Mature B-Cell Neoplasms – Definitions & ICD 11 Codes

Definition of Mature B-Cell Neoplasms: Non-Hodgkin lymphomas that originate from mature B lymphocytes. May reside in lymph nodes, lymphatic tissue of different organs or bone marrow and blood (then frequently called leukaemia).

Follicular lymphoma

Definition of Follicular lymphoma: Follicular lymphoma (FL) is a neoplasm composed of follicle centre (germinal centre) B-cells (typically both centrocytes and centroblasts/large transformed cells), which usually has at least a partially follicular pattern. t(14;18) with BCL2 rearrangement is frequently observed. If diffuse areas of any size comprised predominantly or entirely of blastic cells are present in any case of follicular lymphoma, a diagnosis of diffuse large B-cell lymphoma is also made. Lymphomas composed of centro cytes and centroblasts with an entirely diffuse pattern in the sampled tissue may be included in this category.

ICD 11 Code For Follicular lymphoma

  2A80  Follicular lymphoma

Inclusions:

• follicular lymphoma with or without diffuse areas

Exclusions:

• Mature T-cell or NK-cell neoplasms

2A80.0 Follicular lymphoma grade 1

2A80.1 Follicular lymphoma grade 2

2A80.2 Follicular lymphoma grade 3

2A80.3 Primary cutaneous follicle centre lymphoma

Definition of Primary cutaneous follicle centre lymphoma: A primary lymphoma of the skin composed of various numbers of small and large irregular neoplastic follicle center cells. Its morphologic pattern can be nodular, diffuse, or nodular and diffuse. It presents with solitary or grouped plaques and tumours, and it usually involves the scalp, forehead, or trunk. It rarely involves the legs. This type of cutaneous lymphoma tends to remain localised to the skin, and it has a favorable prognosis.

2A80.4 Paediatric type follicular lymphoma

Definition of Paediatric type follicular lymphoma: A variant of follicular lymphoma often involving cervical or other peripheral lymph nodes and the Waldeyer ring. It is frequently localised, and often lacks BCL-2 protein expression and never has a BCL2 translocation. It is usually but not exclusively seen in the pediatric population. The prognosis is usually favorable.

2A80.5 Follicular lymphoma in situ

2A80.6 Follicular lymphoma of small intestine

2A80.Y Other specified follicular lymphoma

2A80.Z Follicular lymphoma unspecified

Definition of Follicular lymphoma unspecified: Non-Hodgkin lymphomas are characterised by a proliferation of predominantly large neoplastic B lymphocytes.

Diffuse large B-cell lymphomas

ICD 11 Code For Diffuse large B-cell lymphomas

  2A81  Diffuse large B-cell lymphomas

Coded Elsewhere:

• Diffuse large B-cell lymphoma of small intestine (2B80.Y)

2A81.0 Primary mediastinal large B-cell lymphoma

Definition of Primary mediastinal large B-cell lymphoma: A large B-cell non-Hodgkin lymphoma arising in the mediastinum. Morphologically it is characterised by a massive diffuse lymphocytic proliferation associated with compartmentalizing fibrosis.

2A81.1 Intravascular large B-cell lymphoma

2A81.2 Plasmablastic lymphoma

Definition of Plasmablastic lymphoma: An aggressive diffuse large B-cell lymphoma frequently arising in the setting of HIV infection and characterised by the presence of large neoplastic cells resembling B-immunoblasts which have the immunophenotypic profile of plasma cells. Sites of involvement include the oral cavity and other extranodal sites

2A81.3 Lymphomatoid granulomatosis

2A81.4 T-cell/histiocyte rich large B-cell lymphoma

Definition of T-cell/histiocyte rich large B-cell lymphoma: A large B-cell lymphoma characterised by the presence of a limited number of scattered neoplastic large B-lymphocytes which are admixed with numerous non-neoplastic T-lymphocytes and frequently histiocytes.

2A81.5 Primary diffuse large B-cell lymphoma of central nervous system

2A81.6 Epstein-Barr Virus-positive diffuse large B cell lymphoma of the elderly

Definition of Epstein-Barr Virus-positive diffuse large B cell lymphoma of the elderly: An aggressive diffuse large B-cell lymphoma affecting patients older than 50 years. Epstein-Barr virus is present in all cases. There is no known history of immunodeficiency or prior lymphoma. The majority of patients present with extranodal disease.

2A81.7 Diffuse large B-cell lymphoma associated with chronic inflammation

Definition of Diffuse large B-cell lymphoma associated with chronic inflammation: A diffuse large B-cell lymphoma arising in body cavities or narrow spaces of long standing chronic inflammation. The classic example is the pyothorax-associated lymphoma that arises in the pleural cavity of patients with a history of long standing pyothorax.

