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CSF hemophagocytosis

MedGen UID:
1842108
•Concept ID:
C5826670
•
Finding
Synonym: Hemophagocytosis in CSF
 
HPO: HP:0034798

Definition

Phagocytosis of erythrocytes, lymphocytes or other hematopoietic precursors by histiocytes or macrophages observed in the cerebrospinal fluid (CSF). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCSF hemophagocytosis

Professional guidelines

PubMed

HLH and TET2 Mutation Presenting after First Cycle of CLL Treatment.
Geiger AJ, Yasar DG, Sitwala KV
Clin Med Res 2023 Sep;21(3):155-158. doi: 10.3121/cmr.2023.1804. PMID: 37985171Free PMC Article
A xenograft model of macrophage activation syndrome amenable to anti-CD33 and anti-IL-6R treatment.
Wunderlich M, Stockman C, Devarajan M, Ravishankar N, Sexton C, Kumar AR, Mizukawa B, Mulloy JC
JCI Insight 2016 Sep 22;1(15):e88181. doi: 10.1172/jci.insight.88181. PMID: 27699249Free PMC Article
Characterization of PRF1, STX11 and UNC13D genotype-phenotype correlations in familial hemophagocytic lymphohistiocytosis.
Horne A, Ramme KG, Rudd E, Zheng C, Wali Y, al-Lamki Z, Gürgey A, Yalman N, Nordenskjöld M, Henter JI
Br J Haematol 2008 Oct;143(1):75-83. Epub 2008 Aug 15 doi: 10.1111/j.1365-2141.2008.07315.x. PMID: 18710388

Recent clinical studies

Etiology

Cytomorphologic Features Found in Cerebrospinal Fluid Specimens of Hemophagocytic Lymphohistiocytosis Patients.
Shyu S, Luca D, VandenBussche CJ, Ho CY
Am J Clin Pathol 2021 Aug 4;156(3):381-390. doi: 10.1093/ajcp/aqaa248. PMID: 33624013Free PMC Article
Characterization of PRF1, STX11 and UNC13D genotype-phenotype correlations in familial hemophagocytic lymphohistiocytosis.
Horne A, Ramme KG, Rudd E, Zheng C, Wali Y, al-Lamki Z, Gürgey A, Yalman N, Nordenskjöld M, Henter JI
Br J Haematol 2008 Oct;143(1):75-83. Epub 2008 Aug 15 doi: 10.1111/j.1365-2141.2008.07315.x. PMID: 18710388
The neurologic complications in pediatric acute lymphoblastic leukemia patients excluding leukemic infiltration.
Kuskonmaz B, Unal S, Gumruk F, Cetin M, Tuncer AM, Gurgey A
Leuk Res 2006 May;30(5):537-41. Epub 2005 Oct 24 doi: 10.1016/j.leukres.2005.09.009. PMID: 16249027
Thrombocytopenia in the sepsis syndrome: role of hemophagocytosis and macrophage colony-stimulating factor.
François B, Trimoreau F, Vignon P, Fixe P, Praloran V, Gastinne H
Am J Med 1997 Aug;103(2):114-20. doi: 10.1016/s0002-9343(97)00136-8. PMID: 9274894
A case of pleomorphic T-cell lymphoma with a high content of reactive histiocytes presented with hypereosinophilia.
Dargent JL, Jacobovitz D, Pradier O, Velu T, Martiat P, Delplace J, Neve P, Diebold J
Pathol Res Pract 1995 Jun;191(5):463-8; discussion 469-70. doi: 10.1016/S0344-0338(11)80734-X. PMID: 7479365

Diagnosis

Filgrastim-induced hemophagocytic lymphohistiocytosis in a patient with mantle cell lymphoma: A case report.
Higuchi S, Lee S, Fujita K, Hara T, Tsurumi H
J Infect Chemother 2024 Feb;30(2):150-153. Epub 2023 Sep 27 doi: 10.1016/j.jiac.2023.09.026. PMID: 37769993
Characterization of PRF1, STX11 and UNC13D genotype-phenotype correlations in familial hemophagocytic lymphohistiocytosis.
Horne A, Ramme KG, Rudd E, Zheng C, Wali Y, al-Lamki Z, Gürgey A, Yalman N, Nordenskjöld M, Henter JI
Br J Haematol 2008 Oct;143(1):75-83. Epub 2008 Aug 15 doi: 10.1111/j.1365-2141.2008.07315.x. PMID: 18710388
Familial hemophagocytic lymphohistiocytosis with the MUNC13-4 mutation: a case report.
Mizumoto H, Hata D, Yamamoto K, Shirakawa R, Kumakura A, Shiota M, Yokoyama A, Matsubara H, Kobayashi M, Nishikomori R, Adachi S, Nakahata T, Kita T, Horiuchi H, Yasukawa M, Ishii E
Eur J Pediatr 2006 Jun;165(6):384-8. Epub 2006 Jan 14 doi: 10.1007/s00431-005-0065-0. PMID: 16416131
The neurologic complications in pediatric acute lymphoblastic leukemia patients excluding leukemic infiltration.
Kuskonmaz B, Unal S, Gumruk F, Cetin M, Tuncer AM, Gurgey A
Leuk Res 2006 May;30(5):537-41. Epub 2005 Oct 24 doi: 10.1016/j.leukres.2005.09.009. PMID: 16249027
Thrombocytopenia in the sepsis syndrome: role of hemophagocytosis and macrophage colony-stimulating factor.
François B, Trimoreau F, Vignon P, Fixe P, Praloran V, Gastinne H
Am J Med 1997 Aug;103(2):114-20. doi: 10.1016/s0002-9343(97)00136-8. PMID: 9274894

