Pediatric emergency medicine trisk 3000 3000
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TABLE 101.14
DIAGNOSTIC CRITERIA FOR HLH
The diagnosis of HLH can be established if either 1 or 2 (below) is fulfilled:
1. A molecular diagnosis consistent with HLH
2. Diagnostic criteria for HLH fulfilled (5 out of 8 criteria below)
(A) Initial diagnostic criteria
1. Fever
2. Splenomegaly
3. Cytopenias (affecting ≥2 of 3 lineages in the peripheral blood):
1. Hemoglobin <90 g/L (in infants <4 wks: hemoglobin <100 g/L)
2. Platelets <100×109/L
3. Neutrophils <1.0×109/L
4. Hypertriglyceridemia and/or hypofibrinogenemia:
1. Fasting triglycerides ≥3.0 mmol/L (i.e., ≥265 mg/dL)
2. Fibrinogen ≤1.5 g/L
5. Hemophagocytosis in bone marrow or spleen or lymph nodes
1. No evidence of malignancy
(B) New diagnostic criteria
1. Low or absent NK-cell activity (according to local laboratory reference)
2. Ferritin ≥500 mg/L
3. Soluble CD25 (i.e., soluble IL-2 receptor) ≥2,400 U/mL
From Henter JI, Horne A, Arico´ M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic
lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124–131. Copyright © 2006 WileyLiss, Inc. Reprinted by
permission of John Wiley & Sons, Inc.
Management
The goal of the treatment of HLH and MAS is to suppress the hyperactive immune
response. In the setting of acquired HLH or MAS, targeted treatment of the underlying
triggering process is the first step. For example, if an inciting bacterial infection is
suspected, then treatment with antibiotics should be initiated. When the diagnosis of
HLH is made according to the HLH 2004 criteria, then treatment according to that
protocol under the supervision of an oncologist should be started, including
administration of systemic corticosteroids, cyclosporine, and etoposide. In spite of
these regimens, survival remains approximately 50%. In patients with familial
hemophagocytic lymphohistiocytosis (FHL) and those failing the above-mentioned
treatment regimen, hematopoietic stem cell transplantation (HSCT) is indicated.
Because HSCT is the only chance for cure in FHL, the considerable risks of the
procedure are often accepted.
As with HLH, to treat MAS one must also extinguish the rampant immune response.
Systemic corticosteroids are the first line of therapy but may be contraindicated if there
is strong suspicion for an infectious trigger. Biologic response modifiers directed