2A81.8 ALK-positive large B-cell lymphoma

Definition of ALK-positive large B-cell lymphoma: A usually aggressive large B-cell lymphoma characterised by the presence of monomorphic immunoblast-like neoplastic B-lymphocytes in a sinusoidal growth pattern. The neoplastic B-lymphocytes express the ALK kinase but they lack the 2;5 translocation.

2A81.9 Primary effusion lymphoma

Definition of Primary effusion lymphoma: An aggressive non-Hodgkin B-cell lymphoma composed of large cells, presenting as a serous effusion without detectable tumour masses. It is universally associated with human herpes virus 8 (HHV-8)/Kaposi sarcoma herpes virus (KSHV) [HHV-8/KSHV]. It mostly occurs in the setting of immunodeficiency; most cases have been reported in HIV positive patients. The most common sites of involvement are the pleural, pericardial, and peritoneal cavities. The prognosis is extremely unfavorable.

2A81.A Primary cutaneous diffuse large B-cell lymphoma leg type

Definition of Primary cutaneous diffuse large B-cell lymphoma leg type: An aggressive primary cutaneous B-cell lymphoma, usually involving the lower leg. It is composed of a generally monotonous proliferation of immunoblasts, or less frequently centroblasts, with few admixed reactive cells. This type of lymphoma occurs most often in the elderly who present with rapidly growing tumours, usually on one or both legs. Dissemination to extracutaneous sites is frequent.

2A81.Y Other specified diffuse large B-cell lymphomas

2A81.Z Diffuse large B-cell lymphoma not otherwise specified

Mature B-cell neoplasm with leukaemic behaviour

ICD 11 Code For Mature B-cell neoplasm with leukaemic behaviour

  2A82  Mature B-cell neoplasm with leukaemic behaviour

2A82.0 Chronic lymphocytic leukaemia or small lymphocytic lymphoma

Definition of Chronic lymphocytic leukaemia or small lymphocytic lymphoma: An indolent, mature B-cell neoplasm composed of small, round B-lymphocytes. When the bone marrow and peripheral blood are involved, the term chronic lymphocytic leukaemia is used. The term small lymphocytic lymphoma is restricted to cases which do not show leukemic involvement of the bone marrow and peripheral blood.

Inclusions:

• Small cell B-cell lymphoma

2A82.00 Chronic lymphocytic leukaemia of B-cell type

Definition of Chronic lymphocytic leukaemia of B-cell type: Chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL) is a neoplasm composed of monomorphic small, round to slightly irregular B lymphocytes in the peripheral blood (PB), bone marrow (BM), spleen and lymph nodes, admixed with prolymphocytes and paraimmuno – blasts forming proliferation centres in tissue infiltrates.The CLL/SLL cells usually coexpress CD5 and CD23. In the absence of extramedullary tissue involvement, there must be ?5×109/L monoclonal lymphocytes with a CLL phenotype in the PB. The International Workshop on Chronic Lymphocytic leukaemia (IWCLL) report requires that the lymphocytosis be present for at least 3 months and also allows for the diagnosis of CLL to be made with lower lymphocyte counts in patients with cytopenias or disease-related symptoms {873A}. Whether patients who would have fulfilled the criteria in the past for CLL but who fulfill the criteria only for monoclonal B lymphocytosis (MBL) are better considered to have low stage CLL or MBL remains to be determined. Some may prefer to still consider many of these cases more like CLL. The term SLL is used for non-leukaemic cases with the tissue morphology and immunophenotype of CLL. The IWCLL definition of SLL requires lymphadenopathy, no cytopenias due to BM infiltration by CLL/SLL and <5×109/L PB B-cells {873A}.

Inclusions:

• Lymphoplasmacytic leukaemia

Exclusions:

• Lymphoplasmacytic lymphoma (2A85.4)

Coded Elsewhere:

Richter syndrome (2A81.Y)

2A82.0Y Other specified chronic lymphocytic leukaemia or small lymphocytic lymphoma

2A82.0Z Chronic lymphocytic leukaemia or small lymphocytic lymphoma unspecified

2A82.1 B-cell prolymphocytic leukaemia

2A82.10 B-cell prolymphocytic leukaemia in complete remission

2A82.1Y Other specified b-cell prolymphocytic leukaemia

2A82.1Z B-cell prolymphocytic leukaemia unspecified

2A82.2 Hairy-cell leukaemia

Definition of Hairy-cell leukaemia: A neoplasm of small B-lymphocytes with hairy projections in bone marrow, spleen, and peripheral blood. Most patients present with splenomegaly and pancytopenia.

