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Hepatic hemophagocytosis

MedGen UID:
1842040
Concept ID:
C5826669
Finding
HPO: HP:0034797

Definition

Phagocytosis of erythrocytes, lymphocytes or other hematopoietic precursors by histiocytes or macrophages observed in the liver. [from HPO]

Term Hierarchy

Conditions with this feature

Autoimmune disease, multisystem, infantile-onset, 3
MedGen UID:
1841236
Concept ID:
C5830600
Disease or Syndrome
Infantile-onset multisystem autoimmune disease-3 (ADMIO3) is an autosomal recessive disorder of immune dysregulation characterized by the onset of various systemic autoimmune manifestations in the first months or years of life. Features may include hypothyroidism, type 1 diabetes mellitus, systemic inflammatory manifestations (fever, hepatomegaly), and autoimmune cytopenias. Laboratory studies show normal levels of T, B, and NK cells, but CD4+ (see 186940) T cells demonstrate hyperproliferation when stimulated in vitro (Janssen et al., 2022). For a discussion of genetic heterogeneity of ADMIO, see ADMIO1 (615952).

Professional guidelines

PubMed

Lacaille F, Fournet JC, Blanche S
Pediatr Infect Dis J 1999 Feb;18(2):143-7. doi: 10.1097/00006454-199902000-00011. PMID: 10048686

Recent clinical studies

Etiology

Lichtenstein DA, Schischlik F, Shao L, Steinberg SM, Yates B, Wang HW, Wang Y, Inglefield J, Dulau-Florea A, Ceppi F, Hermida LC, Stringaris K, Dunham K, Homan P, Jailwala P, Mirazee J, Robinson W, Chisholm KM, Yuan C, Stetler-Stevenson M, Ombrello AK, Jin J, Fry TJ, Taylor N, Highfill SL, Jin P, Gardner RA, Shalabi H, Ruppin E, Stroncek DF, Shah NN
Blood 2021 Dec 16;138(24):2469-2484. doi: 10.1182/blood.2021011898. PMID: 34525183Free PMC Article
De Gottardi J, Montani M, Angelillo-Scherrer A, Rovo A, Berzigotti A
PLoS One 2019;14(12):e0226899. Epub 2019 Dec 30 doi: 10.1371/journal.pone.0226899. PMID: 31887162Free PMC Article
Zahir H, Belkhir J, Mouhib H, Ait Ameur M, Chakour M
Turk J Med Sci 2019 Oct 24;49(5):1332-1335. doi: 10.3906/sag-1812-65. PMID: 31648438Free PMC Article
Aronson IK, Worobec SM
Dermatol Ther 2010 Jul-Aug;23(4):389-402. doi: 10.1111/j.1529-8019.2010.01339.x. PMID: 20666826
Favara BE
Semin Diagn Pathol 1992 Feb;9(1):63-74. PMID: 1561489

Diagnosis

Lichtenstein DA, Schischlik F, Shao L, Steinberg SM, Yates B, Wang HW, Wang Y, Inglefield J, Dulau-Florea A, Ceppi F, Hermida LC, Stringaris K, Dunham K, Homan P, Jailwala P, Mirazee J, Robinson W, Chisholm KM, Yuan C, Stetler-Stevenson M, Ombrello AK, Jin J, Fry TJ, Taylor N, Highfill SL, Jin P, Gardner RA, Shalabi H, Ruppin E, Stroncek DF, Shah NN
Blood 2021 Dec 16;138(24):2469-2484. doi: 10.1182/blood.2021011898. PMID: 34525183Free PMC Article
De Gottardi J, Montani M, Angelillo-Scherrer A, Rovo A, Berzigotti A
PLoS One 2019;14(12):e0226899. Epub 2019 Dec 30 doi: 10.1371/journal.pone.0226899. PMID: 31887162Free PMC Article
Zahir H, Belkhir J, Mouhib H, Ait Ameur M, Chakour M
Turk J Med Sci 2019 Oct 24;49(5):1332-1335. doi: 10.3906/sag-1812-65. PMID: 31648438Free PMC Article
Aronson IK, Worobec SM
Dermatol Ther 2010 Jul-Aug;23(4):389-402. doi: 10.1111/j.1529-8019.2010.01339.x. PMID: 20666826
Favara BE
Semin Diagn Pathol 1992 Feb;9(1):63-74. PMID: 1561489

