Lymphomatoid Granulomatosis Follow-up
- Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Zab Mosenifar, MD more...
Complications
- Transformation into high-grade lymphoma may occur (13-47%).
- Progressive respiratory disease with increasing respiratory failure may result in pneumothorax, infection, and hemorrhage.
- Hemoptysis, occasionally massive, may complicate cavitation of the diseased pulmonary tissue.
- Pneumothorax may occur.
- Opportunistic infections may develop.
- Seizures, mental status changes, mononeuropathy, or diabetes insipidus may complicate progressive neurological disease.
Prognosis
- The median survival from diagnosis is 14 months. More than 60% of patients die within 5 years.
- The cause of death is usually extensive destruction of the pulmonary parenchyma, resulting in respiratory failure, sepsis, and, occasionally, massive hemoptysis.
- Poor prognostic indicators include an age younger than 30 years, neurological or hepatic involvement, leukopenia or pancytopenia, and anergy.
Liebow AA, Carrington CR, Friedman PJ. Lymphomatoid granulomatosis. Hum Pathol. Dec 1972;3(4):457-558. [Medline].
Araki F, Mimura T, Fukuoka S, et al. Primary orbital lymphomatoid granulomatosis. Br J Ophthalmol. Apr 2009;93(4):554-6. [Medline].
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].
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].
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].
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].
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].
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].
Polizzotto MN, Dawson MA, Opat SS. Failure of rituximab monotherapy in lymphomatoid granulomatosis. Eur J Haematol. Aug 2005;75(2):172-3. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
Katzenstein AL, Carrington CB, Liebow AA. Lymphomatoid granulomatosis: a clinicopathologic study of 152 cases. Cancer. Jan 1979;43(1):360-73. [Medline].
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].
Myers JL. Lymphomatoid granulomatosis: past, present, ... future?. Mayo Clin Proc. Feb 1990;65(2):274-8. [Medline].
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].

