodgkin lymphoma. Hodgkin lymphoma involves primarily lymph nodes.

ICD 11 Codes For Mature T-cell or NK-cell neoplasms unspecified

  2B2Z  Mature T-cell or NK-cell neoplasms unspecified

Hodgkin lymphoma

ICD 11 Codes For Hodgkin lymphoma

  2B30  Hodgkin lymphoma

2B30.0 Nodular lymphocyte predominant Hodgkin lymphoma

Definition of Nodular lymphocyte predominant Hodgkin lymphoma: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is characterised by a nodular, or a nodular and diffuse proliferation of scattered large neoplastic cells known as popcorn or lymphocyte predominant cells (LP cells) —formerly called L&H cells for lymphocytic and/or histiocytic Reed-Sternberg cell variants. At present an overlap between NLPHL and T-cell-rich large B-cell lymphoma cannot be excluded.

2B30.1 Classical Hodgkin lymphoma

Definition of Classical Hodgkin lymphoma: Classical Hodgkin lymphoma is a B-cell lymphoma characterised histologically by the presence of large mononuclear Hodgkin cells and multinucleated Reed-Sternberg (HRS) cells.

A monoclonal B-cell lymphoproliferation in the vast majority of cases. It is characterised by a bimodal age distribution (15-30 years of life and late life) and is often associated with EBV infection. In less than 5% of cases it is a monoclonal proliferation of T-lymphocytes. Morphologically, it is characterised by the presence of Reed-Sternberg cells and mononuclear Hodgkin cells. The Reed-Sternberg and mononuclear Hodgkin cells are CD30 positive in nearly all cases and CD15 positive in the majority of cases.

Inclusions:

  • Classical Hodgkin lymphoma, type not specified

2B30.10 Nodular sclerosis classical Hodgkin lymphoma

Definition of Nodular sclerosis classical Hodgkin lymphoma: A subtype of classical Hodgkin lymphoma characterised by collagen bands surrounding lymphoid nodules. The lymphoid nodules contain lacunar and Reed-Sternberg cells. Mediastinal involvement occurs in 80% of patients. The prognosis of nodular sclerosis Hodgkin lymphoma is slightly better than that of mixed cellularity or lymphocyte depleted subtype.

2B30.11 Lymphocyte-rich classical Hodgkin lymphoma

2B30.12 Mixed cellularity classical Hodgkin lymphoma

Definition of Mixed cellularity classical Hodgkin lymphoma: A subtype of classical Hodgkin lymphoma with a mixed inflammatory stroma containing Hodgkin and Reed-Sternberg cells.

2B30.13 Lymphocyte depleted classical Hodgkin lymphoma

2B30.1Z Classical Hodgkin lymphoma unspecified

2B30.Z Hodgkin lymphoma unspecified

Definition of Hodgkin lymphoma unspecified: True histiocytic malignancies are vanishing diagnoses due to improved understanding of the provenance of malignant cells.

Histiocytic or dendritic cell neoplasms

ICD 11 Codes For Histiocytic or dendritic cell neoplasms

  2B31  Histiocytic or dendritic cell neoplasms

2B31.0 Juvenile xanthogranuloma

Definition of Juvenile xanthogranuloma: It is characterised by the presence of lipid-laden, foamy histiocytes and Touton-type giant cells in the dermis.

2B31.1 Histiocytic sarcoma

Inclusions:

  • Malignant Histiocytosis

2B31.2 Langerhans cell histiocytosis

Definition of Langerhans cell histiocytosis: A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognised: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation.

Inclusions:

  • Histiocytosis X

2B31.20 Langerhans cell histiocytosis involving the skin

2B31.2Y Other specified Langerhans cell histiocytosis

2B31.3 Langerhans cell sarcoma

Definition of Langerhans cell sarcoma: A neoplastic proliferation of Langerhans cells with overtly malignant cytologic features. It can be considered a higher grade variant of Langerhans cell histiocytosis (LCH) and it can present de novo or progress from antecedent LCH.

2B31.4 Interdigitating dendritic cell sarcoma

Definition of Interdigitating dendritic cell sarcoma: A neoplastic proliferation of spindle to ovoid cells which show phenotypic features similar to those of interdigitating dendritic cells. The clinical course is generally aggressive.

