Christopher Cheung, Daniel Owen, Harry Brar, David D. Sweet, Eric Yoshida, Bakul Dalal, Luke Chen. UBCMJ 2013 4(2):17-22.
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ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening condition characterized by pathological immune activation
leading to extreme inflammation and organ damage including cytopenias, hepatitis, coagulopathy, and central nervous system damage. It
occurs most commonly in children, often as a familial disorder, and is very rare in adults, where it tends to be sporadic. Two exemplary
cases of adult HLH are presented here, illustrating the clinical features, treatment and natural history of this aggressive and often fatal
disease. A 21–year–old man presented with jaundice and fever and was found to have cytomegalovirus–triggered HLH. He responded
well to the HLH-2004 protocol and remains in remission. A 55–year–old woman presented with erythroderma and multi-organ failure
and was found to have HLH associated with an abnormal T cell population and low–titre Epstein-Barr virus. She died despite etoposide
(Etopophos)–based therapy. HLH should be considered in patients with persistent unexplained inflammation, cytopenias, hepatitis, and
coagulopathy. Serum ferritin is a useful screening test and hyperferritinemia >10,000 μg/L is thought to be specific for hemophagocytosis.
Tissue biopsy (bone marrow, lymph node or liver) to confirm hemophagocytosis is desirable, but hemophagocytosis is often a late
finding and is not necessary for the diagnosis of HLH. Once the diagnosis is confirmed, rapid initiation of chemo–immunotherapy is
required to induce remission.
KEYWORDS: hemophagocytosis lymphohistiocytosis, adult presentation, life-threatening, organ failure, diagnosis and treatment
Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening condition characterized by pathological immune activation
leading to extreme inflammation and organ damage including cytopenias, hepatitis, coagulopathy, and central nervous system damage. It
occurs most commonly in children, often as a familial disorder, and is very rare in adults, where it tends to be sporadic. Two exemplary
cases of adult HLH are presented here, illustrating the clinical features, treatment and natural history of this aggressive and often fatal
disease. A 21–year–old man presented with jaundice and fever and was found to have cytomegalovirus–triggered HLH. He responded
well to the HLH-2004 protocol and remains in remission. A 55–year–old woman presented with erythroderma and multi-organ failure
and was found to have HLH associated with an abnormal T cell population and low–titre Epstein-Barr virus. She died despite etoposide
(Etopophos)–based therapy. HLH should be considered in patients with persistent unexplained inflammation, cytopenias, hepatitis, and
coagulopathy. Serum ferritin is a useful screening test and hyperferritinemia >10,000 μg/L is thought to be specific for hemophagocytosis.
Tissue biopsy (bone marrow, lymph node or liver) to confirm hemophagocytosis is desirable, but hemophagocytosis is often a late
finding and is not necessary for the diagnosis of HLH. Once the diagnosis is confirmed, rapid initiation of chemo–immunotherapy is
required to induce remission.
KEYWORDS: hemophagocytosis lymphohistiocytosis, adult presentation, life-threatening, organ failure, diagnosis and treatment
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