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Haemopoietic stem cell disorders 

Haemopoietic stem cell disorders

Chapter:
Haemopoietic stem cell disorders
Author(s):

D.C. Linch

DOI:
10.1093/med/9780199204854.003.220202
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date: 25 March 2017

The term stem-cell disorder is usually understood to imply that a disease process has begun in a primitive cell with the potential to develop into cells of different lineages. Difficulties with such a definition include (1) malignant change in a very primitive cell does not necessarily lead to the production of mature cells of multiple lineages; (2) although an immature phenotype of a malignant cell may indicate transformation of a very early cell, it is possible that transformation could arise in a later cell with subsequent dedifferentiation.

Possible stem cell disorders—quantitative and qualitative abnormalities of haemopoietic stem cells could result in one or more of the following: (1) depletion of the stem cell population leading to failure of production of adequate mature progeny; (2) failure of proliferation of a normal number of stem cells also leading to a similar deficiency of end-cells; (3) production of normal numbers of defective end-cells; (4) malignant transformation of stem cells.

A pragmatic approach—the involvement of multiple lineages in a disease process is a ready indicator of a stem cell disorder. Such disorders include (1) myeloproliferative disorders—polycythaemia rubra vera, chronic myeloid leukaemia, myelofibrosis, and various intermediate/transitional forms are characterized by the predominant cell type produced by the malignant clone, but they all involve a primitive stem cell; (2) acute myeloid leukaemia—in adults, red cells and platelets are commonly involved in addition to myeloid cells; (3) aplastic anaemia—by definition refers to involvement of multiple myeloid lineages; (4) paroxysmal nocturnal haemoglobinuria—due to a somatic mutation in the haemopoietic stem cell in the X-linked phosphatidylinositol glycan-A (PIG-A).

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