Lymphomatoid Granulomatosis Medication

  • Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: Jan 18, 2012
 
 

Medication Summary

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.
  • Rituximab[9, 10]
    • 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.[11, 12, 13] These case reports have involved patients with advanced disease refractory to other medical therapies.[14, 15]
    • Although some of these case reports appear promising, larger studies are needed to substantiate the efficacy of rituximab in the treatment of lymphomatoid granulomatosis.
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Contributor Information and Disclosures
Author

Nader Kamangar, MD, FACP, FCCP, FCCM  Associate Professor of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of California, Los Angeles, David Geffen School of Medicine, Olive View-UCLA Medical Center; Associate Program Director, Pulmonary and Critical Care Multi-Campus Fellowship Program, Cedars-Sinai/West Los Angeles Veterans Affairs/Los Angeles Kaiser Permanente/Olive View-UCLA Medical Center; Site Director, Pulmonary/Critical Care Fellowship Program, Olive View-UCLA Medical Center

Nader Kamangar, MD, FACP, FCCP, FCCM 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.

Specialty Editor Board

Ryland P Byrd Jr, MD  Professor, Department of Internal Medicine, Division of Pulmonary Medicine and Critical Care Medicine, Program Director of Pulmonary Diseases and Critical Care Medicine Fellowship, East Tennessee State University, James H Quillen College of Medicine; Medical Director of Respiratory Therapy, 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 and American Thoracic Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St 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, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

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.

References

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  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].

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  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. Castrale C, El Haggan W, Chapon F, Reman O, Lobbedez T, Ryckelynck JP, et al. Lymphomatoid granulomatosis treated successfully with rituximab in a renal transplant patient. J Transplant. 2011;2011:865957. [Medline]. [Full Text].

  11. 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].

  12. 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].

  13. 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].

  14. 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].

  15. 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].

  16. 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].

  17. Chung JH, Wu CC, Gilman MD, Palmer EL, Hasserjian RP, Shepard JA. Lymphomatoid granulomatosis: CT and FDG-PET findings. Korean J Radiol. Nov 2011;12(6):671-8. [Medline]. [Full Text].

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

  19. 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].

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

  21. 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].

 
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Contrast-enhanced chest CT scan showing poorly defined nodular peribronchovascular infiltrates with air-bronchograms.
Contrast-enhanced chest CT scan showing poorly defined nodular peribronchovascular infiltrates with air-bronchograms.
 
 
 
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