[Hemophagocytic syndrome and toxoplasmic primo-infection].
Hemophagocytic syndrome (HPS) is a clinical entity that combines the clinical, biological and histological symptoms. The physiopathological mechanism involves the interaction between T lymphocytes/NK cells and macrophages, at the origin of an uncontrolled activation of the macrophages. The consequence is a hemophagocytosis extending to numerous organs, preferentially bone marrow. Clinical symptoms include cytopenia, fever unresponsive to antibiotics and multiple organ dysfunctions. Infections, lymphoproliferative disorders, cancers, systemic diseases are the most prevalent triggers or etiologies of HPS. Because of its high risk of mortality, HPS constitutes a diagnostic and therapeutic urgency. The search for an aetiology, in particular by serological testing, is essential because it conditions the treatment and thus the evolution of the disease. We report here the case of a 12 years-old boy presenting a HPS secondary to a toxoplasmic primo-infection. The objective of this work is to present the step of the biological diagnosis of HPS. Moreover, this observation allows the study of a very rare clinical presentation of toxoplasmic primo-infection, in an immunocompetant patient.
Briand
P Y
PY
Laboratoire d'hématologie-immunologie-thérapie cellulaire, CHU de Pontchaillou, Rennes. pierreyvesbriand@hotmail.com
Gangneux
J P
JP
Favaretto
G
G
Ly-Sunnaram
B
B
Godard
M
M
Robert-Gangneux
F
F
Fest
T
T
D000900:Anti-Bacterial Agents / Q000008:administration & dosage / Q000627:therapeutic use; D002648:Child; D004797:Enzyme-Linked Immunosorbent Assay; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007075:Immunoglobulin M / Q000097:blood; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology / Q000276:immunology / Q000503:physiopathology; D008297:Male; D009192:Myelography; D011379:Prognosis; D015572:Spiramycin / Q000008:administration & dosage / Q000627:therapeutic use; D013997:Time Factors; D014123:Toxoplasmosis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D016896:Treatment Outcome
D000900:Anti-Bacterial Agents; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D015572:Spiramycin