U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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
Epperly R, Furman W, Hines M, Santiago T, Li Y, Madden R, Mamcarz E, Cervi D, Federico S, Triplett B, Talleur A
Pediatr Blood Cancer 2019 Nov;66(11):e27964. Epub 2019 Aug 12 doi: 10.1002/pbc.27964. PMID: 31407508
Sankhyan N, Saptharishi LG, Sasidaran K, Kanga A, Singhi SC
Indian Pediatr 2014 Aug;51(8):651-3. doi: 10.1007/s13312-014-0470-4. PMID: 25129000
Favara BE
Semin Diagn Pathol 1992 Feb;9(1):63-74. PMID: 1561489

Diagnosis

Zhao M, Guan Y, Lin J, Qiu Y, Zhao S, Duan M
Front Immunol 2024;15:1396124. Epub 2024 Jun 18 doi: 10.3389/fimmu.2024.1396124. PMID: 38957461Free PMC Article
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
Sankhyan N, Saptharishi LG, Sasidaran K, Kanga A, Singhi SC
Indian Pediatr 2014 Aug;51(8):651-3. doi: 10.1007/s13312-014-0470-4. PMID: 25129000
Shah I, Bhatnagar S
Trop Gastroenterol 2010 Apr-Jun;31(2):125. PMID: 20862993

Therapy

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
Epperly R, Furman W, Hines M, Santiago T, Li Y, Madden R, Mamcarz E, Cervi D, Federico S, Triplett B, Talleur A
Pediatr Blood Cancer 2019 Nov;66(11):e27964. Epub 2019 Aug 12 doi: 10.1002/pbc.27964. PMID: 31407508
Thiam K, Sagne JMAN, Ndiaye EHM, Cissé MF, Mbaye FBR, Touré NO, Dia Kane Y, Diatta A, Niang S, Kombila UD, Dia S, Ndao M, Ka W
Med Sante Trop 2017 Aug 1;27(3):233-234. doi: 10.1684/mst.2017.0714. PMID: 28947398
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

Zhao M, Guan Y, Lin J, Qiu Y, Zhao S, Duan M
Front Immunol 2024;15:1396124. Epub 2024 Jun 18 doi: 10.3389/fimmu.2024.1396124. PMID: 38957461Free PMC Article
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
Funakoshi Y, Matsuoka H, Yamamoto K, Yakushijin K, Inui Y, Okamura A, Takeuchi M, Yokozaki H, Minami H
Intern Med 2011;50(24):3031-5. Epub 2011 Dec 15 doi: 10.2169/internalmedicine.50.6168. PMID: 22185999
Shah I, Bhatnagar S
Trop Gastroenterol 2010 Apr-Jun;31(2):125. PMID: 20862993

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
Yilmaz S, Duman N, Ozer E, Kavas N, Oren H, Demircioğlu F, Kumral A, Ozkan H, Irken G, Ozer E
J Pediatr Hematol Oncol 2006 May;28(5):290-2. doi: 10.1097/01.mph.0000212906.07018.93. PMID: 16772878

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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...