Factor VIII inhibitor prior to and during secondary acute nonlymphocytic leukemia in a patient with cured Hodgkin's disease.
We report a 54-year-old patient with Hodgkin's disease who achieved a complete remission after combined modality treatment. Three years later the patient developed a severe hemorrhagic syndrome, concomitant with 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 years later, a secondary acute nonlymphocytic leukemia (ANLL) was diagnosed. Two courses of chemotherapy with fludarabine, cytosine arabinoside and G-CSF (FLAG) were able to obtain a complete remission. Hemorrhagic complications were mainly linked to thrombocytopenia and continued until recovery of thrombopoiesis. Factor VIII inhibitor levels and related clinical symptoms decreased progressively. In conclusion, we suggest that FLAG succeeded in inhibiting an abnormal lymphoid clone responsible for factor VIII inhibitor production, suggesting a possible role for intensive chemotherapy in similar situations, which are often refractory to conventional immunosuppressive and depletive therapy.
Bendandi
M
M
Institute of Hematology Lorenzo e Ariosto Seràgnoli, Bologna University, Italy.
Zaccaria
A
A
Zinzani
P L
PL
Visani
G
G
Stefanati
V
V
Cantagalli
F
F
Mancino
A
A
Macchi
S
S
Simoncelli
F
F
Tura
S
S
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood*; D001803:Blood Transfusion; D003131:Combined Modality Therapy; D003561:Cytarabine / Q000008:administration & dosage; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000627:therapeutic use; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006467:Hemophilia A / Q000209:etiology* / Q000276:immunology; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy; D006689:Hodgkin Disease / Q000188:drug therapy* / Q000532:radiotherapy; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015470:Leukemia, Myeloid, Acute / Q000150:complications* / Q000276:immunology; D015182:Lymphatic Irradiation; D008466:Mechlorethamine / Q000008:administration & dosage; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000150:complications* / Q000276:immunology; D010956:Plasmapheresis; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D011344:Procarbazine / Q000008:administration & dosage; D011516:Prothrombin / Q000627:therapeutic use; D012074:Remission Induction; D013921:Thrombocytopenia / Q000209:etiology; D014740:Vidarabine / Q000008:administration & dosage / Q000031:analogs & derivatives; D014750:Vincristine / Q000008:administration & dosage
D001323:Autoantibodies; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D003561:Cytarabine; D016179:Granulocyte Colony-Stimulating Factor; D011344:Procarbazine; D008466:Mechlorethamine; D014750:Vincristine; D011516:Prothrombin; D005169:Factor VIII; D014740:Vidarabine; C024352:fludarabine; D011241:Prednisone