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First Author: Abdul-Razaq Adeniyi, Sidney Kimmel Medical
College, Thomas Jefferson University, Class of 2015
Hemaphagocytic lymphcytosis (HLH) is characterized by
progressive histiocytic proliferation of non-malignant lymphocytes
and macrophages. Excessive proliferation of these cells is the
result of unchecked immune cell activation which leads to abnormal
inflammation and tissue destruction. There is generally impaired
regulatory activity of natural killer cells and cytotoxic
lymphocytes leading to excessive activation of macrophages with
abnormal cytokine release. The disease has both sporadic and
familial presentations. Familial or primary HLH is a heritable
disorder that is associated with 1 of 5 identified gene
abnormalities, and symptoms usually present within the first few
months of life. Sporadic or secondary HLH is often triggered by
activation of the immune system during infections, autoimmune
disorders, or other inflammatory states and can present at any
A 32 year old African American female presented to the
outpatient adult medicine office of Wilmington Hospital complaining
of 2 weeks of intermittent left leg and hand cramping with
associated numbness. The symptoms were not triggered by any
specific activities and were the latest symptoms in a presentation
that included 3 months of persistent high grade fevers, chills,
night sweats, fatigue, progressively worsening dyspnea on exertion,
abdominal distention, poor appetite with associated nausea and
vomiting, and a 25 pound weight loss. She was also concerned about
worsening "yellowing of her eyes" over the last 2 months. Her
physical exam was significant for scleral icterus, hepatomegaly and
inguinal lymphadenopathy. She presented to the emergency room 2
months ago with similar symptoms and left neck swelling. At that
point, imaging, including a PET scan, had demonstrated diffuse
bulky lymphadenopathy and hepatosplenomegaly. Blood cultures were
negative and viral assays ruled out HIV, CMV, EBV and hepatitis.
She underwent a cervical lymph node biopsy and bone marrow biopsy
that was consistent with lymphohistiocytic proliferation but
negative for lymphoma. Repeat viral assays remained negative and
she was subsequently referred to infectious disease for further
workup. The diagnosis of HLH was made soon after.
HLH, though rare, should be strongly considered in presentations
of diffuse lymphyadenopathy and weight loss as demonstrated by this
case. The presentation is similar to more frequently considered
etiologies such as HIV and B-cell lymphomas, and as a result is
often not considered until much later in the diagnostic work up.
Delayed diagnosis significantly increases the risk of mortality as
prompt induction of immunotherapy is essential for survival.
Earlier consideration of this disease entity during the work up
phase might facilitate prompter treatment and eliminate unnecessary
July 2015 Issue of IMpact