E. chaffeensis is the most common cause of human infection
E. ewingii, E. muris, R. muris-like agent, E. canis, and E. ruminantium can all cause human disease, with slightly different tick vectors and mammalian hosts
Cells have two ultrastructural forms: larger reticulate cells (RC) and small, dense core cells (DC)
Intracellularly, they form aggregates called morulae (mulberries)
Epidemiology
E. chaffeensis is primarily found in south-central and eastern North America
E. chafeensis is transmitted by Amblyomma americanum ticks (the Lone Star tick)
A. americanum and Dermacentor variabilis for E. ewingii
Ixodes persulcatus and Haemaphysalis flava for E. muris
I. scapularis for E. muris-like agent
White-tailed deer are the reservoir
Also humans, dogs, coyotes, and marsh deer (E. chaffeensis)
Dogs, humans deer (E. ewingii)
Not transmitted transovarially
Incidence peaks in May to August
Exposure is rural or suburban, and usually involves recreational, peridomestic, occupational, and military acitivities
Therefore, 60% male
Pathophysiology
After innoculation, spread lymphangitically and hematogenously to reach and invade macrophages and granulocytes
Intracellular morulae also found in blood, bone marrow, liver, lymph nodes, spleen, and CSF macrophages
Affects almost any organ except for endothelium
Surface porin proteins are responsible for antigenic variation and host cell adhesion
The dense core cells also secrete TRP120, which is involved adhesion but also has effects on host cell DNA and DNA transcription
DC cells predominate soon after infection, then give way to RC cells
Most of the pathology is caused by host responses, including toxic shock
Increased TNF-alpha, IL 1-alpha and 1-beta, IL-6, IL-10, and defective Th1 cytokines (IFN-gamma and IL-2)
Clinical Presentation
E. chaffeensis (human monocytotropic ehrlichiosis; HME)
Usually causes a mild-to-severe multisystem illness in immunocompetent
Can cause overwhelming infection in immunosuppressed, especially HIV/AIDS
Incubation period of 7 days
Fever, headache, myalgia, and malaise are most common
Nausea and vomiting in a half, and weight loss
Can also have diarrhea, rash, cough, altered mentation
Rash is maculopapular or petechial, and more frequent in children
Often accompanied by leukopenia, thrombocytopenia, and anemia
Likely from peripheral consumption rather than bone marrow suppression
Causes leuneutropenia and lymphopenia
Thrombocytopenia usually not severe
Bone marrow may show a compensatory hypercellularity
Elevated liver enzymes is almost universal, and AKI in a third of patients
Severe compplications include ARDS, DIC, and death
Also, hemophagocytic lymphohistiocytosis with E. chaffeensis
50% of cases require hospitalization, and 10% have severe complications, and 2-3% die
The illness usually lasts about 3 weeks (1 week in hospital), followed by prolonged convalescence
E. ewingii and E. muris
Mostly in immunocompromised patients
Presents similarly to HME, but less severe
Differential Diagnosis
Rocky Mountain spotted fever (RMSF)
Diagnosis
Should be treated empirically without waiting for diagnosis
Major method of diagnosis is IFA serology, looking for a fourfold rise in titres over the course of disease, with a minimum peak of 1:64
Usually peaks at 6 weeks
Diagnosis can be suggested by morulae on blood film (in monocytes), but insensitive unless overwhelming infection
