Hemophagocytic Lymphohistiocytosis (HLH)
Overview
Hemophagocytic lymphohistiocytosis (HLH) is a rare but very serious condition that causes the body’s immune system to become dangerously overactive. In HLH, certain white blood cells that usually protect the body start attacking the body’s own healthy cells, leading to severe inflammation and damage to organs like the liver, spleen, and bone marrow.
Types
It is most commonly seen in young children and can be classified into two types:
- Primary (or familial) HLH, which is genetic and present from birth due to inherited genes.
- Secondary HLH, which can be triggered by infections, autoimmune disorders, or other medical conditions.
Common signs and symptoms
- Recurrent high fevers
- Swollen lymph nodes, spleen, or liver
- Easy bruising or bleeding
- Pallor
- Persistent fatigue
- Breathing difficulties or altered mental status in severe cases
- Yellowish skin or eyes
- Skin rash
Diagnostic tests and procedures
- Physical examination to check for lumps, swelling, or other illness indicators.
- Complete blood count (CBC) can reveal abnormalities in blood cell levels
- laboratory tests including ferritin, fibrinogen, triglycerides and soluble IL-2 receptor levels.
- Bone marrow aspiration and biopsy to check for abnormal cells .
- Genetic tests if primary HLH is suspected.
The Journey of Healing Starts Here
If you or your loved one is experiencing symptoms of Hemophagocytic Lymphohistiocytosis, our expert hematology team is here to help. We provide comprehensive diagnostic services, individualized treatment plans, and ongoing support to ensure the best outcomes.
Treatment options
Medications
Medications to calm down the immune system (such as steroids and chemotherapy drugs).
Bone marrow transplant (BMT)
In cases of primary HLH or severe secondary HLH that do not respond to other treatments, BMT may be recommended.
Treating triggers
Treating triggers such as infections or cancer that may have triggered HLH.
Prognosis
HLH is a serious condition, but with prompt medical care, some people recover fully, especially if treatment starts early. Ongoing follow-up and support are important for patients and families.