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Haematological Malignancies: Introduction and Index

The Haematological Malignancies are a group of neoplasms that arise through malignant transformation of bone marrow derived cells. The great diversity seen in this group of disorders is a reflection of the complexity of normal haematopoeisis and the immune system.

Over the past 100 years a number of classifications have been devised in an attempt to subdivide haematological malignancies in a clinically and biologically relevant way. The primary basis of classification is the distinction between tumours of lymphocytes (the primary cell of the immune systems) and myeloid malignancies.

Myeloproliferative disorders are currently classified using the FAB classification. The acute myeloid leukaemia consist of very early stem cell and progenitor cells and are subclassified according to evidence of lineage differentiation. Myelodysplastic syndrome is a stem cell disorder in which there is failure of non-haematopoeisis leading to pancytopaenia. In many cases myelodysplasia undergoes progresison to acute leukaemia. In the chronic myeloproliferative disorders there is overproduction of one or more types of peripheral blood cell.

Lymphoproliferative disorders are sub-classified used the REAL (Revised European American Lymphoma) classification. This classification defines a number of clinico-pathological entities based on the stage of differentiation of the tumour cells as defined by their morphological appearances and expression of specific cell markers, the presence of genetic abnormalities in the cells and clinical features.

One of the major subdivisions is between tumours of precursor lymphocytes (lymphoblastic leukaemia) and those that consist of peripheral or mature T or B-cells. The nature of Hodgkin's lymphoma, which is a specific clinical entity, has been the subject of controversy for many years. However recent evidence clearly suggests that this is a variant of a peripheral B-cell lymphoma.

Key to the Haematological Malignancies

Precursor Lymphoid Cell Disorders

Lymphoblastic:Acute Lymphoblastic Leukaemia

Mature Lymphoid Disorders: B-cell

CLL:Chronic Lymphoid Leukaemia
DLBCL:Diffuse Large B-cell Lymphoma
FCL:Follicle Centre Lymphoma
Hodgkin's:Hodgkin's Lymphoma
MCL:Mantle Cell Lymphoma
MZL & WM:Marginal Zone Lymphoma and Waldenstrom's Macroglobulinaemia
Myeloma:Myeloma and Monoclonal Gammopathy of Uncertain Significance (MGUS)

Mature Lymphoid Disorders: T-cell

Leukaemic:Large Granular Lymphocyte, T-Prolymphocytic, Sezary Syndrome
Nodal:Common, Angio-immunoblastic, and Anaplastic Large Cell Lymphomas
Extranodal:Mycosis fungoides, Lymphomatoid Papulosis, Small intestinal lymphoma

Myeloid Disorders

AML:Acute Myeloid Leukaemia
MDS:Myelodysplastic Syndrome
MPD:Myeloproliferative Disorders
PNH & aplasia:Paroxysmal Nocturnal Haemoglobinuria and Aplastic Anaemia

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Useful Links

Guide to Internet Resources for Cancer: The Leukaemia Page at the University of Newcastle.
This site features many useful links to other resources on the internet.

Bloodline - The Online Resource for Hematology Education and News.
Mainly for healthcare professionals, and requires registration for site access.

GrannyBarb and Art's Leukaemia Links.
One of the best resources for patient-orientated information on leukamia available on the internet.
Part of the Association of Cancer Online Resources internet site.


Document last updated: Tuesday, 16 May 2000

Comments & Feedback to: [email protected]

Copyright © HMDS, 1999

[URL: www.hmds.org.uk/disorders.shtml]