eMedicine Specialties > Pulmonology > Idiopathic Lung Disorders

Lymphomatoid Granulomatosis: Treatment & Medication

Author: Nader Kamangar, MD, FACP, FCCP, FAASM,, Associate Professor of Clinical Medicine, Director of Hospitalist/Intensivist Program, Division of Pulmonary, Critical Care and Sleep Medicine, David Geffen School of Medicine at University of California Los Angeles; Associate Director, Combined Pulmonary and Critical Care Fellowship Program, Cedars-Sinai/Olive View-UCLA/West Los Angeles Veterans Affairs Medical Center
Coauthor(s): Anthony W O'Regan, MD, Clinical Lecturer of Medicine, Department of Internal Medicine, Section of Respiratory Medicine, National University of Ireland, Galway; Adjunct Professor of Medicine, Boston University Medical Center
Contributor Information and Disclosures

Updated: Apr 16, 2009

Treatment

Medical Care

The therapeutic approach and optimal management have not been well defined. In several studies, therapy has ranged from observation to treatment with prednisone or chemotherapy. In the largest reported study of 152 patients, no significant difference in mortality or disease-free survival was found in treatment options, and the mortality rate exceeded 50%. New therapeutic approaches are necessary. In view of the association of lymphomatoid granulomatosis (LYG) with EBV and the similarity to posttransplant lymphoma, the use of antiviral drugs with minimal immunosuppressive therapy is advocated.

  • Patients with a benign course require no treatment. Spontaneous remission has been reported.
  • Corticosteroids, with or without chemotherapy, may be recommended.
    • Treat symptomatic or progressive disease.
    • In general, therapy involves prednisone with antineoplastic agents (eg, cyclophosphamide).
    • More than 50% of patients with lymphomatoid granulomatosis respond to treatment.
    • Recurrence is usual and may include refractory disease or progression to high-grade lymphoma (13-47%).
    • When lymphomatoid granulomatosis progresses to high-grade lymphoma, combination antilymphoma regimens are used, but response rates are poor at this stage.
  • Localized disease may respond to radiotherapy.
  • Surgical resection of isolated pulmonary masses followed by chemotherapy has been associated with disease-free survival for at least 2 years.
  • Other treatment options include ganciclovir, interferon alfa-2, or, depending on histologic grade, combination chemotherapy.6,7

Medication

No well-studied effective treatment exists for this disease. Apart from immunosuppressive regimens, experimental therapeutic options include interferon alfa-2b and ganciclovir.

  • Interferon alfa-2b
    • The association with EBV and posttransplant lymphoma prompted a group to treat lymphomatoid granulomatosis with interferon alfa-2b.8
    • This drug has antiviral, antiproliferative, and immunomodulatory effects.
    • All 4 patients who were treated responded, with 3 patients achieving a complete response at 3 months.
    • Most patients responded to a dosage of 10 million units administered subcutaneously 3 times a week.
    • At follow-up of 36-60 months, 3 patients remained disease-free.
  • Ganciclovir
    • A patient with lymphomatoid granulomatosis and positive EBV serology post–stem cell transplant for multiple myeloma was reported to have complete radiologic remission following 2 weeks of ganciclovir therapy.
    • It should be noted that immune reconstitution also coincided with recovery.
  • Rituximab9
    • Rituximab is a monoclonal antibody that targets the B-cell surface molecule CD20. Several case reports of the efficacy of monoclonal antibodies have been published.10,11,12 These case reports have involved patients with advanced disease refractory to other medical therapies.13,14
    • Although some of these case reports appear promising, larger studies are needed to substantiate the efficacy of rituximab in the treatment of lymphomatoid granulomatosis.

More on Lymphomatoid Granulomatosis

Overview: Lymphomatoid Granulomatosis
Differential Diagnoses & Workup: Lymphomatoid Granulomatosis
Treatment & Medication: Lymphomatoid Granulomatosis
Follow-up: Lymphomatoid Granulomatosis
Multimedia: Lymphomatoid Granulomatosis
References
[ CLOSE WINDOW ]

References

  1. Liebow AA, Carrington CR, Friedman PJ. Lymphomatoid granulomatosis. Hum Pathol. Dec 1972;3(4):457-558. [Medline].

  2. Araki F, Mimura T, Fukuoka S, et al. Primary orbital lymphomatoid granulomatosis. Br J Ophthalmol. Apr 2009;93(4):554-6. [Medline].

  3. Pereira AC, Oliveira TM, Nomelini RS, Saldanha JC, Calil MC, Murta EF. Lymphomatoid granulomatosis of the vulva: case report with immunohistochemical analysis. J Obstet Gynaecol. Apr 2009;29(3):255-6. [Medline].

  4. Canovas D, Vinas J, Martinez J, Viguera M, Estela J, Ribera G. [Lymphomatoid granulomatosis with exclusively neurological involvement.]. Neurologia. Mar 2009;24(2):140-1. [Medline].

  5. Lucantoni C, De Bonis P, Doglietto F, et al. Primary cerebral lymphomatoid granulomatosis: report of four cases and literature review. J Neurooncol. Mar 26 2009;[Medline].

