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Acquired hemophilia A and delta storage pool deficiency in a patient with indolent non-Hodgkin lymphoma.
Rossio, Raffaella; Cassin, Ramona; Lecchi, Anna; La Marca, Silvia; Femia, Eti Alessandra; Novembrino, Cristina; Siboni, Simona M; Noto, Alessandro; Reda, Gianluigi; Peyvandi, Flora.
Afiliación
  • Rossio R; Department of Pathophysiology and Transplantation, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Cassin R; Hematology Unit, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Lecchi A; Department of Pathophysiology and Transplantation, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • La Marca S; Department of Pathophysiology and Transplantation, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Femia EA; Department of Pathophysiology and Transplantation, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Novembrino C; Department of Pathophysiology and Transplantation, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Siboni SM; Department of Pathophysiology and Transplantation, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Noto A; Hematology Unit, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Reda G; Hematology Unit, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Peyvandi F; Department of Pathophysiology and Transplantation, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
Platelets ; 33(1): 168-170, 2022 Jan 02.
Article en En | MEDLINE | ID: mdl-33426985
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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Linfoma no Hodgkin / Albinismo / Síndrome de Hermanski-Pudlak / Hemofilia A / Trastornos Hemorrágicos Límite: Aged / Humans / Male Idioma: En Revista: Platelets Asunto de la revista: HEMATOLOGIA Año: 2022 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Linfoma no Hodgkin / Albinismo / Síndrome de Hermanski-Pudlak / Hemofilia A / Trastornos Hemorrágicos Límite: Aged / Humans / Male Idioma: En Revista: Platelets Asunto de la revista: HEMATOLOGIA Año: 2022 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Reino Unido