WHO Lymphoma Classification: System of Lymphoma Types

Over the years, various classification systems have been used to differentiate lymphoma types including the Rappaport Classification (used until the 70's), the Working Formulation, the National Cancer Institute Working Formulation, and the Revised European-American Lymphoma Classification (REAL).

We have chosen to present the World Health Organization's International Classification of Diseases (WHO ICD).

The WHO classification has its origins in the 1850s. The first edition, known as the International List of Causes of Death, was adopted by the International Statistical Institute in 1893. The ICD is the international standard diagnostic classification. It is used to classify diseases and other health problems recorded on many types of health and vital records including death certificates and health records. These records also provide the basis for the compilation of national mortality and morbidity statistics by WHO Member States.

The older Rappaport, Working Formulation, and REAL categories are described in a separate section for reference. This might be helpful if a patient's records state some of the classifications of older lymphoma types.

Hodgkin's Disease Classification

  • Lymphocytic-histiocytic predominance
  • Nodular sclerosis
  • Mixed cellularity
  • Lymphocytic depletion
  • Other Hodgkin's disease
  • Hodgkin's disease, unspecified

Follicular Lymphoma Classification

  • Small cleaved cell, follicular
  • Mixed small cleaved and large cell, follicular
  • Large cell, follicular
  • Other follicular non-Hodgkin's lymphoma types
  • Follicular non-Hodgkin's lymphoma, unspecified
    • Nodular non-Hodgkin's lymphoma NOS

Diffuse Non-Hodgkin's Lymphoma Classification

Peripheral and cutaneous T-cell lymphomas

  • Mycosis fungoides
  • Sézary's disease
  • T-zone lymphoma
  • Lymphoepithelioid lymphoma
    • Lennert's lymphoma
  • Peripheral T-cell lymphoma
  • Other and unspecified T-cell lymphomas

Other and unspecified types of non-Hodgkin's lymphoma

  • Lymphosarcoma
  • B-cell Lymphoma Classification, unspecified
  • Other specified types of non-Hodgkin's lymphoma
    • Malignant:
      • reticuloendotheliosis
      • reticulosis
    • Microglioma
  • Non-Hodgkin's lymphoma, unspecified type
    • Lymphoma NOS
    • Malignant lymphoma NOS
    • Non-Hodgkin's lymphoma NOS

Malignant immunoproliferative diseases

  • Waldenström's macroglobulinaemia
  • Alpha heavy chain disease
  • Gamma heavy chain disease
    • Franklin's disease
  • Immunoproliferative small intestinal disease
    • Mediterranean disease
  • Other malignant immunoproliferative diseases
  • Malignant immunoproliferative disease, unspecified
    • Immunoproliferative disease NOS

Multiple myeloma and malignant plasma cell neoplasms

  • Multiple myeloma
    • Kahler's disease
    • Myelomatosis
    • Excludes: solitary myeloma
  • Plasma cell leukemia
  • Plasmacytoma, extramedullary
    • Malignant plasma cell tumour NOS
    • Plasmacytoma NOS
    • Solitary myeloma

Lymphoid leukemia

Myeloid leukemia

  • Includes:
    • granulocytic
    • myelogenous
  • Acute myeloid leukemia
    • Excludes: acute exacerbation of chronic myeloid leukemia
  • Chronic myeloid leukemia
  • Subacute myeloid leukemia
  • Myeloid sarcoma
    • Chloroma
    • Granulocytic sarcoma
  • Acute promyelocytic leukemia
  • Acute myelomonocytic leukemia
  • Other myeloid leukemia
  • Myeloid leukemia, unspecified

Monocytic leukemia

  • Includes: monocytoid leukemia
  • Acute monocytic leukemia
    • Excludes: acute exacerbation of chronic monocytic leukemia
  • Chronic monocytic leukemia
  • Subacute monocytic leukemia
  • Other monocytic leukemia
  • Monocytic leukemia , unspecified

Other leukemias of specified cell type

  • Acute erythraemia and erythroleukemia
    • Acute erythraemic myelosis
    • Di Guglielmo's disease
  • Chronic erythraemia
    • Heilmeyer-Schöner disease
  • Acute megakaryoblastic leukemia
    • leukemia :
      • megakaryoblastic (acute)
      • megakaryocytic (acute)
  • Mast cell leukemia
  • Acute panmyelosis
  • Acute myelofibrosis
  • Other specified leukemia s
    • Lymphosarcoma cell leukemia

Leukemia of unspecified cell type

  • Acute leukemia of unspecified cell type
    • Blast cell leukemia
    • Stem cell leukemia
  • Chronic leukemia of unspecified cell type
  • Subacute leukemia of unspecified cell type
  • Other leukemia of unspecified cell type
  • leukemia , unspecified

Other and unspecified malignant neoplasms of lymphoid, haematopoietic and related tissue

  • Letterer-Siwe disease
    • Nonlipid:
      • reticuloendotheliosis
      • reticulosis
  • Malignant histiocytosis
    • Histiocytic medullary reticulosis
  • Malignant mast cell tumour
    • Malignant:
      • mastocytoma
      • mastocytosis
    • Mast cell sarcoma
      • Excludes: mast cell leukemia
      • mastocytosis (cutaneous)
  • True histiocytic lymphoma
  • Other specified malignant neoplasms of lymphoid, haematopoietic and related tissue
  • Malignant neoplasm of lymphoid, haematopoietic and related tissue, unspecified

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