Lymphoproliferative syndromes

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A term used to describe a number of neoplasms, at different stages of maturity, originating in lymphoid cells. The lymphatic system is a network of organs (spleen, thymus), lymph nodes, ducts and lymphatic vessels that produce and transport lymph from the tissues to the blood stream. It is a fundamental part of the body's immune system. There are two main types of lymphocyte in the blood called B lymphocytes (B cell) and T lymphocytes (T cell).

B lymphocytes: account for 5 - 15% of all lymphocytes and give rise to plasma cells that produce antibodies which help the body protect itself against bacteria and viruses.

T lymphocytes: are responsible for coordinating the immune cell response and have the role of cooperating to develop all the forms of the immune response, as well as the production of antibodies by the B lymphocytes.

B and T circulating lymphocytes derive, like all the other blood cells, from the hematopoietic stem cells in the bone marrow. The stem cell gives rise, amongst others, to the lymphoblasts (less mature lymphoid cells) which, after successive stages of maturation, become lymphocytes. The neoplastic transformation of these cells can occur during any of these stages of the lymphocyte's maturation and this is what leads to the various kinds of lymphoproliferative syndrome (LPS). It also explains the great variety and biological and clinical heterogeneity of these tumours.

For the purposes of clarification the following large groups of lymphoid neoplasms are usually distinguished:

— Immature lymphoid cell neoplasms

— Acute lymphoblastic leukaemia (various subtypes, see ALL)

— Mature lymphoid cell neoplasms

— Non-Hodgkin lymphoma (NHL) (more than 20 types, see table)

— Other LPS with leukaemic expression (hairy cell leukaemia, splenic marginal zone lymphoma, prolymphocytic leukemia, granular lymphocytic leukaemia, amongst others)

— Monoclonal gammopathies (multiple myelomaWaldenström macroglobulinemiaprimary amyloidosisheavy chain disease, amongst others)

— Chronic lymphocytic leukaemia (LLC)

— Hodgkin lymphoma (LH) (various subtypes, see table)

Furthermore, many of these diseases can be sub-classified according to their origin in B or T cells.

Consequently, the classification proposed for lymphoid neoplasms by the WHO (2008) is rather complex:

Precursor cell lymphoid neoplasms

  • Leukaemia / Lymphoblastic Lymphoma B
  • Leukaemia / Lymphoblastic Lymphoma T

Mature B-cell neoplasms

  • Chronic Lymphocytic Leukemia / small lymphocytic lymphoma
  • B-cell prolymphocytic leukemia
  • Splenic marginal zone B-cell lymphoma
  • Hairy cell leukaemia
  • Lymphoplasmacytic lymphoma / Waldenström macroglobulinemia 
  • Plasma cell myeloma / plasmacytoma
  • Extranodal marginal zone B-cell lymphoma of MALT type
  • Nodal marginal zone B-cell lymphoma
  • Follicular lymphoma
  • Primary cutaneous centrofollicular lymphoma
  • Mantle-cell lymphoma
  • Diffuse large B-cell lymphoma
  • T-cell histocyte rich primary large B-cell lymphoma
  • Diffuse large B-cell lymphoma of the central nervous system
  • Primary large B-cell lymphoma of the central nervous system
  • Primary cutaneous large B-cell lymphoma, leg type
  • Lymphomatous granulomatosis
  • Primary mediastinal lymphoma
  • Intravascular lymphoma
  • Plasmablastic lymphoma 
  • Primary effusion lymphoma
  • Lymphoma / Burkitt cell leukaemia

Mature T-cell and NK-cell neoplasms

  • T-cell prolymphocytic leukemia
  • T-cell large granular lymphocyte leukaemia
  • Indolent, NK-cell granular lymphoproliferative process
  • Aggressive NK-cell leukaemia
  • Adult T-cell lymphoma/leukaemia (HTLV-1positive)
  • Extranodal NK/T-cell lymphoma of T/NK cells
  • Enteropathy-associated T-cell lymphoma
  • Hepatosplenic T-cell lymphoma
  • Subcutaneous panniculities-like T-cell lymphoma
  • Mycosis fungoides/Sezary syndrome
    • Primary cutaneous C30 positive lymphoproliferative syndrome
    • Lymphomatoid papulosis
    • Anaplastic large-cell lymphoma, primary cutaneous type
  • Peripheral T-cell lymphoma, not otherwise characterised
  • Angioimmunoblastic T-cell lymphoma
  • Anaplastic T-cell lymphoma, ALK positive
  • Anaplastic T-cell lymphoma, ALK negative

Hodgkin lymphoma

  • Nodular lymphocyte-predominant Hodgkin lymphoma
  • Classical Hodgkin lymphoma
  • Nodular sclerosis classical Hodgkin lymphoma
  • Lymphocyte-rich classical Hodgkin lymphoma
  • Mixed cellularity Hodgkin lymphoma
  • Lymphocyte depletion Hodgkin lymphoma

*Most frequent indicated in bold type.

Webpage updated 12/01/2023 13:27:05