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2014 ; 30
(Suppl 1
): 145-7
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Macrophage activation syndrome secondary to human monocytic ehrlichiosis
#MMPMID25332563
Kumar N
; Goyal J
; Goel A
; Shakoory B
; Chatham W
Indian J Hematol Blood Transfus
2014[Sep]; 30
(Suppl 1
): 145-7
PMID25332563
show ga
OBJECTIVES: To present a case of human monocytic ehrlichiosis (HME) that was
complicated by macrophage activation syndrome (MAS), also known as secondary
hemophagocytic lymphohistiocytosis (sHLH). METHODS: Data was collected from
patient's electronic medical records at the University of Alabama at Birmingham.
The patient is a part of a larger cohort of patients with all-cause MAS treated
at our center. CASE: A 63 year old renal transplant recipient male on maintenance
immunosuppressive therapy presented with high grade fever, leukopenia,
thrombocytopenia and elevated transaminases and initially met clinical criteria
for severe sepsis. On further investigation, clinical and laboratory criteria for
MAS were met. He was treated with a combination of doxycycline for HME and a
novel combination of anakinra (interleukin-1 receptor antagonist), and high dose
corticosteroids. The discussion focuses on clinical presentation, pathogenesis
and treatment of MAS with an emphasis on MAS secondary to HME. CONCLUSION:
Macrophage activation syndrome or sHLH is a dysfunctional, hyperactive and
potentially fatal immune system response that results in multi-organ dysfunction.
With increasing incidence of Ehrlichia chaffeensis as an emerging pathogen,
clinicians should be aware of this fulminant and potentially fatal complication
of HME.