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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/01/02 10:27:28」(JST)
[Wiki en表示]
| Lymphomatoid granulomatosis |
| Classification and external resources |
| Specialty |
Hematology and oncology |
| 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 characteristics, polymorphic lymphoid infiltrates and focal necrosis within it. While most commonly found in middle age patients,[2] it has been observed in young people with a study identifying 47 cases of patients aged 0–18 years in the literature.[3] Males are found to be affected twice as often as females.[4]
Contents
- 1 Causes
- 2 Pathophysiology
- 3 Treatment
- 4 Prognosis
- 5 See also
- 6 References
Causes
The disease is believed to be induced by a combination of Epstein Barr virus infection and immunosuppression through; immunosuppressive drugs (with case reports of methotrexate[5][6][7][8][9][10][11][12][13] and azathioprine[14][15]), infections such as HIV or chronic viral hepatitis or endogenous T-cell defects.[16]
Pathophysiology
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.[16]
The disease has been seen to transform to diffuse large B-cell lymphoma[17] 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.[18][4]
Treatment
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.[5][6] Interferon alpha has been used by the US National Cancer Institute with varying results.[19] 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.[20]
Prognosis
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.[19]
See also
- List of cutaneous conditions
References
- ^ Liebow, Averill A.; Carrington, Charles R.B.; Friedman, Paul J. (1972). "Lymphomatoid granulomatosis". Human Pathology 3 (4): 457–558. doi:10.1016/S0046-8177(72)80005-4. PMID 4638966.
- ^ Song, Joo Y.; Pittaluga, Stefania; Dunleavy, Kieron; Grant, Nicole; White, Therese; Jiang, Liuyan; Davies-Hill, Theresa; Raffeld, Mark; Wilson, Wyndham H.; Jaffe, Elaine S. (2015). "Lymphomatoid Granulomatosis—A Single Institute Experience". The American Journal of Surgical Pathology 39 (2): 141–56. doi:10.1097/PAS.0000000000000328. PMC 4293220. PMID 25321327.
- ^ Tacke, Zwanique C. A.; Eikelenboom, M. Judith; Vermeulen, R. Jeroen; Van Der Knaap, Marjo S.; Euser, Anne M.; Van Der Valk, Paul; Kaspers, Gertjan J. L. (2014). "Childhood Lymphomatoid Granulomatosis: A Report of 2 Cases and Review of the Literature". Journal of Pediatric Hematology/Oncology 36 (7): e416–22. doi:10.1097/MPH.0000000000000090. PMID 24390446.
- ^ a b Katzenstein, Anna-Luise A.; Doxtader, Erika; Narendra, Sonia (2010). "Lymphomatoid Granulomatosis". The American Journal of Surgical Pathology 34 (12): e35–48. doi:10.1097/PAS.0b013e3181fd8781. PMID 21107080.
- ^ a b Ochi, N.; Yamane, H.; Yamagishi, T.; Monobe, Y.; Takigawa, N. (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.
- ^ a b Blanchart, K; Paciencia, M; Seguin, A; Chantepie, S; Du Cheyron, D; Charbonneau, P; Galateau-Salle, F; Terzi, N (2014). "Fatal pulmonary lymphomatoid granulomatosis in a patient taking methotrexate for rheumatoid arthritis". Minerva anestesiologica 80 (1): 119–20. PMID 23857444.
- ^ Oiwa, Hiroshi; Mihara, Keichiro; Kan, Takanobu; Tanaka, Maiko; Shindo, Hajime; Kumagai, Kazuhiko; Sugiyama, Eiji (2014). "Grade 3 Lymphomatoid Granulomatosis in a Patient Receiving Methotrexate Therapy for Rheumatoid Arthritis". Internal Medicine 53 (16): 1873–5. doi:10.2169/internalmedicine.53.2593. PMID 25130128.