2B31.5 Follicular dendritic cell sarcoma

Definition of Follicular dendritic cell sarcoma: A neoplasm composed of spindle to ovoid cells which have morphologic and immunophenotypic characteristics of follicular dendritic cells. It affects lymph nodes and other sites including the tonsils, gastrointestinal tract, spleen, liver, soft tissues, skin, and oral cavity. It usually behaves as a low grade sarcoma. Recurrences have been reported in up to half of the cases.

2B31.6 Indeterminate cell histiocytosis

Definition of Indeterminate cell histiocytosis: A very rare dendritic cell tumour composed of spindle to ovoid cells with a phenotype that is similar to the Langerhans cells. Patients usually present with cutaneous papules, nodules, and plaques. Systemic symptoms are usually absent. The clinical course is variable.

2B31.7 Fibroblastic reticular cell tumour

Definition of Fibroblastic reticular cell tumour: A very rare dendritic cell tumour affecting the lymph nodes, spleen, and soft tissues. Morphologically it is similar to the interdigitating dendritic cell sarcoma or follicular dendritic cell sarcoma. The tumour cells are positive for cytokeratin and CD68. Clinical outcome is variable.

2B31.Y Other specified histiocytic or dendritic cell neoplasms

2B31.Z Histiocytic or dendritic cell neoplasms unspecified

Definition of Histiocytic or dendritic cell neoplasms unspecified: Post-transplant lymphoproliferative disorder (PTLD) is a polyclonal (benign) or clonal (malignant) proliferation of lymphoid cells that develops as a consequence of immunosuppression in a recipient of a solid organ or bone marrow allograft. PTLDs comprise a spectrum ranging from early, Epstein-Barr virus (EBV)-driven polyclonal lymphoid proliferations to EBV-positive or EBV- negative lymphomas of predominantly B-cell or less often T-cell type. In other Immunodeficiency-associated lymphoproliferative disorders, association with EBV is less pronounced.

Immunodeficiency-associated lymphoproliferative disorders

ICD 11 Codes For Immunodeficiency-associated lymphoproliferative disorders

  2B32  Immunodeficiency-associated lymphoproliferative disorders

Inclusions:

  • PTLD – [Post transplant lymphoproliferative disorder]

2B32.0 Post-transplant lymphoproliferative disorder early lesion

Definition of Post-transplant lymphoproliferative disorder early lesion: A lymphoproliferative disorder arising as a result of post-transplant immunosuppression therapy. It is characterised by the lack of tissue destruction and the architectural preservation of the involved tissues. It includes two morphologic variants: plasmacytic hyperplasia and infectious mononucleosis-like lymphoproliferative disorders.

2B32.1 Reactive plasmacytic hyperplasia

2B32.2 Post-transplant lymphoproliferative disorder, Infectious mononucleosis-like

2B32.3 Polymorphic post-transplant lymphoproliferative disorder

2B32.Y Other specified immunodeficiency-associated lymphoproliferative disorders

2B32.Z Immunodeficiency-associated lymphoproliferative disorders unspecified

Malignant haematopoietic neoplasms without further specification

ICD 11 Codes For Malignant haematopoietic neoplasms without further specification

  2B33  Malignant haematopoietic neoplasms without further specification

Coding Note:

  • Only to be designated in cases with incomplete diagnostics.

2B33.0 Acute leukaemia not elsewhere classified

Coding Note:

  • Only to be designated in cases with incomplete diagnostics.

2B33.1 Myeloid leukaemia

2B33.2 Chronic myeloid leukaemia not elsewhere classified

Coding Note:

  • Only to be designated in cases with incomplete diagnostics

2B33.3 Lymphoid leukaemia not elsewhere classified

Coding Note:

  • Only to be designated in cases with incomplete diagnostics.

2B33.4 Leukaemia unspecified

Coding Note:

  • Only to be designated in cases with incomplete diagnostics.

2B33.5 Malignant lymphoma not elsewhere classified

Coding Note:

  • Only to be designated in cases with incomplete diagnostics.

2B33.Y Other malignant haematopoietic neoplasms without further specification

Coding Note:

  • Only to be designated in cases with incomplete diagnostics.

Neoplasms of haematopoietic or lymphoid tissues unspecified

ICD 11 Codes For Neoplasms of haematopoietic or lymphoid tissues unspecified

  2B3Z  Neoplasms of haematopoietic or lymphoid tissues unspecified

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