Trial Outcomes & Findings for Evaluation of IFNγ and Inflammatory Mediators in Patients With Hemophagocytic Lymphohistiocytosis (NCT NCT06047210)

NCT ID: NCT06047210

Last Updated: 2024-09-19

Results Overview

Serum samples was collected at various times after HLH diagnosis to determine the concentration of CXCL10.

• Recruitment status: COMPLETED
• Target enrollment: 14 participants
• Primary outcome timeframe: 11 months
• Results posted on: 2024-09-19

Participant Flow

This Phase 4 observational study was conducted in adult patients diagnosed with hemophagocytic lymphohistiocytosis (A-HLH) at a single center in the United States of America.

A total of 14 patients were enrolled in the study.

Participant milestones

Measure	A-HLH Patients Patients diagnosed with A-HLH.	Malignancy-Associated Hemophagocytic Lymphohistiocytosis (M-HLH) Patients Patients diagnosed with HLH in the context of a malignancy.
Overall Study STARTED	10	4
Overall Study COMPLETED	10	4
Overall Study NOT COMPLETED	0	0

Reasons for withdrawal

Withdrawal data not reported

Baseline Characteristics

Evaluation of IFNγ and Inflammatory Mediators in Patients With Hemophagocytic Lymphohistiocytosis

Baseline characteristics by cohort

Measure	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	Total n=14 Participants Total of all reporting groups
Age, Continuous	44.8 years STANDARD_DEVIATION 12.4 • n=5 Participants	54.3 years STANDARD_DEVIATION 4.9 • n=7 Participants	47.5 years STANDARD_DEVIATION 11.5 • n=5 Participants
Sex: Female, Male Female	4 Participants n=5 Participants	2 Participants n=7 Participants	6 Participants n=5 Participants
Sex: Female, Male Male	6 Participants n=5 Participants	2 Participants n=7 Participants	8 Participants n=5 Participants
Race/Ethnicity, Customized Caucasian	8 Participants n=5 Participants	3 Participants n=7 Participants	11 Participants n=5 Participants
Race/Ethnicity, Customized African descent	1 Participants n=5 Participants	0 Participants n=7 Participants	1 Participants n=5 Participants
Race/Ethnicity, Customized Mixed/multi-racial	1 Participants n=5 Participants	0 Participants n=7 Participants	1 Participants n=5 Participants
Race/Ethnicity, Customized Other	0 Participants n=5 Participants	1 Participants n=7 Participants	1 Participants n=5 Participants

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of CXCL10.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - C-X-C Chemokine Ligand 10 (CXCL10)	15197.143 picogram/milliliter (pg/mL) Standard Deviation 21288.948	2079.286 picogram/milliliter (pg/mL) Standard Deviation 2659.228

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of CXCL9.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - C-X-C Chemokine Ligand 9 (CXCL9)	6602.853 pg/mL Standard Deviation 7777.846	4474.685 pg/mL Standard Deviation 10207.638

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of IFNg.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - Interferon Gamma (IFNg)	809.810 pg/mL Standard Deviation 769.433	81.782 pg/mL Standard Deviation 140.870

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of IL-10.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - Interleukin 10 (IL-10)	266.948 pg/mL Standard Deviation 257.770	24.157 pg/mL Standard Deviation 87.640

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of IL-1B.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - Interleukin 1 Beta (IL-1B)	2.140 pg/mL Standard Deviation 0.000	2.140 pg/mL Standard Deviation 0.000

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of IL-6.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - Interleukin 6 (IL-6)	29.695 pg/mL Standard Deviation 52.403	3.455 pg/mL Standard Deviation 3.727

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of neopterin.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - Neopterin	839.775 nanomoles Standard Deviation 950.070	33.995 nanomoles Standard Deviation 29.227

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of TNFa.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - Tumor Necrosis Factor Alpha (TNFa)	14.308 pg/mL Standard Deviation 12.010	6.050 pg/mL Standard Deviation 6.405

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of hIFNg.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - Total Human Interferon Gamma (hIFNg)	1069.215 pg/mL Standard Deviation 1987.409	34.178 pg/mL Standard Deviation 25.950

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of sCD163.

Outcome measures

Measure	M-HLH Patients n=1 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=4 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - Soluble CD163 (sCD163)	878.880 nanogram (ng)/mL Standard Deviation NA Standard deviation could not be determined when only 1 patient analyzed.	1431.488 nanogram (ng)/mL Standard Deviation 1078.058

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Serum samples was collected at various times after HLH diagnosis to determine the concentration of sIL2Ra.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Inflammatory Biomarker - Soluble IL-2 Receptor Alpha (sIL2Ra)	27052.498 pg/mL Standard Deviation 24009.516	2273.193 pg/mL Standard Deviation 2383.154

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of white blood cells.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of White Blood Cells	2.459 cells*10^3/microliter (mcL) Standard Deviation 2.854	4.263 cells*10^3/microliter (mcL) Standard Deviation 3.480

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of red blood cells.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Red Blood Cells	3.206 cells*10^6/mcL Standard Deviation 0.396	3.420 cells*10^6/mcL Standard Deviation 0.745

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of hemoglobin.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Hemoglobin	9.83 gram/deciliter (g/dL) Standard Deviation 1.09	10.11 gram/deciliter (g/dL) Standard Deviation 1.93

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of hematocrit.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Hematocrit	28.55 percentage of volume of red cells Standard Deviation 3.55	30.03 percentage of volume of red cells Standard Deviation 5.75

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of platelets.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Platelets	92.9 cells*10^3/mcL Standard Deviation 87.1	182.6 cells*10^3/mcL Standard Deviation 138.2

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of neutrophils.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Neutrophils	22.303 cells*10^3/mcL Standard Deviation 37.944	7.715 cells*10^3/mcL Standard Deviation 14.003

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of lymphocytes.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Lymphocytes	1.956 cells*10^3/mcL Standard Deviation 3.424	3.636 cells*10^3/mcL Standard Deviation 10.880

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of monocytes.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Monocytes	0.948 cells*10^3/mcL Standard Deviation 1.721	1.306 cells*10^3/mcL Standard Deviation 3.602

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of eosinophils.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Eosinophils	1.002 cells*10^3/mcL Standard Deviation 2.449	0.252 cells*10^3/mcL Standard Deviation 0.707

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of basophils.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Basophils	0.222 cells*10^3/mcL Standard Deviation 0.399	0.181 cells*10^3/mcL Standard Deviation 0.516

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of D-dimer.

