Publication details
Hemofagocytující lymfohistiocytóza
| Title in English | Hemophagocytic lymphohistiocytosis syndrome |
|---|---|
| Authors | |
| Year of publication | 2010 |
| Type | Article in Periodical |
| Magazine / Source | Vnitř. Lék. |
| MU Faculty or unit | |
| Citation | |
| Field | Epidemiology, infectious diseases and clinical immunology |
| Keywords | hemophagocytic lymphohistiocytosis; immunodeficiency; lymphoproliferation; macrophage activation syndrome; immunosuppressive theraphy; stem-cell transplantation |
| Description | Hemophagocytic lymphohistiocytosis (HLH) represents a heterogenous group of specific immune-systeme deficiencies, characterized by uncontrolled proliferation of T lymphocytes and macrophages, resulting in overproduction of cytokines and inadequate hemophagocytic activity in lymphoreticular systeme and CNS. A series of 17 primary HLH patients diagnosed in the Czech Republic between 1999 and 2009, representing our experience with this rare disease, has outcome comparable to data published by European HLH group (4yrs OS 74%, EFS 56%). Diagnostic dilemma in HLH is demonstrated through several case reports of both primary and secondary HLH patients, revealing significance of clinical experience, awareness of differential diagnosis and application of modern immunological analyses in diagnostic procedure in HLH. All these parametres contribute to early onset of treatment determinating prognosis of HLH patients. |