  6. Lemieux J, Bernier V, Martel N, Delage R. Autologous hematopoietic stem cell transplantation for refractory lymphomatoid granulomatosis. Hematology. Dec 2002;7(6):355-8. [Medline].

  7. Rao R, Vugman G, Leslie WT, Loew J, Venugopal P. Lymphomatoid granulomatosis treated with rituximab and chemotherapy. Clin Adv Hematol Oncol. Nov 2003;1(11):658-60; discussion 660. [Medline].

  8. Wilson WH, Kingma DW, Raffeld M, Wittes RE, Jaffe ES. Association of lymphomatoid granulomatosis with Epstein-Barr viral infection of B lymphocytes and response to interferon-alpha 2b. Blood. Jun 1 1996;87(11):4531-7. [Medline].

  9. Polizzotto MN, Dawson MA, Opat SS. Failure of rituximab monotherapy in lymphomatoid granulomatosis. Eur J Haematol. Aug 2005;75(2):172-3. [Medline].

  10. Jordan K, Grothey A, Grothe W, Kegel T, Wolf HH, Schmoll HJ. Successful treatment of mediastinal lymphomatoid granulomatosis with rituximab monotherapy. Eur J Haematol. Mar 2005;74(3):263-6. [Medline].

  11. Sebire NJ, Haselden S, Malone M, Davies EG, Ramsay AD. Isolated EBV lymphoproliferative disease in a child with Wiskott-Aldrich syndrome manifesting as cutaneous lymphomatoid granulomatosis and responsive to anti-CD20 immunotherapy. J Clin Pathol. Jul 2003;56(7):555-7. [Medline].

  12. Zaidi A, Kampalath B, Peltier WL, Vesole DH. Successful treatment of systemic and central nervous system lymphomatoid granulomatosis with rituximab. Leuk Lymphoma. Apr 2004;45(4):777-80. [Medline].

  13. Jaffre S, Jardin F, Dominique S, Duet E, Hubscher P, Genevois A. Fatal haemoptysis in a case of lymphomatoid granulomatosis treated with rituximab. Eur Respir J. Mar 2006;27(3):644-6. [Medline].

  14. Ishiura H, Morikawa M, Hamada M, Watanabe T, Kako S, Chiba S, et al. Lymphomatoid granulomatosis involving central nervous system successfully treated with rituximab alone. Arch Neurol. May 2008;65(5):662-5. [Medline].

  15. Bolaman Z, Kadiköylü G, Polatli M, Barutca S, Culhaci N, Sentürk T. Migratory nodules in the lung: lymphomatoid granulomatosis. Leuk Lymphoma. Jan 2003;44(1):197-200. [Medline].

  16. Katzenstein AL, Carrington CB, Liebow AA. Lymphomatoid granulomatosis: a clinicopathologic study of 152 cases. Cancer. Jan 1979;43(1):360-73. [Medline].

  17. McNiff JM, Cooper D, Howe G, Crotty PL, Tallini G, Crouch J, et al. Lymphomatoid granulomatosis of the skin and lung. An angiocentric T-cell-rich B-cell lymphoproliferative disorder. Arch Dermatol. Dec 1996;132(12):1464-70. [Medline].

  18. Myers JL. Lymphomatoid granulomatosis: past, present, ... future?. Mayo Clin Proc. Feb 1990;65(2):274-8. [Medline].

  19. Wu SM, Min Y, Ostrzega N, Clements PJ, Wong AL. Lymphomatoid granulomatosis: a rare mimicker of vasculitis. J Rheumatol. Nov 2005;32(11):2242-5. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

lymphomatoid granulomatosis, angiocentric lymphoproliferative lesion, LYG, Wegener granulomatosis, Wegener's granulomatosis, WG, B-cell lymphoma, pulmonary angiitis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Nader Kamangar, MD, FACP, FCCP, FAASM,, Associate Professor of Clinical Medicine, Director of Hospitalist/Intensivist Program, Division of Pulmonary, Critical Care and Sleep Medicine, David Geffen School of Medicine at University of California Los Angeles; Associate Director, Combined Pulmonary and Critical Care Fellowship Program, Cedars-Sinai/Olive View-UCLA/West Los Angeles Veterans Affairs Medical Center
Nader Kamangar, MD, FACP, FCCP, FAASM, is a member of the following medical societies: American Academy of Sleep Medicine, American Association of Bronchology, American College of Chest Physicians, American College of Physicians, American Lung Association, American Medical Association, American Thoracic Society, California Thoracic Society, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Anthony W O'Regan, MD, Clinical Lecturer of Medicine, Department of Internal Medicine, Section of Respiratory Medicine, National University of Ireland, Galway; Adjunct Professor of Medicine, Boston University Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Ryland P Byrd Jr, MD, Professor, Department of Internal Medicine, Division of Pulmonary Medicine and Critical Care Medicine, James H Quillen College of Medicine, East Tennessee State University; Chief of Pulmonary Medicine, Medical Director of Respiratory Therapy, Intensive Care Unit, Program Director of Pulmonary Diseases and Critical Care Medicine Fellowship, James H Quillen Veterans Affairs Medical Center
Ryland P Byrd Jr, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

RELATED EMEDICINE ARTICLES
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.