Therapy

Jacquot R, Gerfaud-Valentin M, Lega JC, Becker A, Jamilloux Y, Seve P
Rev Med Interne 2022 Oct;43(10):617-621. Epub 2022 Jun 10 doi: 10.1016/j.revmed.2022.05.011. PMID: 35697578
Lichtenstein DA, Schischlik F, Shao L, Steinberg SM, Yates B, Wang HW, Wang Y, Inglefield J, Dulau-Florea A, Ceppi F, Hermida LC, Stringaris K, Dunham K, Homan P, Jailwala P, Mirazee J, Robinson W, Chisholm KM, Yuan C, Stetler-Stevenson M, Ombrello AK, Jin J, Fry TJ, Taylor N, Highfill SL, Jin P, Gardner RA, Shalabi H, Ruppin E, Stroncek DF, Shah NN
Blood 2021 Dec 16;138(24):2469-2484. doi: 10.1182/blood.2021011898. PMID: 34525183Free PMC Article
Díaz LA, Idalsoaga F, Cannistra M, Candia R, Cabrera D, Barrera F, Soza A, Graham R, Riquelme A, Arrese M, Leise MD, Arab JP
World J Gastroenterol 2020 Dec 28;26(48):7693-7706. doi: 10.3748/wjg.v26.i48.7693. PMID: 33505145Free PMC Article
Hu S, Bansal P, Lynch D, Rojas Hernandez CM, Dayao Z
J Med Case Rep 2016 Dec 20;10(1):365. doi: 10.1186/s13256-016-1143-9. PMID: 27998299Free PMC Article
Rajam L, Prasad V, Yatheesha BL
Indian J Pediatr 2008 Dec;75(12):1261-3. Epub 2008 Sep 22 doi: 10.1007/s12098-008-0170-y. PMID: 18810357

Prognosis

Masood M, Siddique A, Krishnamoorthi R, Kozarek RA
Adv Ther 2024 Feb;41(2):553-566. Epub 2023 Dec 25 doi: 10.1007/s12325-023-02768-8. PMID: 38145441
Sun Y, Blieden C, Merritt BY, Sosa R, Rivero G
J Med Case Rep 2021 Mar 1;15(1):98. doi: 10.1186/s13256-020-02623-2. PMID: 33648567Free PMC Article
De Gottardi J, Montani M, Angelillo-Scherrer A, Rovo A, Berzigotti A
PLoS One 2019;14(12):e0226899. Epub 2019 Dec 30 doi: 10.1371/journal.pone.0226899. PMID: 31887162Free PMC Article
Aronson IK, Worobec SM
Dermatol Ther 2010 Jul-Aug;23(4):389-402. doi: 10.1111/j.1529-8019.2010.01339.x. PMID: 20666826
Brown DE, McCoy MW, Pilonieta MC, Nix RN, Detweiler CS
PLoS One 2010 Feb 26;5(2):e9441. doi: 10.1371/journal.pone.0009441. PMID: 20195482Free PMC Article

Clinical prediction guides

Jacquot R, Gerfaud-Valentin M, Lega JC, Becker A, Jamilloux Y, Seve P
Rev Med Interne 2022 Oct;43(10):617-621. Epub 2022 Jun 10 doi: 10.1016/j.revmed.2022.05.011. PMID: 35697578
Sun Y, Blieden C, Merritt BY, Sosa R, Rivero G
J Med Case Rep 2021 Mar 1;15(1):98. doi: 10.1186/s13256-020-02623-2. PMID: 33648567Free PMC Article
De Gottardi J, Montani M, Angelillo-Scherrer A, Rovo A, Berzigotti A
PLoS One 2019;14(12):e0226899. Epub 2019 Dec 30 doi: 10.1371/journal.pone.0226899. PMID: 31887162Free PMC Article
Zahir H, Belkhir J, Mouhib H, Ait Ameur M, Chakour M
Turk J Med Sci 2019 Oct 24;49(5):1332-1335. doi: 10.3906/sag-1812-65. PMID: 31648438Free PMC Article
Chen JH, Fleming MD, Pinkus GS, Pinkus JL, Nichols KE, Mo JQ, Perez-Atayde AR
Am J Surg Pathol 2010 Jun;34(6):852-67. doi: 10.1097/PAS.0b013e3181dbbb17. PMID: 20442642

Recent systematic reviews

Satturwar S, Fowkes M, Farver C, Wilson AM, Eccher A, Girolami I, Pujadas E, Bryce C, Salem F, El Jamal SM, Paniz-Mondolfi A, Petersen B, Gordon RE, Reidy J, Fraggetta F, Marshall DA, Pantanowitz L
Am J Surg Pathol 2021 May 1;45(5):587-603. doi: 10.1097/PAS.0000000000001650. PMID: 33481385Free PMC Article
Díaz LA, Idalsoaga F, Cannistra M, Candia R, Cabrera D, Barrera F, Soza A, Graham R, Riquelme A, Arrese M, Leise MD, Arab JP
World J Gastroenterol 2020 Dec 28;26(48):7693-7706. doi: 10.3748/wjg.v26.i48.7693. PMID: 33505145Free PMC Article

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