Inclusions:

• Leukaemic reticuloendotheliosis

2A82.3 Splenic B-cell lymphoma or leukaemia unclassifiable

Definition of Splenic B-cell lymphoma or leukaemia unclassifiable: A small B-cell clonal lymphoproliferative disorder of the spleen that does not fall into any of the other categories of mature B-cell neoplasms.

2A82.Y Other specified mature B-cell neoplasm with leukaemic behaviour

2A82.Z Mature B-cell neoplasm with leukaemic behaviour unspecified

Definition of Mature B-cell neoplasm with leukaemic behaviour unspecified: Plasma cells, usually secreting monoclonal immunoglobulin (M-protein) and/or immunoglobulin light chains.

Plasma cell neoplasms

ICD 11 Code For Plasma cell neoplasms

  2A83  Plasma cell neoplasms

2A83.0 Monoclonal gammopathy of undetermined significance

2A83.1 Plasma cell myeloma

Definition of Plasma cell myeloma: A bone marrow-based plasma cell neoplasm usually characterised by a serum monoclonal protein and/or urinary light chains. ”CRAB” criteria (osteolytic lesions, hypercalcaemia, renal failure, and anaemia) separate symptomatic plasma cell myeloma form asymptomatic (smoldering) myeloma.

Inclusions:

• Kahler disease
• Myelomatosis
• Medullary plasmacytoma
• multiple myeloma

Exclusions:

• Solitary plasmacytoma (2A83.2)

2A83.2 Solitary plasmacytoma

Definition of Solitary plasmacytoma: A single focus of clonal (malignant) plasma cells either in the bone or in another anatomic site without peripheral blood involvement. –2003

Inclusions:

• Solitary myeloma

2A83.3 Extraosseous plasmacytoma

2A83.4 Plasma cell leukaemia

Definition of Plasma cell leukaemia: An aggressive plasma cell neoplasm. It is characterised by the presence of neoplastic plasma cells in the peripheral blood. The peripheral blood plasma cells comprise more than 20% of the peripheral blood white cells or the number of clonal plasma cells in the PB exceeds 2×10?/L.

2A83.5 Monoclonal immunoglobulin deposition disease

2A83.50 Heavy chain deposition disease

Definition of Heavy chain deposition disease: A disease of the kidney, caused by proliferation and deposition of pieces of truncated or abnormal alpha, gamma, delta, or mu immunoglobulin heavy chain segments of white blood cells. This disease is characterised by fibrillar or granular tissue deposits and renal dysfunction, which may lead to organ failure. Confirmation is by identifying heavy chain deposition tissue biopsy using immunofluorescence under a microscope.

Exclusions:

• Heavy chain diseases or malignant immunoproliferative diseases (2A84)
• Immunoglobulin heavy chain deficiency (4A01.04)

2A83.51 Light and heavy chain deposition disease

Definition of Light and heavy chain deposition disease: A disease of the kidney, caused by proliferation and deposition of pieces of truncated or abnormal light and heavy chain segments of white blood cells. This disease is characterised by fibrillar or granular tissue deposits and renal dysfunction, which may lead to organ failure. Confirmation is by identification of light and heavy chain deposition tissue biopsy under a microscope.

2A83.52 Light chain deposition disease

Definition of Light chain deposition disease: A disease of the kidney, caused by the deposition of pieces of truncated or abnormal light chain segments of white blood cells. This disease is characterised by fibrillar or granular tissue deposits and renal dysfunction, which may lead to organ failure. Confirmation is by identification of light chain deposition tissue biopsy under an electron microscope.

Exclusions:

Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells (4A01.04)

2A83.Y Other specified multiple myeloma and plasma cell neoplasms

2A83.Z Plasma cell neoplasm unspecified

Definition of Plasma cell neoplasm unspecified: A group of rare disorders of immunoglobulin synthesis associated with B-cell proliferative disorders that produce monoclonal heavy chains and typically no light chains.

Heavy chain diseases or malignant immunoproliferative diseases

ICD 11 Code For Heavy chain diseases or malignant immunoproliferative diseases

  2A84  Heavy chain diseases or malignant immunoproliferative diseases

2A84.0 Alpha heavy chain disease

Definition of Alpha heavy chain disease: The small intestinal morphologic changes are consistent with a mucosa-associated lymphoid tissue lymphoma (MALT lymphoma).

