💬In plain words
What the trial is, and whether it could suit you
Background:
Hemophagocytic lymphohistiocytosis (HLH) is a disease caused by disrupted immune function. People with HLH are prone to fevers and illnesses, which can be fatal. Some people develop a genetic form of this disease (pHLH), but researchers do not understand why some other people develop a nongenetic form (sHLH). They also do not have good ways to diagnose and treat sHLH.
Objective:
To learn about sHLH and why some people get it and others do not.
Eligibility:
Adults aged 18 years and older with sHLH.
Design:
Participants will be admitted to the study based on a review of their medical records. Those who join will have at least 3 clinical evaluations over 9 to 12 months. These may occur during an inpatient hospitalization if they require medical care or in the outpatient clinic.
Participants will also have a physical exam at each visit. Up to half a cup of blood will be d
Sign in to get the eligibility rules as simple questions to take to your doctor.
📋 Eligibility rules (as published)
* INCLUSION CRITERIA:
* Aged 18 years or older.
* Established diagnosis of sHLH defined by meeting any published criteria, per Table 1:
* Meeting the HLH-2004 criteria.
* HScore of \>168. For those without a bone marrow biopsy to evaluate for hemophagocytosis (worth 35 points in the criteria), HScore\>134 will be used.
* For those with underlying rheumatologic disease: meeting the 2016 American College of Rheumatology criteria for macrophage activation syndrome.
* Agree to storage and sharing of study data and biospecimens for future research use.
Table 1: Published Criteria for HLH
HLH-2004 Criteria:
Molecular diagnosis of HLH OR At least 5 of 8 below criteria:
* Fever (\>38.4 Degrees Celcius)
* Splenomegaly
* Cytopenias affecting \>=2 of 3 lineages: Hgb \<9 g/dL, platelets \<10\^5/microliter, neutrophils \<10\^6/microliter
* Hypertriglyceridemia (\>256 mg/dL) and/or fibrinogen \<1.5 g/L
* Hemophagocytosis on biopsy
* Serum ferritin \>=500 ng/mL
* Increased serum sCD25 (\>2400 U/mL)
* Low or absent NK cell activity
HScore:
Known immunosuppression:
0 (no) or 18 (yes)
Temperature (degrees, Celsius):
0 (\<38.4), 33 (38.4-39.4), 49 (\>39.4)
Organomegaly:
0 (no), 23 (liver/spleen), 38 (both)
Number of cytopenias:
0 (1 lines), 24 (2 lines), 34 (3 lines)
Ferritin (ng/mL):
0 (\<2000), 35 (2000-6000), 50 (\>6000)
Triglycerides (mg/dL):
0 (\<1.5), 44 (1.5-4), 66 (\>4)
Fibrinogen (g/L):
0 (\>2.5), 30 (\<2.5)
AST (IU/mL):
0 (\<30), 19 (\>30)
Hemophagocytosis:
0 (no) or 35 (yes)
Cutoff value=169
ACR 2016-MAS Criteria:
A febrile patient with known or suspected sJIA is classified as having macrophage activation syndrome if the following criteria are met:
Ferritin \>684 ng/mL
AND any 2 of the following:
* Platelets \<=181,000/microliter
* AST \>48 IU/mL
* Triglycerides \>156 mg/dL
* Fibrinogen \<=3.6 g/L
Abbreviations: ACR, American College of Rheumatology; AST, aspartate transaminase; Hgb, hemoglobin; HLH, hemophagocytic lymphohistiocytosis; MAS, macrophage activation syndrome; NK, natural killer, sJIA, systemic juvenile idiopathic arthritis.
EXCLUSION CRITERIA:
An individual who meets any of the following criteria will be excluded from participation in this study:
* Currently pregnant.
* Any condition that, in the judgment of the investigator, may put the participant at undue risk or make them unsuitable for participation in the study.
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