TY - JOUR
T1 - Acquired hemophilia A and delta storage pool deficiency in a patient with indolent non-Hodgkin lymphoma
AU - Rossio, Raffaella
AU - Cassin, Ramona
AU - Lecchi, Anna
AU - La Marca, Silvia
AU - Femia, Eti Alessandra
AU - Novembrino, Cristina
AU - Siboni, Simona M.
AU - Noto, Alessandro
AU - Reda, Gianluigi
AU - Peyvandi, Flora
N1 - Publisher Copyright:
© 2021 Taylor & Francis Group, LLC.
Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2022
Y1 - 2022
N2 - B-cell lymphoproliferative diseases may be associated with acquired hemostasis disorders, such as acquired hemophilia A (AHA) caused by autoantibodies that neutralize factor VIII activity, and δ-storage pool deficiency, an abnormality of platelet function due to defective dense granules and impaired secretion. We describe the case of a 67-year-old man in whom these two acquired bleeding disorders were concomitantly present as the first clinical manifestation of an indolent non-Hodgkin lymphoma. Immunosuppressive therapy with prednisone was initially started to eradicate anti-FVIII antibodies, subsequently boosted with cyclophosphamide and rituximab, these medications being also chosen to treat the associated indolent lymphoma. Bleeding symptoms were first tackled with limited benefit by using rFVIIa and then rescued using recombinant porcine FVIII. After a 6 month’s follow-up lymphoma and AHA were in remission and platelet function was improved. This case underlines the need of multiple and complex diagnostic and therapeutic approaches to rare acquired bleeding disorders associated with lymphoproliferative diseases.
AB - B-cell lymphoproliferative diseases may be associated with acquired hemostasis disorders, such as acquired hemophilia A (AHA) caused by autoantibodies that neutralize factor VIII activity, and δ-storage pool deficiency, an abnormality of platelet function due to defective dense granules and impaired secretion. We describe the case of a 67-year-old man in whom these two acquired bleeding disorders were concomitantly present as the first clinical manifestation of an indolent non-Hodgkin lymphoma. Immunosuppressive therapy with prednisone was initially started to eradicate anti-FVIII antibodies, subsequently boosted with cyclophosphamide and rituximab, these medications being also chosen to treat the associated indolent lymphoma. Bleeding symptoms were first tackled with limited benefit by using rFVIIa and then rescued using recombinant porcine FVIII. After a 6 month’s follow-up lymphoma and AHA were in remission and platelet function was improved. This case underlines the need of multiple and complex diagnostic and therapeutic approaches to rare acquired bleeding disorders associated with lymphoproliferative diseases.
KW - Acquired hemophilia A
KW - bleeding
KW - hemostatic disorders
KW - lymphoma
KW - platelet dysfunction
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U2 - 10.1080/09537104.2020.1869928
DO - 10.1080/09537104.2020.1869928
M3 - Article
AN - SCOPUS:85099351374
VL - 33
SP - 168
EP - 170
JO - Platelets
JF - Platelets
SN - 0953-7104
IS - 1
ER -