Lymphomatoid granulomatosis

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Lymphomatoid granulomatosis
Classification and external resources
ICD-O: 9766/1
DiseasesDB 33208
eMedicine med/1369
MeSH D008230

Lymphomatoid granulomatosis (LYG or LG) is a very rare lymphoproliferative disorder first characterized in 1972[1] with lymphomatoid meaning lymphoma-like and granulomatosis denoting one of its microscopic character, polymorphic lymphoid infiltrates and focal necrosis within it. While most commonly found in older patients, it has been observed in young people with a study identifying 47 cases of patients aged 0–18 years in the literature.[2] Males are found to be affected twice as often as females.[3]

Causes[edit]

The disease is believed to be induced by a combination of Epstein Barr virus infection and immunosuppression through immunosuppressive drugs, infections such as HIV or chronic viral hepatitis, autoimmune diseases such as rheumatoid arthritis, sarcoidosis, ulcerative colitis or endogenous T-cell defects.[4][5]

Pathophysiology[edit]

The onset of the disease results in proliferation of EBV-infected malignant B-cells and a cytotoxic T-cell response which in turn leads to organ infiltration and dysfunction of the affected organs. The disease typically always relapse after successful treatment due to inability of the immune system and current viral drugs to eliminate an EBV-infection. If the onset of the disease can be linked to use of immunosuppressive drugs then discontinuation of these drugs may hinder a relapse. Organs usually affected are the skin, lungs, central nervous system while liver and kidney are affected to lesser extent. The pulmonary complications are usually what leads to death, however, CNS involvement that affects up to one third of the patients can be very severe with mental status changes, ataxia, hemiparesis, seizures, unconsciousness and death, typically followed in that order.[4]

The disease has been seen to transform to diffuse large B-cell lymphoma[6] and while LYG is graded I-III based on the number of large EBV-positive B-cells, grade II and III can be considered as a variant of T-cell rich diffuse large B-cell lymphoma.[7][8]

Treatment[edit]

Treatment depends on the grade (I-III) but typically consist of cortisone, rituximab and chemotherapy (etoposide, vincristine, cyclophosphamide, doxorubicin). Methotrexate has been seen to induce LYG.[9][10] Interferon alpha has been used by the US National Cancer Institute with varying results.[11] In recent years hematopoietic stem cell transplantation has been performed on LYG-patients with relative good success; a 2013 study identifying 10 cases found that 8 patients survived the treatment and were disease free several years later. Two of the disease free patients later died, one from suicide and one from graft versus host disease after a second transplantation 4 years later. The remaining two patients died from sepsis after the transplantation.[12]

Prognosis[edit]

The current mortality is over 60% after 5 years. However, due to hematopoietic stem cell transplantation being performed only in recent years, this number could potentially be lowered in the future. In patients with CNS involvement, treatment with Interferon alpha at US National Cancer Institute resulted in complete remission in 90% of patients.[11]

See also[edit]

References[edit]

  1. ^ Liebow AA, Carrington CR, Friedman PJ (1972). "Lymphomatoid granulomatosis". Hum. Pathol. 3 (4): 457–558. doi:10.1016/S0046-8177(72)80005-4. PMID 4638966. 
  2. ^ Tacke ZC, Eikelenboom MJ, Vermeulen RJ, et al. (January 2014). "Childhood Lymphomatoid Granulomatosis: A Report of 2 Cases and Review of the Literature". Journal of Pediatric Hematology/oncology: 1. doi:10.1097/MPH.0000000000000090. PMID 24390446. 
  3. ^ Katzenstein AL, Doxtader E, Narendra S (December 2010). "Lymphomatoid granulomatosis: insights gained over 4 decades". The American Journal of Surgical Pathology 34 (12): e35–48. doi:10.1097/PAS.0b013e3181fd8781. PMID 21107080. 
  4. ^ a b Roschewski M, Wilson WH (2012). "Lymphomatoid granulomatosis". Cancer Journal 18 (5): 469–74. doi:10.1097/PPO.0b013e31826c5e19. PMID 23006954. 
  5. ^ Agarwal V, Agarwal A, Pal L, Misra R (February 2004). "Arthritis in lymphomatoid granulomatosis: report of a case and review of literature". Indian Journal of Medical Sciences 58 (2): 67–71. PMID 14993719. 
  6. ^ Boone JM, Zhang D, Fan F (2013). "Lymphomatoid granulomatosis: a case report with unique clinical and histopathologic features". Annals of Clinical and Laboratory Science 43 (2): 181–5. PMID 23694794. 
  7. ^ Tagliavini E, Rossi G, Valli R, et al. (August 2013). "Lymphomatoid granulomatosis: a practical review for pathologists dealing with this rare pulmonary lymphoproliferative process". Pathologica 105 (4): 111–6. PMID 24466760. 
  8. ^ Katzenstein AL, Doxtader E, Narendra S (December 2010). "Lymphomatoid granulomatosis: insights gained over 4 decades". The American Journal of Surgical Pathology 34 (12): e35–48. doi:10.1097/PAS.0b013e3181fd8781. PMID 21107080. 
  9. ^ Ochi N, Yamane H, Yamagishi T, Monobe Y, Takigawa N (July 2013). "Methotrexate-induced lymphoproliferative disease: Epstein-Barr virus-associated lymphomatoid granulomatosis". Journal of Clinical Oncology 31 (20): e348–50. doi:10.1200/JCO.2012.46.2770. PMID 23733760. 
  10. ^ Blanchart K, Paciencia M, Seguin A, et al. (January 2014). "Fatal pulmonary lymphomatoid granulomatosis in a patient taking methotrexate for rheumatoid arthritis". Minerva Anestesiologica 80 (1): 119–20. PMID 23857444. 
  11. ^ a b Dunleavy K, Roschewski M, Wilson WH (September 2012). "Lymphomatoid granulomatosis and other Epstein-Barr virus associated lymphoproliferative processes". Current Hematologic Malignancy Reports 7 (3): 208–15. doi:10.1007/s11899-012-0132-3. PMID 22814713. 
  12. ^ Siegloch K, Schmitz N, Wu HS, et al. (October 2013). "Hematopoietic stem cell transplantation in patients with lymphomatoid granulomatosis: a European group for blood and marrow transplantation report". Biology of Blood and Marrow Transplantation 19 (10): 1522–5. doi:10.1016/j.bbmt.2013.07.023. PMID 23948061.