M. Bendandi et al., FACTOR-VIII INHIBITOR PRIOR TO AND DURING SECONDARY ACUTE NONLYMPHOCYTIC LEUKEMIA IN A PATIENT WITH CURED HODGKINS-DISEASE, Leukemia & lymphoma, 16(5-6), 1995, pp. 511-513
We report a 54-year-old patient with Hodgkin's disease who achieved a
complete remission after combined modality treatment. Three years late
r the patient developed a severe hemorrhagic syndrome, concomitant wit
h the onset of a factor VIII inhibitor in plasma. The control of very
proteiform bleedings was extremely difficult, even with plasmaphereses
, as well as with immunosuppressive and substitutive therapies. Two ye
ars later, a secondary acute nonlymphocytic leukemia (ANLL) was diagno
sed. Two courses of chemotherapy with fludarabine, cytosine arabinosid
e and G-CSF (FLAG) were able to obtain a complete remission. Hemorrhag
ic complications were mainly linked to thrombocytopenia and continued
until recovery of thrombopoiesis. Factor VIII inhibitor levels and rel
ated clinical symptoms decreased progressively. In conclusion, we sugg
est that FLAG succeeded in inhibiting an abnormal lymphoid clone respo
nsible for factor VIII inhibitor production, suggesting a possible rol
e for intensive chemotherapy in similar situations, which are often re
fractory to conventional immunosuppressive and depletive therapy.