Outcome measures

Measure	M-HLH Patients Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=1 Participants Patients diagnosed with A-HLH.
Mean Plasma Concentration of D-Dimer	—	2300.0 microgram (mcg)/mL Standard Deviation NA Standard deviation could not be determined when only 1 patient analyzed.

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of fibrinogen.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=4 Participants Patients diagnosed with A-HLH.
Mean Plasma Concentration of Fibrinogen	172.5 milligram (mg)/dL Standard Deviation 83.0	306.8 milligram (mg)/dL Standard Deviation 145.7

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the aPTT.

Outcome measures

Measure	M-HLH Patients Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=3 Participants Patients diagnosed with A-HLH.
Mean Activated Partial Thromboplastin Time (aPTT)	—	27.98 seconds Standard Deviation 8.21

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the prothrombin time.

Outcome measures

Measure	M-HLH Patients n=3 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=6 Participants Patients diagnosed with A-HLH.
Mean Prothrombin Time	16.75 seconds Standard Deviation 4.60	13.81 seconds Standard Deviation 0.90

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of c-reactive protein.

Outcome measures

Measure	M-HLH Patients n=3 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=5 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of C-Reactive Protein	13.35 mg/dL Standard Deviation 12.06	27.97 mg/dL Standard Deviation 31.13

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of ferritin.

Outcome measures

Measure	M-HLH Patients n=3 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Ferritin	24331.4517 ng/mL Standard Deviation 38189.6158	5050.2273 ng/mL Standard Deviation 5302.9183

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of blood urea nitrogen.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Plasma Concentration of Blood Urea Nitrogen	50.2 mg/dL Standard Deviation 37.3	17.3 mg/dL Standard Deviation 8.3

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of creatinine.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Creatinine	1.449 mg/dL Standard Deviation 0.748	0.768 mg/dL Standard Deviation 0.293

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of albumin.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Albumin	3.21 gram/dL Standard Deviation 0.87	3.68 gram/dL Standard Deviation 0.55

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of sodium.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Sodium	135.3 millimoles/L Standard Deviation 3.2	136.4 millimoles/L Standard Deviation 3.7

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of fasting triglycerides.

Outcome measures

Measure	M-HLH Patients n=1 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients Patients diagnosed with A-HLH.
Mean Serum Concentration of Fasting Triglycerides	645.0 mg/dL Standard Deviation NA Standard deviation could not be determined when only 1 patient analyzed.	—

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of total bilirubin.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Total Bilirubin	4.204 mg/dL Standard Deviation 6.359	0.590 mg/dL Standard Deviation 0.559

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of conjugated bilirubin.

Outcome measures

Measure	M-HLH Patients n=2 Samples Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=8 Samples Patients diagnosed with A-HLH.
Mean Serum Concentration of Conjugated Bilirubin	12.80 mg/dL Standard Deviation 1.13	0.25 mg/dL Standard Deviation 0.31

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of alanine aminotransferase.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Alanine Aminotransferase	54.8 international units (IU)/L Standard Deviation 35.1	93.2 international units (IU)/L Standard Deviation 145.6

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of aspartate aminotransferase.

Outcome measures

Measure	M-HLH Patients n=4 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=10 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Aspartate Aminotransferase	48.6 IU/L Standard Deviation 35.4	66.0 IU/L Standard Deviation 105.8

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of alkaline phosphatase.

Outcome measures

Measure	M-HLH Patients n=2 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Alkaline Phosphatase	258.2 IU/L Standard Deviation 188.5	121.1 IU/L Standard Deviation 98.8

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of lactate dehydrogenase.

Outcome measures

Measure	M-HLH Patients n=3 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=9 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Lactate Dehydrogenase	362.7 IU/L Standard Deviation 274.4	539.8 IU/L Standard Deviation 660.8

PRIMARY outcome

Timeframe: 11 months

Population: The enrolled set included all patients who signed an informed consent. Only patients with data collected for this outcome measure are reported.

Blood samples was collected at various times after HLH diagnosis to determine the concentration of IgG.

Outcome measures

Measure	M-HLH Patients n=1 Participants Patients diagnosed with HLH in the context of a malignancy.	A-HLH Patients n=5 Participants Patients diagnosed with A-HLH.
Mean Serum Concentration of Immunoglobulin G (IgG)	996.0 mg/dL Standard Deviation NA Standard deviation could not be determined when only 1 patient analyzed.	763.6 mg/dL Standard Deviation 579.3

Adverse Events

A-HLH Patients

Serious events: 0 serious events

Other events: 0 other events

Deaths: 0 deaths

M-HLH Patients

Serious events: 0 serious events

Other events: 0 other events

Deaths: 0 deaths

Serious adverse events

Adverse event data not reported

Other adverse events

Adverse event data not reported

Additional Information

Medical Director

Swedish Orphan Biovitrum

Phone: +4686972000

Email: clinical@sobi.com

Results disclosure agreements

• Principal investigator is a sponsor employee
• Publication restrictions are in place