Adult-onset Primary Hemophagocytic Lymphohistiocytosis: Reporting a Rare Case with Review of Literature

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon, aggressive hematological syndrome. It is caused by an increased and unchecked proliferation of T lymphocytes and histiocytes. These cells secrete a large number of inflammatory cytokines and infiltrate various tissues causing multi-organ system failure. HLH may be primary or associated with different types of infections, autoimmune disorders, or malignancies. Primary or familial HLH is fatal and is frequently considered a disorder of infants and young children. Only a few cases of primary HLH have been reported in adults. We present a case of a 37-year-old man who presented with fever, pancytopenia, and hepatosplenomegaly. Lymph node biopsy showed collections of histiocytes with lymphoplasmacytic cells. After excluding all the secondary causes a final diagnosis of primary HLH was made. The patient was started on HLH-2004 protocol (etoposide, cyclosporin A, dexamethasone) along with empiric antituberculous drugs as necrotic granulomas were also noted in the biopsy. HLH has a very poor prognosis and familiarity with clinical symptoms, and diagnostic criteria can aid in timely diagnosis.

Keywords: adult-onset; autoimmune; hemophagocytic lymphohistiocytosis; hlh; t lymphocytes.

Figure 1. CT scan of chest showing variable-sized nodules (red arrows) in bilateral lung bases.

Figure 2. CT CAP showing hepatosplenomegaly (red arrows).

CAP: chest, abdomen, and pelvis.

Figure 3. X-ray chest (AP view) showing worsening of pulmonary infilterates (red arrows).

AP view: anterio-posterior view.