Secondary hemophagocytic lymphohistiocytosis

Summary

Secondary hemophagocytic lymphohistiocytosis (MONDO:0015542) is a disease and 6 clinical trials. Top therapeutic interventions include emapalumab and rituximab. A subtype of hemophagocytic syndrome — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Clinical trials: 6

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	secondary hemophagocytic lymphohistiocytosis
Mondo ID	MONDO:0015542
Orphanet	158041
NCIT	C121184
UMLS	C4054044
MedGen	883550
GARD	0020026
Is cancer (heuristic)	no

Also known as: acquired hemophagocytic lymphohistiocytosis · acquired hemophagocytic syndrome · reactive hemophagocytic syndrome

Disease family

This is a subtype of hemophagocytic syndrome. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › syndromic disease › hemophagocytic syndrome › secondary hemophagocytic lymphohistiocytosis

Related subtypes (1): hereditary hemophagocytic lymphohistiocytosis

Subtypes (2): acquired hemophagocytic lymphohistiocytosis associated with malignant disease, macrophage activation syndrome

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 6.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	3
PHASE3	2
PHASE2/PHASE3	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT05416307	PHASE2/PHASE3	RECRUITING	Open-label Study of ELA026 in Participants With Secondary Hemophagocytic Lymphohistiocytosis (sHLH)
NCT05001737	PHASE3	COMPLETED	Evaluate Efficacy, Safety and Tolerability, PK and PD of Emapalumab in Children and Adults With MAS in Still’s or SLE
NCT05384743	PHASE3	UNKNOWN	Rituximab Monotherapy for EBV-HLH and CAEBV
NCT06339177	Not specified	RECRUITING	Hemophagocytic Lymphohistiocytosis (HLH) Evaluation and Research of Clinical, ImmUnoLogic and TranscriptomE Study
NCT05841342	Not specified	UNKNOWN	Prospective Study of Immune Function and PD-1 Antibody Therapy Efficacy Predictors on CAEBV and EBV-HLH Patients
NCT07531069	Not specified	COMPLETED	Efficacy and Safety of Interferon-Gamma Monoclonal Antibody Combined With Dexamethasone in the Treatment of Refractory Adult Secondary Hemophagocytic Lymphohistiocytosis

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
EMAPALUMAB	4	1
RITUXIMAB	4	1