Inherited immune disorders
Familial hemophagocytic lymphohistiocytosis
Familial hemophagocytic lymphohistiocytosis (familial HLH) is an inherited immune disorder in which immune activation does not switch off properly. It can cause severe inflammation and organ damage; it is not a blood cancer.
Other names and abbreviations
FHL · FHLH · primary HLH · familial HLH · HLH · hemophagocytic lymphohistiocytosis · Primary familial hemophagocytic lymphohistiocytosis · Familial erythrophagocytic lymphohistiocytosis
Where transplant fits
Allogeneic transplantation replaces the defective blood-forming immune system and is the established curative treatment for familial HLH. Inflammation is controlled as well as possible before transplant. Related, unrelated and alternative grafts can be appropriate; potential family donors require assessment for the relevant genetic condition.
Treatment depends on the exact diagnosis, disease stage, prior treatment and the person’s health. These categories are not estimates of donor demand.
Treatment at a glance
- Who it affects
- Familial HLH often presents in infancy or early childhood, but later childhood and adult presentations occur.
- Other treatment options
- Urgent treatment controls inflammation, commonly using etoposide- and dexamethasone-based therapy or selected cytokine-directed treatment. Infection treatment and support for affected organs are essential. Genetic evaluation and specialist immune/transplant care guide the longer-term plan.
- Cells used for transplantation
- Donated blood-forming cells for allogeneic transplantation. Marrow, peripheral blood or cord blood and donor type are selected for the patient and transplant approach.
Questions to bring to your care team
What is the exact diagnosis or subtype? What is the goal of each treatment option? If transplant is being considered, why does it fit this situation, which cells would be used and what are the alternatives?
Sources and further reading
- Familial Hemophagocytic Lymphohistiocytosis
GeneReviews / NCBI Bookshelf · Accessed 2026-09-05 - Stem Cell and Bone Marrow Transplants for Cancer
NCI · Accessed 2026-09-05 - Donor and cord blood unit selection guidelines
NMDP / CIBMTR · Accessed 2026-09-05
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