Lymphomatoid Granulomatosis

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Jean-pierre Guignard - One of the best experts on this subject based on the ideXlab platform.

  • Lymphomatoid Granulomatosis in a renal transplant patient
    Pediatric Nephrology, 2003
    Co-Authors: François Cachat, Kathleen Meagher-villemure, Jean-pierre Guignard
    Abstract:

    Lymphomatoid Granulomatosis is a rare angiocentric and angiodestructive pulmonary angiitis considered as a variant of the lymphoproliferative disorder group. Patients with organ transplantation are at an increased risk for post-transplant lymphoproliferative disorders secondary to their immunosuppression. However, Lymphomatoid Granulomatosis has rarely been described in patients with renal transplantation. It often presents with severe pulmonary signs. We describe a case whose initial presentation was an isolated VIth nerve palsy. We review the radiological and pathological findings and discuss the etiopathogenesis and therapeutic options of this particular lymphoproliferative disorder. With careful and stepwise reduction in her immunosuppression, our patient showed a complete disappearance of her Lymphomatoid Granulomatosis, and she is clinically well more than 3 years after the diagnosis, with good kidney function.

François Cachat - One of the best experts on this subject based on the ideXlab platform.

  • Lymphomatoid Granulomatosis in a renal transplant patient
    Pediatric Nephrology, 2003
    Co-Authors: François Cachat, Kathleen Meagher-villemure, Jean-pierre Guignard
    Abstract:

    Lymphomatoid Granulomatosis is a rare angiocentric and angiodestructive pulmonary angiitis considered as a variant of the lymphoproliferative disorder group. Patients with organ transplantation are at an increased risk for post-transplant lymphoproliferative disorders secondary to their immunosuppression. However, Lymphomatoid Granulomatosis has rarely been described in patients with renal transplantation. It often presents with severe pulmonary signs. We describe a case whose initial presentation was an isolated VIth nerve palsy. We review the radiological and pathological findings and discuss the etiopathogenesis and therapeutic options of this particular lymphoproliferative disorder. With careful and stepwise reduction in her immunosuppression, our patient showed a complete disappearance of her Lymphomatoid Granulomatosis, and she is clinically well more than 3 years after the diagnosis, with good kidney function.

Stephen J. Schuster - One of the best experts on this subject based on the ideXlab platform.

  • Successful treatment of relapsed Lymphomatoid Granulomatosis with bexarotene.
    Cancer biology & therapy, 2008
    Co-Authors: Sigrid Berg, Lisa H. Downs, Drew A. Torigian, Elise A. Chong, Donald E. Tsai, Mariusz A. Wasik, Stephen J. Schuster
    Abstract:

    Lymphomatoid Granulomatosis is a rare lymphoproliferative disorder involving the lungs, skin and other organs. Advanced-stage disease does not tend to respond well to cytotoxic chemotherapy and is associated with a poor prognosis. We present a case of successful treatment of relapsed Lymphomatoid Granulomatosis with bexarotene, a novel retinoid agent.

Kathleen Meagher-villemure - One of the best experts on this subject based on the ideXlab platform.

  • Lymphomatoid Granulomatosis in a renal transplant patient
    Pediatric Nephrology, 2003
    Co-Authors: François Cachat, Kathleen Meagher-villemure, Jean-pierre Guignard
    Abstract:

    Lymphomatoid Granulomatosis is a rare angiocentric and angiodestructive pulmonary angiitis considered as a variant of the lymphoproliferative disorder group. Patients with organ transplantation are at an increased risk for post-transplant lymphoproliferative disorders secondary to their immunosuppression. However, Lymphomatoid Granulomatosis has rarely been described in patients with renal transplantation. It often presents with severe pulmonary signs. We describe a case whose initial presentation was an isolated VIth nerve palsy. We review the radiological and pathological findings and discuss the etiopathogenesis and therapeutic options of this particular lymphoproliferative disorder. With careful and stepwise reduction in her immunosuppression, our patient showed a complete disappearance of her Lymphomatoid Granulomatosis, and she is clinically well more than 3 years after the diagnosis, with good kidney function.

Hans-joachim Schmoll - One of the best experts on this subject based on the ideXlab platform.

  • Successful treatment of mediastinal Lymphomatoid Granulomatosis with rituximab monotherapy.
    European journal of haematology, 2005
    Co-Authors: Karin Jordan, Axel Grothey, Wilfried Grothe, T. Kegel, H.-h. Wolf, Hans-joachim Schmoll
    Abstract:

    Lymphomatoid Granulomatosis is a rare Epstein-Barr virus (EBV)-positive-B-cell lymphoproliferative disorder. Treatment options include corticosteroids, antiviral therapy, interferon-alpha and chemotherapy. However, long-term prognosis is poor and no therapeutic standard has been established yet. In a 21-year-old woman, a biopsy of mediastinal mass revealed Lymphomatoid Granulomatosis. Combined therapy with valganciclovir and interferon-alpha proved ineffective. In view of the CD20 expression of the tumor cells, monotherapy with rituximab was intiated. After 3 months a complete remission was achieved. Rituximab was continued for another 6 months with subsequent consolidation radiotherapy. This is the first report of an enduring complete remission (20 months) of a non-CNS Lymphomatoid Granulomatosis treated with rituximab.