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.
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.
2B00 Subcutaneous panniculitis-like T-cell lymphoma
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.
2B01 Mycosis fungoides
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.
2B02 Sézary syndrome
Definition of Primary cutaneous CD-30 positive T-cell lymphoproliferative disorders: Primary skin disorders characterised immunohistologically by infiltration by neoplastic CD30+ lymphocytes.
2B03 Primary cutaneous CD-30 positive T-cell lymphoproliferative disorders
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.
2B0Y Other specified primary cutaneous mature T-cell or NK-cell lymphomas and lymphoproliferative disorders
2B0Z Primary cutaneous T-cell lymphoma of undetermined or unspecified type
2B2Y Other specified mature T-cell or NK-cell neoplasms
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.
2B2Z Mature T-cell or NK-cell neoplasms unspecified
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.
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.
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
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.
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.
2B32 Immunodeficiency-associated lymphoproliferative disorders
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
2B33 Malignant haematopoietic neoplasms without further specification
2B33.0 Acute leukaemia not elsewhere classified
2B33.1 Myeloid leukaemia
2B33.2 Chronic myeloid leukaemia not elsewhere classified
2B33.3 Lymphoid leukaemia not elsewhere classified
2B33.4 Leukaemia unspecified
2B33.5 Malignant lymphoma not elsewhere classified
2B33.Y Other malignant haematopoietic neoplasms without further specification
2B3Z Neoplasms of haematopoietic or lymphoid tissues unspecified
Definition of Disorders Of The Thyroid Gland Or Thyroid Hormones System: Disorders due to dysfunction…
Definitions of Certain Disorders Involving The Immune System: Disorders in which disturbed immune regulation plays…
Coded Elsewhere: Immunodeficiencies with predominantly antibody defects (4A01.0)Combined immunodeficiencies (4A01.1)Defects in the complement system (4A00.1)Diseases…
Definition of Allergic Or Hypersensitivity Conditions: Allergy is a hypersensitivity reaction initiated by a proven…
Coded Elsewhere: Schnitzler syndrome (EB03) Monogenic autoinflammatory syndromes Definition of Monogenic autoinflammatory syndromes: Monogenic hereditary…
Coded Elsewhere: Rheumatoid arthritis (FA20) Lupus erythematosus Definition of Lupus erythematosus: An autoimmune non-organ specific…