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FN-C1q and C1 INH C1r-C1s complexes as indicators of complement activation in patients with chronic lymphocytic
T Hidvégi1, G A Ermolin, E E Efremov
1National Institute of Haematology and Blood Transfusion, Budapest, Hungary.
Insights
Chronic lymphocytic leukemia (CLL) patients show increased levels of early classical complement pathway activation complexes. This suggests the classical complement pathway is activated in CLL, potentially explaining hypocomplementaemia in these patients.
Area of Science:
- Immunology
- Hematology
- Complement System
Background:
- Previous studies indicated low C1 inhibitor (C1 INH) and C4 INH levels in chronic lymphocytic leukemia (CLL) patients.
- Hypocomplementaemia in CLL was hypothesized to result from continuous classical complement pathway activation.
Purpose of the Study:
- To investigate the role of the classical complement pathway in CLL pathogenesis.
- To compare the levels of C1 INH-C1rC1s and C1q-FN complexes in CLL patients versus healthy controls.
Main Methods:
- Serum samples from 95 CLL patients and 100 healthy controls were analyzed.
- Levels of C1 INH-C1rC1s and C1q-FN complexes, markers of early classical pathway activation, were quantified.
Main Results:
- A significant elevation in both C1 INH-C1rC1s and C1q-FN complex levels was observed in CLL patients compared to healthy controls.
- These findings provide direct evidence for early classical complement pathway activation in CLL.
Conclusions:
- The classical complement pathway is demonstrably activated in patients with chronic lymphocytic leukemia.
- This activation may contribute to the observed hypocomplementaemia and disease progression in CLL.
Abstract:
We have previously found low levels of C1 and C4 INH in the sera of chronic lymphocytic leukaemia (CLL) patients. Hypocomplementaemia was supposed to be the consequence of a permanent activation of the classical pathway. We have compared the levels of C1 INH-C1rC1s and C1q-FN complexes in the sera of 95 CLL patients and 100 healthy controls, because these complexes are known to be formed in the early stage of classical pathway activation. A significant increase in the level of both types of complexes was found in sera of CLL patients as compared to the controls. These findings support the assumption that the classical complement pathway is activated in the patients with CLL.