- ^ Yamakawa, T; Kurosawa, M; Yonezumi, M; Suzuki, S; Suzuki, H (2014). "メトトレキサート中止と脳病変への放射線照射が奏効したメトトレキサート関連リンパ腫様肉芽腫症" [Methotrexate-related lymphomatoid granulomatosis successfully treated with discontinuation of methotrexate and radiotherapy to brain]. Rinsho Ketsueki (in Japanese) 55 (3): 321–6. doi:10.11406/rinketsu.55.321 (inactive 2015-04-11). PMID 24681935.
- ^ Kobayashi, Shinichi; Kikuchi, Yuichi; Sato, Kimiya; Matsukuma, Susumu; Matsuki, Yasunori; Horikoshi, Hideyuki; Nagumo, Morichika; Kobayashi, Ayako; Masuoka, Kazuhiro; Kimura, Fumihiko; Oshima, Satoshi; Hakozaki, Yukiya; Kondo, Toshiro (2013). "Reversible iatrogenic, MTX-associated EBV-driven lymphoproliferation with histopathological features of a lymphomatoid granulomatosis in a patient with rheumatoid arthritis". Annals of Hematology 92 (11): 1561–4. doi:10.1007/s00277-013-1741-1. PMID 23529185.
- ^ Inaba, M; Ushijim, S; Hirata, N; Saisyoji, T; Kitaoka, M; Yoshinaga, T (2011). "Methotrexate-related lymphomatoid granulomatosis in a patient with rheumatoid arthritis". Nihon Kokyuki Gakkai zasshi 49 (8): 597–601. PMID 21894776.
- ^ Schalk, Enrico; Krogel, Christian; Scheinpflug, Katrin; Mohren, Martin (2009). "Lymphomatoid Granulomatosis in a Patient with Rheumatoid Arthritis Receiving Methotrexate: Successful Treatment with the Anti-CD20 Antibody Mabthera". Onkologie 32 (7): 440–1. doi:10.1159/000218356. PMID 19556825.
- ^ Shimada, K.; Matsui, T.; Kawakami, M.; Nakayama, H.; Ozawa, Y.; Mitomi, H.; Tohma, S. (2007). "Methotrexate‐related lymphomatoid granulomatosis: A case report of spontaneous regression of large tumours in multiple organs after cessation of methotrexate therapy in rheumatoid arthritis". Scandinavian Journal of Rheumatology 36 (1): 64–7. doi:10.1080/03009740600902403. PMID 17454938.
- ^ Kameda, Hideto; Okuyama, Ayumi; Tamaru, Jun-Ichi; Itoyama, Shinji; Iizuka, Atsushi; Takeuchi, Tsutomu (2007). "Lymphomatoid granulomatosis and diffuse alveolar damage associated with methotrexate therapy in a patient with rheumatoid arthritis". Clinical Rheumatology 26 (9): 1585. doi:10.1007/s10067-006-0480-2. PMID 17200802.
- ^ Barakat, Athar; Grover, Karan; Peshin, Rohit (2014). "Rituximab for pulmonary lymphomatoid granulomatosis which developed as a complication of methotrexate and azathioprine therapy for rheumatoid arthritis". SpringerPlus 3: 751. doi:10.1186/2193-1801-3-751 (inactive 2015-04-11). PMC 4320142. PMID 25674479.
- ^ Connors, William; Griffiths, Cameron; Patel, Jay; Belletrutti, Paul J (2014). "Lymphomatoid granulomatosis associated with azathioprine therapy in Crohn disease". BMC Gastroenterology 14: 127. doi:10.1186/1471-230X-14-127. PMC 4105046. PMID 25022612.
- ^ a b Roschewski, Mark; Wilson, Wyndham H. (2012). "Lymphomatoid Granulomatosis". The Cancer Journal 18 (5): 469–74. doi:10.1097/PPO.0b013e31826c5e19. PMID 23006954.
- ^ Boone, J. M.; 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.
- ^ Tagliavini, E; Rossi, G; Valli, R; Zanelli, M; Cadioli, A; Mengoli, M. C.; Bisagni, A; Cavazza, A; Gardini, G (2013). "Lymphomatoid granulomatosis: A practical review for pathologists dealing with this rare pulmonary lymphoproliferative process" (PDF). Pathologica 105 (4): 111–6. PMID 24466760.
- ^ a b Dunleavy, Kieron; Roschewski, Mark; Wilson, Wyndham H. (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.
- ^ Siegloch, Kristina; Schmitz, Norbert; Wu, Huei-Shan; Friedrichs, Birte; Van Imhoff, Gustaaf W.; Montoto, Silvia; Holler, Ernst; Ribera, Josep Maria; Delage, Robert; Dührsen, Ulrich; Castillo, Nerea del; Harrison, Beth; Dreger, Peter; Sureda, Anna; Working Party Lymphoma of the European Group for Blood Marrow Transplantation (EBMT) (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.
|
Hematological malignancy/leukemia histology (ICD-O 9590–9989, C81–C96, 200–208)
Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590–9739, 9800–9839)
|
|
B cell
(lymphoma,
leukemia)
(most CD19
|
By development/
marker |
| TdT+ |
- ALL (Precursor B acute lymphoblastic leukemia/lymphoma)
|
|
| CD5+ |
- mantle zone (Mantle cell)
|
|
| CD22+ |
- Prolymphocytic
- CD11c+ (Hairy cell leukemia)
|
|
| CD79a+ |
- germinal center/follicular B cell (Follicular
- Burkitt's
- GCB DLBCL
- Primary cutaneous follicular lymphoma)
- marginal zone/marginal-zone B cell (Splenic marginal zone
- MALT
- Nodal marginal zone
- Primary cutaneous marginal zone lymphoma)
|
|
| RS (CD15+, CD30+) |
- Classic Hodgkin's lymphoma (Nodular sclerosis)
- CD20+ (Nodular lymphocyte predominant Hodgkin's lymphoma)
|
|
PCDs/PP
(CD38+/CD138+) |
- see immunoproliferative immunoglobulin disorders
|
|
|
| By infection |
- KSHV (Primary effusion)
- EBV (Lymphomatoid granulomatosis
- Post-transplant lymphoproliferative disorder)
- HIV (AIDS-related lymphoma)
- Helicobacter pylori (MALT lymphoma)
|
|
| Cutaneous |
- Diffuse large B-cell lymphoma
- Intravascular large B-cell lymphoma
- Primary cutaneous marginal zone lymphoma
- Primary cutaneous immunocytoma
- Plasmacytoma
- Plasmacytosis
- Primary cutaneous follicular lymphoma
|
|
|
| T/NK |
T cell
(lymphoma,
leukemia)
(most CD3
|
By development/
marker |
- TdT+: ALL (Precursor T acute lymphoblastic leukemia/lymphoma)
- prolymphocyte (Prolymphocytic)
- CD30+ (Anaplastic large-cell lymphoma
- Lymphomatoid papulosis type A)
|
|
| Cutaneous |
| MF+variants |
- indolent: Mycosis fungoides
- Pagetoid reticulosis
- Granulomatous slack skin
aggressive: Sézary disease
- Adult T-cell leukemia/lymphoma
|
|
| Non-MF |
- CD30-: Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
- Pleomorphic T-cell lymphoma
- Lymphomatoid papulosis type B
- CD30+: CD30+ cutaneous T-cell lymphoma
- Secondary cutaneous CD30+ large cell lymphoma
- Lymphomatoid papulosis type A
|
|
|
| Other peripheral |
- Hepatosplenic
- Angioimmunoblastic
- Enteropathy-associated T-cell lymphoma
- Peripheral T-cell lymphoma-Not-Otherwise-Specified (Lennert lymphoma)
- Subcutaneous T-cell lymphoma
|
|
| By infection |
- HTLV-1 (Adult T-cell leukemia/lymphoma)
|
|
|
NK cell/
(most CD56) |
- Aggressive NK-cell leukemia
- Blastic NK cell lymphoma
|
|
| T or NK |
- EBV (Extranodal NK-T-cell lymphoma/Angiocentric lymphoma)
- Large granular lymphocytic leukemia
|
|
|
| Lymphoid+myeloid |
- Acute biphenotypic leukaemia
|
|
| Lymphocytosis |
- Lymphoproliferative disorders (X-linked lymphoproliferative disease
- Autoimmune lymphoproliferative syndrome)
- Leukemoid reaction
- Diffuse infiltrative lymphocytosis syndrome
|
|
|
| Cutaneous lymphoid hyperplasia |
- Cutaneous lymphoid hyperplasia
- with bandlike and perivascular patterns
- with nodular pattern
- Jessner lymphocytic infiltrate of the skin
|
|
|
Index of the immune system
|
|
| Description |
- Physiology
- cells
- autoantigens
- autoantibodies
- complement
- surface antigens
- IG receptors
|
|
| Disease |
- Allergies
- Immunodeficiency
- Immunoproliferative immunoglobulin disorders
- Hypersensitivity and autoimmune disorders
- Neoplasms and cancer
|
|
| Treatment |
- Procedures
- Drugs
- antihistamines
- immunostimulants
- immunosuppressants
- monoclonal antibodies
|
|
|
UpToDate Contents
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English Journal
- Lymphomatoid Granulomatosis: Two Different Phenotypes of Computed Tomography Findings.
- Yamanouchi T1, Kawanami S, Kamitani T, Sagiyama K, Yamasaki Y, Tanaka Y, Nagao M, Yabuuchi H, Hamada N, Okamoto T, Yamamoto H, Honda H.
- Journal of thoracic imaging.J Thorac Imaging.2016 Nov;31(6):W80-W82.
- PMID 27768635
- Brandão LA1, Castillo M2.
- Neuroimaging clinics of North America.Neuroimaging Clin N Am.2016 Nov;26(4):537-565. doi: 10.1016/j.nic.2016.06.005.
- There are 2 types of central nervous system lymphoma: primary and secondary. Both have variable imaging features making them diagnostic challenges. Furthermore, a patient's immune status significantly alters the imaging findings. Familiarity with typical appearances, variations, and common mimics ai
- PMID 27712793
- Lymphomatoid granulomatosis associated with azathioprine use for immune-mediated neuropathy.
- Burwick N1, Buckley SA2, Dong ZM3, Richard RE1.
- BMJ case reports.BMJ Case Rep.2016 Sep 26;2016. pii: bcr2016216930. doi: 10.1136/bcr-2016-216930.
- Lymphomatoid granulomatosis (LG) is a rare Epstein-Barr virus-driven lymphoproliferative disorder that generally arises in immunosuppressed patients and which can be life-threatening. Here we describe the development of pulmonary LG in a patient on long-term azathioprine for immune-mediated neuropat
- PMID 27671987
Japanese Journal
- 画像診断 リンパ腫様肉芽腫症様の画像経過をたどった肺原発リンパ上皮腫様癌の1例
- 押方 智也子,釣木澤 尚実,三井 公彦 [他]
- 日本呼吸器学会誌 = Annals of the Japanese Respiratory Society 2(3), 316-320, 2013-05-10
- NAID 40019737827
- EBV関連リンパ増殖性疾患の肺病変 (特集 血液疾患と肺病変)
Related Links
- LYGは呼吸器内科医が知っておかねばならない疾患の1つ。 リンパ腫様肉芽腫症（lymphomatoid granulomatosis） LYGは1972 年にLiebow らにより提唱された概念で、 組織学的壊死を伴い血管中心性の多彩な細胞浸潤を特徴とする ...
- Pulmonary lymphomatoid granulomatosis (PLG) is an uncommon pulmonary disorder characterized by multiple pulmonary nodular lesions with lymphocytic invasion of vascular walls on biopsy. Averill Liebow and colleagues first ...
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