Can HLH be misdiagnosed?
HLH is a difficult to diagnose early and can hide behind a maze of contradictory symptoms. This often results in misdiagnosis. The only curative therapy for primary HLH is bone marrow transplant, a high-risk procedure that isn’t suitable for all patients.
Is there a blood test for HLH?
HLH can be diagnosed only with the proper tests. Blood tests include blood cell counts, liver function, infection studies, and markers of immune system activation such as ferritin and soluble IL-2 receptor levels. A bone marrow aspirate and biopsy may be performed to look for microscopic evidence of hemophagocytosis.
What is blood disorder HLH?
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal condition in which certain white blood cells (histiocytes and lymphocytes) build up in and damage organs, including the bone marrow, liver, and spleen, and destroy other blood cells.
Is HLH an autoimmune disorder?
According to common classifications, HLH does not fulfill the criteria of an autoimmune disease (ie, an immune reaction to a more or less defined self-antigen).
Is it possible to diagnose HLH in a patient?
The diagnosis of HLH is often difficult. HLH symptoms may mimic bad infections or malignancies. Additionally, HLH is often observed in these settings and can occur in patients with rheumatologic or autoimmune disorders.
What does HLH stand for in medical category?
Hemophagocytic lymphohistiocytosis (HLH) is a devastating disorder of uncontrolled immune activation characterized by clinical and laboratory evidence of extreme inflammation.
How often does HLH occur in pediatric patients?
Based on the incidence of HLH at our institutions, we estimate that tertiary care pediatric hospitals should expect 1 case of HLH per 3000 inpatient admissions. 8 Practical considerations for diagnosis include prompt assessment and recognition of the signs of HLH, especially in critically ill patients.
When does HLH occur in the human body?
HLH can occur as the initial presentation of the underlying disorder or later in the course, occasionally in the setting of disease progression or the addition of an additional trigger. A retrospective study at Mayo Clinic identified 62 adults with HLH.