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Immunological dysfunction in paroxysmal nocturnal haemoglobinuria

E Vellenga, N H Mulder, T H The

    Clinical and Laboratory Haematology
    |January 1, 1981
    PubMed
    Summary

    Patients with paroxysmal nocturnal haemoglobinuria (PNH) exhibit impaired T-cell immunity and potential B-cell dysfunction, suggesting immune system disturbances linked to their primary bone marrow disease.

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    Area of Science:

    • Immunology
    • Hematology
    • Cell Biology

    Background:

    • Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired blood disorder characterized by the destruction of red blood cells.
    • The impact of PNH on the immune system, particularly T-cell and B-cell function, requires further elucidation.

    Purpose of the Study:

    • To investigate the immune capacity in patients diagnosed with paroxysmal nocturnal haemoglobinuria.
    • To assess T-cell and B-cell function, including lymphocyte proliferation and immunoglobulin levels.

    Main Methods:

    • In vitro lymphocyte proliferation assays using allogeneic lymphocytes, concanavalin A (Con A), phytohaemagglutinin (PHA), and pokeweed mitogen (PWM).
    • Assessment of T-cell and B-cell numbers.
    • In vivo dinitrochlorobenzene (DNCB) response testing.
    • Measurement of immunoglobulin (Ig) levels and anti Helix pomatia haemocyanin (HPH) antibody response.
    • Evaluation of inulin's effect on lymphocyte function.

    Main Results:

    • Impaired in vitro lymphocyte proliferation was observed in response to allogeneic lymphocytes and Con A, indicating disturbed T-cell immunity despite normal T-cell numbers.
    • Most patients showed adequate PHA response and normal in vivo DNCB response, suggesting a partially preserved T-cell population.
    • Normal immunoglobulin levels and anti-HPH antibody response were noted in most patients.
    • Some patients displayed a very low response to PWM, alongside a decreased number of B cells, suggesting B-cell dysfunction.
    • Inulin exposure enhanced the in vitro effects on lymphocytes from PNH patients, pointing to functional lymphocyte damage.

    Conclusions:

    • Patients with PNH exhibit functional immunological disturbances, primarily affecting T-cell and B-cell compartments.
    • The observed immune dysfunctions may stem from an abnormal cell clone characteristic of PNH.
    • These immunological abnormalities are suggested to be a consequence of the primary bone marrow disease in PNH.

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