Therapy

Filgrastim-induced hemophagocytic lymphohistiocytosis in a patient with mantle cell lymphoma: A case report.
Higuchi S, Lee S, Fujita K, Hara T, Tsurumi H
J Infect Chemother 2024 Feb;30(2):150-153. Epub 2023 Sep 27 doi: 10.1016/j.jiac.2023.09.026. PMID: 37769993
HLH and TET2 Mutation Presenting after First Cycle of CLL Treatment.
Geiger AJ, Yasar DG, Sitwala KV
Clin Med Res 2023 Sep;21(3):155-158. doi: 10.3121/cmr.2023.1804. PMID: 37985171Free PMC Article
Florid histiocytic hemophagocytosis following therapy with long acting G-CSF (pegfilgrastim).
Glasser L, Legolvan M, Horwitz HM
Am J Hematol 2007 Aug;82(8):753-7. doi: 10.1002/ajh.20854. PMID: 17315211
Hemophagocytosis exacerbated by G-CSF/GM-CSF treatment in a patient with myelodysplasia.
Wang S, Degar BA, Zieske A, Shafi NQ, Rose MG
Am J Hematol 2004 Dec;77(4):391-6. doi: 10.1002/ajh.20202. PMID: 15551287
Hemophagocytosis complicating Kawasaki disease.
al-Eid W, al-Jefri A, Bahabri S, al-Mayouf S
Pediatr Hematol Oncol 2000 Jun;17(4):323-9. doi: 10.1080/088800100276316. PMID: 10845231

Prognosis

Virological and immunological characteristics of fatal Epstein-Barr virus mononucleosis in a 17-year-old Caucasian male presenting with meningoencephalitis and hemophagocytic syndrome.
Vince A, Lepej SZ, Kurelac I, Barsic B, Kozic S, Klinar I, Zarkovic K
J Neurovirol 2007 Aug;13(4):389-96. doi: 10.1080/13550280701447042. PMID: 17849323
The neurologic complications in pediatric acute lymphoblastic leukemia patients excluding leukemic infiltration.
Kuskonmaz B, Unal S, Gumruk F, Cetin M, Tuncer AM, Gurgey A
Leuk Res 2006 May;30(5):537-41. Epub 2005 Oct 24 doi: 10.1016/j.leukres.2005.09.009. PMID: 16249027
Hemophagocytosis exacerbated by G-CSF/GM-CSF treatment in a patient with myelodysplasia.
Wang S, Degar BA, Zieske A, Shafi NQ, Rose MG
Am J Hematol 2004 Dec;77(4):391-6. doi: 10.1002/ajh.20202. PMID: 15551287
Hemophagocytosis complicating Kawasaki disease.
al-Eid W, al-Jefri A, Bahabri S, al-Mayouf S
Pediatr Hematol Oncol 2000 Jun;17(4):323-9. doi: 10.1080/088800100276316. PMID: 10845231
Thrombocytopenia in the sepsis syndrome: role of hemophagocytosis and macrophage colony-stimulating factor.
François B, Trimoreau F, Vignon P, Fixe P, Praloran V, Gastinne H
Am J Med 1997 Aug;103(2):114-20. doi: 10.1016/s0002-9343(97)00136-8. PMID: 9274894

Clinical prediction guides

Disseminated tuberculosis: interesting hematological observations.
Avasthi R, Mohanty D, Chaudhary SC, Mishra K
J Assoc Physicians India 2010 Apr;58:243-4. PMID: 21046880
Virological and immunological characteristics of fatal Epstein-Barr virus mononucleosis in a 17-year-old Caucasian male presenting with meningoencephalitis and hemophagocytic syndrome.
Vince A, Lepej SZ, Kurelac I, Barsic B, Kozic S, Klinar I, Zarkovic K
J Neurovirol 2007 Aug;13(4):389-96. doi: 10.1080/13550280701447042. PMID: 17849323
Familial hemophagocytic lymphohistiocytosis with the MUNC13-4 mutation: a case report.
Mizumoto H, Hata D, Yamamoto K, Shirakawa R, Kumakura A, Shiota M, Yokoyama A, Matsubara H, Kobayashi M, Nishikomori R, Adachi S, Nakahata T, Kita T, Horiuchi H, Yasukawa M, Ishii E
Eur J Pediatr 2006 Jun;165(6):384-8. Epub 2006 Jan 14 doi: 10.1007/s00431-005-0065-0. PMID: 16416131
Thrombocytopenia in the sepsis syndrome: role of hemophagocytosis and macrophage colony-stimulating factor.
François B, Trimoreau F, Vignon P, Fixe P, Praloran V, Gastinne H
Am J Med 1997 Aug;103(2):114-20. doi: 10.1016/s0002-9343(97)00136-8. PMID: 9274894

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