Acquired Factor X (FX) Deficiency, Chronic Lymphocytic Leukaemia (CLL) and Amyloidosis (A).

BASTOS L; GROSSO SH; INGRATTI M; SÁNCHEZ-LUCEROS A; REY I; RODRIGUEZ A; MESCHENGIESER SS; BLANCO AN; LAZZARI MA

Boston, USA

Congreso; XXII Congress of the International Society on Thrombosis and Haemostasis; 2009

International Society on Thrombosis and Haemostasis

ACQUIRED FACTOR X (FX) DEFICIENCY, CHRONIC LYMPHOCYTIC LEUKAEMIA (CLL) AND AMYLOIDOSIS (A).   L. A. Bastos*1, S. H. Grosso1, M. Ingratti1, A. Sanchez Luceros1, I. Rey2, A. Rodriguez1, S. Meschengieser1, A. Blanco1, M. Lazzari11Hemostasia y Trombosis, IIHEMA-Academia Nacional de Medicina, 2Hematolog?a, Sanatorio Santa Isabel, Buenos Aires, Argentina Abstract: Association of CLL and A is rare. Low FX, abnormal thrombin time (TT) and reptilase?s clotting time (RT) were observed in a patient in whom initially, A was not confirmed. Considering the therapeutic implication of the diagnosis, we tried to demonstrate A, and exclude the presence of antibodies anti-FX, possible causes of low FX. Case: A 55 year old man, with recurrent multiple haematomas and abdominal bleeding that required surgery and transfusion of red-cells, fresh-frozen plasma, activated prothrombin complex (PCC) and vit.K. He had no history of bleeding or coagulopathies. CLL had been diagnosed 5 years ago. Laboratory: Platelets: 850x109/L; PT: 22%(N:100; P+N:66); APTT: 64sec (NV: 37-48) (N: 43; P+N: 50); FX: 4%(N:100; P+N:52); abnormal TT (26/18sec) and RT (28/18sec) not corrected by normal plasma; normal liver profile; glomerular proteinuria; negative Congo´s red abdominal fat biopsy. Low FX and renal biopsy: Administration of PCC to achieve 60% of FX, showed no effect (2.5%). No aFX activity was detected in plasma samples (FX:2.4%, N:100, P+N:52; ELISA: patient 0.211, blank 0.242). Despite no laboratory response to substitutive treatment, transjugular renal biopsy was performed with PCC. The only complication observed was persistent haematuria without hemodynamic decompensation. Diffuse renal A was identified (positive Congo´s red). Conclusion: Low FX, abnormal TT and RT are often associated to A; also, the rapid removal of FX from the circulation can be an expression of adsorption to the kidney´s amyloide. Amyloidosis should be suspected and investigated in this kind of patients