Summary:
Haemophagocytic lymphohistiocytosis (HLH) is a rare but
potentially fatal disease of normal but overactive histiocytes
and lymphocytes that commonly appears in infancy,
although it has been seen in all age groups. The disease may
be inherited or acquired due to infections, collagen vascular
diseases and malignancies. The pathological hallmark of
the syndrome is uncontrolled activation of T lymphocytes
and macrophages, together with an impaired cytotoxic
function of NK cells and CD8+ T lymphocytes, resulting
into massive cytokine release (e.g., interferon ”, TNF á,
interleukin[IL]-6, 8,10,12,18 etc) from these cells and
overwhelming inflammation. Lymphocytes and
macrophages, sometimes with haemophagocytic activity
accumulate in bone marrow, spleen, liver, or lymph nodes.
This immune dysregulatory disorder is characterized by fever,
hepatosplenomegaly, lymphadenopathy, skin rash,
cytopenias, hepatitis, coagulopathy, neurological symptoms.
We report a case of 65 years old male presenting with fever
and erythroderma who developed typical clinical and
laboratory findings consistent with diagnosis of HLH
according to HLH-2004 guidelines. Despite receiving
etoposide based chemotherapy, the patient succumbed rapidly
from progressive HLH. This case high lightened the
diagnostic challenge and the need for keeping a high index
of suspicion for promptly diagnosis and treatment of this
potentially life threatening condition as clinical features
and laboratory investigations are non specific.
Key words: Haemophagocytic lymphohistiocytosis.
(J Banagladesh Coll Phys Surg 2015; 33: 166-176)