2A84.1 Gamma heavy chain disease

Definition of Gamma heavy chain disease: A clonal disorder characterised by the secretion of a truncated gamma chain. In most cases, it is associated with morphologic changes also seen in lymphoplasmacytic lymphomas, but the clinical course is typically more aggressive than in lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinemia.

Inclusions:

• Franklin disease

2A84.2 Mu heavy chain disease

2A84.Y Other specified malignant immunoproliferative diseases

2A84.Z Heavy chain diseases unspecified

Other specified mature B-cell neoplasms or lymphoma

ICD 11 Code For Other specified mature B-cell neoplasms or lymphoma

  2A85  Other specified mature B-cell neoplasms or lymphoma

2A85.0 Nodal marginal zone lymphoma

Definition of Nodal marginal zone lymphoma: A primary nodal B-cell non-Hodgkin lymphoma which morphologically resembles lymph nodes involved by marginal zone lymphomas of extranodal or splenic types, but without evidence of extranodal or splenic disease. This is a rare entity, and most patients present with localised or generalised lymphadenopathy. The clinical course is indolent.

2A85.1 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue of stomach

Definition of Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue of stomach: A low grade, indolent B-cell lymphoma, usually associated with Helicobacter pylori infection. Morphologically it is characterised by a dense mucosal atypical lymphocytic (centrocyte-like cell) infiltrate with often prominent lymphoepithelial lesions and plasmacytic differentiation. Some of gastric MALT lymphomas carry the t(11;18)(q21;q21). Such cases are resistant to Helicobacter pylori therapy.

2A85.2 Extranodal marginal zone B-cell lymphoma primary site skin

Definition of Extranodal marginal zone B-cell lymphoma primary site skin: A low-grade, extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue that arises from the skin. It usually presents with multifocal papular or nodular lesions in the arms or trunk. It rarely disseminates to internal organs or progresses to high grade lymphoma.

2A85.3 Extranodal marginal zone B-cell lymphoma primary site excluding stomach or skin

2A85.4 Lymphoplasmacytic lymphoma

Definition of Lymphoplasmacytic lymphoma: Neoplasm of small B lymphocytes and plasma cells, mostly residing in the bone marrow. Frequently associated with the production of an IgM serum monoclonal protein, then called Waldenström macroglobulinemia (WM).

Inclusions:

• primary macroglobulinaemia
• Waldenström macroglobulinaemia
• Waldenström macroglobulinaemia without mention of remission

Exclusions:

• small cell B-cell lymphoma (2A82.0)
• Chronic lymphocytic leukaemia or small lymphocytic lymphoma (2A82.0)

2A85.5 Mantle cell lymphoma

Definition of Mantle cell lymphoma: Mantle cell lymphoma is a rare form of malignant non-Hodgkin lymphoma affecting B lymphocytes in the lymph nodes in a region called the “mantle zone”. It accounts for 2-10% of lymphomas.

Inclusions:

• Small cell mantle cell lymphoma

2A85.6 Burkitt lymphoma including Burkitt leukaemia

Definition of Burkitt lymphoma including Burkitt leukaemia: A highly aggressive lymphoma composed of monomorphic medium-sized B-cells with basophilic cytoplasm and numerous mitotic figures. It is often associated with the presence of Epstein-Barr virus (EBV) and is commonly seen in AIDS patients. Three morphologic variants are recognised: classical Burkitt lymphoma, Burkitt lymphoma with plasmacytoid differentiation, and atypical Burkitt/Burkitt-like lymphoma. All cases express the MYC translocation [t(8;14)].

Inclusions:

• “Burkitt-like” lymphoma

Coded Elsewhere:

• HIV – [human immunodeficiency virus] disease associated with Burkitt lymphoma (1C62.3)

2A85.Y Further specified mature B-cell neoplasms or lymphoma

B-cell lymphoma mixed features

ICD 11 Code For B-cell lymphoma mixed features

  2A86  B-cell lymphoma mixed features

2A86.0 Malignant lymphoma of B cell type not elsewhere classified

Coding Note:

• If B-cell lineage or involvement is mentioned in conjunction with a specific lymphoma, code to the more specific description.

2A86.1 B-cell lymphoma unclassifiable with features intermediate between Burkitt lymphoma and diffuse large B-cell lymphoma

2A86.2 B-cell lymphoma unclassifiable with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma

2A86.Y Other specified B-cell lymphoma mixed features

2A86.Z B-cell lymphoma mixed features unspecified

Mature B-cell neoplasms unspecified

ICD 11 Code For Mature B-cell neoplasms unspecified

  2A8Z  Mature B-cell neoplasms unspecified