Hemophagocytic Lympho Histiocytosis (HLH) (original) (raw)

Hemophagocytic Lymphohistiocytosis in a Pediatric Patient

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP, 2021

Hemophagocytic Lymphohistiocytosis (HLH) is an uncommon but a potentially fatal disease characterised by high grade fever, hepato-splenomegaly, deranged liver functions, cytopenias and multi-organ involvement. HLH has its own diagnostic criteria, which comprise of various clinical and laboratory features, which were revised in 2004 by HLH Society for prompt identification and recognition. Here, we present a case of 9-year developmentally normal female with all the complaints as mentioned above, who received treatment for almost eight months as a case of pyrexia of unknown origin (PUO) without a definitive diagnosis. The differential diagnosis of HLH was considered in the patient and later, it was corroborated by hemophagocytic activity on bone marrow aspiration examination. The diagnosis of HLH was made after fulfillment of its criteria which was addressed completely and found veracious. After addressing the active complaints of the patient, she was referred to a pediatric oncologis...

Hemophagocytic lymphohistiocytosis: a rare and life-threatening diagnosis

International Journal of Research in Medical Sciences

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome of excessive activation of immune system. It frequently affects infants from birth to 18 months of age, but is also observed in children and adults of all ages. HLH can occur as a familial or sporadic disorder, and it is triggered by a variety of events, Infection being the most common trigger both in familial and in sporadic cases. Prompt treatment is very critical in cases of HLH, but the greatest barrier is often delay in diagnosis due to the rarity of this syndrome, variable clinical presentation, and lack of specificity of the clinical and laboratory findings. The key clinical features of HLH are high persistent fever, hepatosplenomegaly, blood cytopenia, elevated aminotransferase and ferritin levels, and coagulopathy. A diagnosis of HLH is mostly under-recognized, and is associated with high mortality, especially in adults; thus, prompt diagnosis and treatment are essential. We here present a rare...

Infection Associated Hemophagocytic Lymphohistiocytosis

The British journal of clinical practice

The term hemophagocytosis describes the pathologic finding of activated macrophages, engulfing erythrocytes, leukocytes, platelets and their precursor cells. This phenomenon is an important finding in patients with hemophagocytic syndrome, more properly referred to as hemophagocytic lymphohistiocytosis (HLH). HLH is a distinct clinical entity characterized by fever, pancytopenia, splenomegaly and hemophagocytosis in bone marrow, liver or lymph nodes. It has been associated with a variety of viral, bacterial, fungal and parasitic infections, as well as collagen vascular diseases and malignancies and is uniformly fatal if left untreated. We report Staphylococcus aureus-induced hemophagocytic lymphohistiocytosis in a 3-month-old girl presenting with respiratory distress, sepsis and multiorgan failure. This case report may at least in part guide pediatricians and other physicians to recognize this rare entity of infection triggering fatal HLH and thus proper treatment may be instituted ...