Lymphomatoid granulomatosis with pulmonary and gastrointestinal involvement.
We present a rare case of grade II lymphomatoid granulomatosis (LYG) with pulmonary and gastrointestinal involvement. LYG is considered an Epstein-Barr virus-driven lymphoproliferative disorder that often presents with multiple nodular lesions in the lungs and sometimes involvement of skin and the central nervous system. Although the aetiology is unknown, it is associated with the use of immunosuppressives. Involvement of other organ systems is very rare. We successfully treated our patients with 6 cycles of R-CHOP and autologous stem cell transplantation with a major response at 20 months follow-up.
Department of Pulmonology, Sint Franciscus Vlietland Groep, Rotterdam, The Netherlands.
J H
JH
Kappen
Department of Haematology, Sint Franciscus Vlietland Groep, Rotterdam, The Netherlands.
H C T
HC
van Zaanen
Department of Internal Medicine, Sint Franciscus Vlietland Groep, Rotterdam, The Netherlands.
S M
SM
Snelder
Department of Gastroenterology and Hepatology, Sint Franciscus Vlietland Groep, Rotterdam, The Netherlands.
A J P
AJ
van Tilburg
Department of Pulmonology, Sint Franciscus Vlietland Groep, Rotterdam, The Netherlands.
A
A
Rudolphus
D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D005334:Fever / Q000209:etiology; D006396:Hematemesis / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D004854:Herpesvirus 4, Human; D006801:Humans; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology*; D008230:Lymphomatoid Granulomatosis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D000069283:Rituximab / Q000627:therapeutic use; D013274:Stomach Neoplasms / Q000175:diagnosis* / Q000188:drug therapy; D014750:Vincristine / Q000627:therapeutic use; D015431:Weight Loss
D000970:Antineoplastic Agents; D000069283:Rituximab; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone