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Intravascular large B-cell lymphoma

MedGen UID:
90821
Concept ID:
C0334660
Neoplastic Process
Synonyms: Angioendotheliomatosis; Angioendotheliomatosis proliferans systemisata; angioendotheliomatosis proliferans systemisata; Angiotropic Large Cell Lymphoma; Angiotropic large cell lymphoma; angiotropic large cell lymphoma; Angiotropic lymphoma; Angiotropic Lymphoma; angiotropic lymphoma; Intravascular B-cell lymphoma; Intravascular B-Cell Lymphoma; intravascular B-cell lymphoma; Intravascular Large B-Cell Lymphoma; intravascular large B-cell lymphoma; Intravascular lymphomatosis; intravascular lymphomatosis; IVBCL; Malignant angioendotheliomatosis; Malignant Angioendotheliomatosis; malignant angioendotheliomatosis; Tappeiner-Pfleger disease
SNOMED CT: Intravascular large B-cell lymphoma (399648005); Intravascular large B-cell lymphoma (1157162007); Angioendotheliomatosis (1157162007); Malignant angioendotheliomatosis (1157162007); Intravascular lymphomatosis (1157162007); Intravascular B-cell lymphoma (399648005); Angiotropic lymphoma (399648005); Angioendotheliomatosis (399648005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0020324
Orphanet: ORPHA98839

Definition

Intravascular large B-cell lymphoma (IVLBCL) is a very rare form of diffuse large B-cell lymphoma (see this term) characterized by the selective growth of lymphoma cells within the lumina of small blood vessels (especially the capillaries) that most often presents with a wide range of clinical manifestations (as potentially any tissue can be involved), with patients from Western countries more frequently manifesting with neurological and cutaneous symptoms while patients from Asian countries more frequently displaying hepatosplenomegaly and thrombocytopenia. IVLBCL is characterized by an absence of lymphadenopathy, an aggressive clinical course and a poor prognosis. [from ORDO]

Professional guidelines

PubMed

Chen ST, Barnes J, Duncan L
Semin Cutan Med Surg 2018 Mar;37(1):49-55. doi: 10.12788/j.sder.2018.014. PMID: 29719020
Pinter-Brown LC
Dermatol Clin 2015 Oct;33(4):835-40. doi: 10.1016/j.det.2015.05.003. PMID: 26433853
Shimada K, Kinoshita T, Naoe T, Nakamura S
Lancet Oncol 2009 Sep;10(9):895-902. doi: 10.1016/S1470-2045(09)70140-8. PMID: 19717091

Recent clinical studies

Etiology

Breakell T, Waibel H, Schliep S, Ferstl B, Erdmann M, Berking C, Heppt MV
Curr Oncol 2022 Apr 19;29(5):2909-2919. doi: 10.3390/curroncol29050237. PMID: 35621627Free PMC Article
Shimada K, Kiyoi H
Cancer Sci 2021 Oct;112(10):3953-3961. Epub 2021 Aug 9 doi: 10.1111/cas.15091. PMID: 34327781Free PMC Article
Ong YC, Kao HW, Chuang WY, Hung YS, Lin TL, Chang H, Kuo MC
Biomed J 2021 Aug;44(4):479-488. Epub 2020 Apr 25 doi: 10.1016/j.bj.2020.04.005. PMID: 32344119Free PMC Article
Shimada K, Yamaguchi M, Atsuta Y, Matsue K, Sato K, Kusumoto S, Nagai H, Takizawa J, Fukuhara N, Nagafuji K, Miyazaki K, Ohtsuka E, Okamoto M, Sugita Y, Uchida T, Kayukawa S, Wake A, Ennishi D, Kondo Y, Izumi T, Kin Y, Tsukasaki K, Hashimoto D, Yuge M, Yanagisawa A, Kuwatsuka Y, Shimada S, Masaki Y, Niitsu N, Kiyoi H, Suzuki R, Tokunaga T, Nakamura S, Kinoshita T
Lancet Oncol 2020 Apr;21(4):593-602. Epub 2020 Mar 11 doi: 10.1016/S1470-2045(20)30059-0. PMID: 32171071
Hope CB, Pincus LB
Clin Lab Med 2017 Sep;37(3):547-574. doi: 10.1016/j.cll.2017.05.009. PMID: 28802500

Diagnosis

Cheng JW, Li JH
N Engl J Med 2023 Dec 7;389(23):2188. doi: 10.1056/NEJMicm2307122. PMID: 38055255
Davis JW, Auerbach A, Crothers BA, Lewin E, Lynch DT, Teschan NJ, Schmieg JJ
Arch Pathol Lab Med 2022 Sep 1;146(9):1160-1167. doi: 10.5858/arpa.2021-0165-RA. PMID: 34979566
Shimada K, Yamaguchi M, Atsuta Y, Matsue K, Sato K, Kusumoto S, Nagai H, Takizawa J, Fukuhara N, Nagafuji K, Miyazaki K, Ohtsuka E, Okamoto M, Sugita Y, Uchida T, Kayukawa S, Wake A, Ennishi D, Kondo Y, Izumi T, Kin Y, Tsukasaki K, Hashimoto D, Yuge M, Yanagisawa A, Kuwatsuka Y, Shimada S, Masaki Y, Niitsu N, Kiyoi H, Suzuki R, Tokunaga T, Nakamura S, Kinoshita T
Lancet Oncol 2020 Apr;21(4):593-602. Epub 2020 Mar 11 doi: 10.1016/S1470-2045(20)30059-0. PMID: 32171071
Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ
Pathology 2020 Jan;52(1):53-67. Epub 2019 Nov 15 doi: 10.1016/j.pathol.2019.08.013. PMID: 31735345
Goyal A, LeBlanc RE, Carter JB
Hematol Oncol Clin North Am 2019 Feb;33(1):149-161. doi: 10.1016/j.hoc.2018.08.006. PMID: 30497672

Therapy

Breakell T, Waibel H, Schliep S, Ferstl B, Erdmann M, Berking C, Heppt MV
Curr Oncol 2022 Apr 19;29(5):2909-2919. doi: 10.3390/curroncol29050237. PMID: 35621627Free PMC Article
Shimada K, Kiyoi H
Cancer Sci 2021 Oct;112(10):3953-3961. Epub 2021 Aug 9 doi: 10.1111/cas.15091. PMID: 34327781Free PMC Article
Ong YC, Kao HW, Chuang WY, Hung YS, Lin TL, Chang H, Kuo MC
Biomed J 2021 Aug;44(4):479-488. Epub 2020 Apr 25 doi: 10.1016/j.bj.2020.04.005. PMID: 32344119Free PMC Article
Shimada K, Yamaguchi M, Atsuta Y, Matsue K, Sato K, Kusumoto S, Nagai H, Takizawa J, Fukuhara N, Nagafuji K, Miyazaki K, Ohtsuka E, Okamoto M, Sugita Y, Uchida T, Kayukawa S, Wake A, Ennishi D, Kondo Y, Izumi T, Kin Y, Tsukasaki K, Hashimoto D, Yuge M, Yanagisawa A, Kuwatsuka Y, Shimada S, Masaki Y, Niitsu N, Kiyoi H, Suzuki R, Tokunaga T, Nakamura S, Kinoshita T
Lancet Oncol 2020 Apr;21(4):593-602. Epub 2020 Mar 11 doi: 10.1016/S1470-2045(20)30059-0. PMID: 32171071
Shimada K, Kinoshita T, Naoe T, Nakamura S
Lancet Oncol 2009 Sep;10(9):895-902. doi: 10.1016/S1470-2045(09)70140-8. PMID: 19717091

Prognosis

Breakell T, Waibel H, Schliep S, Ferstl B, Erdmann M, Berking C, Heppt MV
Curr Oncol 2022 Apr 19;29(5):2909-2919. doi: 10.3390/curroncol29050237. PMID: 35621627Free PMC Article
Fujikura K, Yamashita D, Yoshida M, Ishikawa T, Itoh T, Imai Y
J Clin Pathol 2021 Apr;74(4):244-250. Epub 2020 Aug 6 doi: 10.1136/jclinpath-2020-206573. PMID: 32763919
Valenzuela J, Yeaney GA, Hsi ED, Azzato EM, Peereboom DM, Singh AD
Surv Ophthalmol 2020 May-Jun;65(3):361-370. Epub 2019 Oct 30 doi: 10.1016/j.survophthal.2019.10.008. PMID: 31676312
Goyal A, LeBlanc RE, Carter JB
Hematol Oncol Clin North Am 2019 Feb;33(1):149-161. doi: 10.1016/j.hoc.2018.08.006. PMID: 30497672
Ponzoni M, Campo E, Nakamura S
Blood 2018 Oct 11;132(15):1561-1567. Epub 2018 Aug 15 doi: 10.1182/blood-2017-04-737445. PMID: 30111607

Clinical prediction guides

Gonzalez-Farre B, Ramis-Zaldivar JE, Castrejón de Anta N, Rivas-Delgado A, Nadeu F, Salmeron-Villalobos J, Enjuanes A, Karube K, Balagué O, Cobo F, Kelleher N, Victoria I, Veloza L, Teixido C, Giné E, Lopez-Guerra M, Quintanilla-Martinez L, Lopez-Guillermo A, Salaverria I, Campo E
Am J Surg Pathol 2023 Feb 1;47(2):202-211. Epub 2022 Oct 12 doi: 10.1097/PAS.0000000000001978. PMID: 36221796Free PMC Article
Fujikura K, Yamashita D, Yoshida M, Ishikawa T, Itoh T, Imai Y
J Clin Pathol 2021 Apr;74(4):244-250. Epub 2020 Aug 6 doi: 10.1136/jclinpath-2020-206573. PMID: 32763919
Valenzuela J, Yeaney GA, Hsi ED, Azzato EM, Peereboom DM, Singh AD
Surv Ophthalmol 2020 May-Jun;65(3):361-370. Epub 2019 Oct 30 doi: 10.1016/j.survophthal.2019.10.008. PMID: 31676312
Ponzoni M, Campo E, Nakamura S
Blood 2018 Oct 11;132(15):1561-1567. Epub 2018 Aug 15 doi: 10.1182/blood-2017-04-737445. PMID: 30111607
Pinter-Brown LC
Dermatol Clin 2015 Oct;33(4):835-40. doi: 10.1016/j.det.2015.05.003. PMID: 26433853

Recent systematic reviews

Puente-Hernandez M, Rivero-de-Aguilar A, Varela-Lema L
J Neurol Sci 2023 Mar 15;446:120580. Epub 2023 Feb 4 doi: 10.1016/j.jns.2023.120580. PMID: 36764185

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