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1262 Part IX: Lymphocytes and Plasma Cells Chapter 82: Mononucleosis Syndromes 1263

TABLE 82–2. Signs and Symptoms of Epstein-Barr Virus and Cytomegalovirus Mononucleosis: Effect of Age
(Percent of Patients)
†
Signs and Symptoms EBV (Age 14–35 Years*) EBV (Age 40–72 Years ) CMV (Age 30–70 Years )
‡
Fever 95 94 85
Pharyngitis 95 46 15
Lymphadenopathy 98 49 24
Splenomegaly 65 33 3
Hepatomegaly 23 42 N/A
Jaundice 8 27 24

CMV, cytomegalovirus; EBV, Epstein-Barr virus.
*Data from RJ Hoagland. 10
† Data from Hoagland RJ: The clinical manifestations of infectious mononucleosis: A report of 200 cases. Am J Med Sci 240:55, 1960; Schmader KE,
van der Horst CM, Klotman ME: Epstein-Barr virus and the elderly host. Rev Infect Dis 11:64–73, 1989; Axelrod P, Finestone AJ: Infectious mono-
nucleosis in older adults. Am Fam Physician 42:1599, 1990; Hurwitz CA, Henle W, Henle G, et al: Infectious mononucleosis in patients aged 40 to
72 years: Report of 27 cases, including 3 without heterophile-antibody responses. Medicine 62:256, 1983.
‡ Data from reference Just-Nubling, G. Korn, S. Ludwig, B. et.al: Primary cytomegalovirus infection in an outpatient setting–laboratory markers
and clinical aspects. Infection 31:318, 2003.

induces polyclonal proliferation of infected B cells in the nodes in the There are exceptions to the usual situation of most people becom-
pharynx. Initial symptoms are lassitude and fever with no evidence of ing infected by age 25 years. The first is found in a woman who is pro-
lymphocytosis or pharyngitis. Fever results from infection and pro- tected by her family from intimate male contact. She avoids infection
liferation of the B lymphocytes. From the nodes in the pharynx, the until she is married at which time she may become infected from her
infected cells make their way into the circulating lymphocyte pool. 42,43 husband. The second situation occurs when a long-term relationship
Although the duration of virus in the blood is much shorter than it is established when a couple is young. If they are both seronegative,
is in the secretions, it is the movement into the blood that leads to infection does not occur. When they reach parenting age or older, they
the manifestation of the disease. Subsequent, massive T-cell response become infected by a child or a grandchild. When that occurs, the pre-
to the neoantigens on the infected B lymphocyte is evident by the sentation is less likely to include lymphadenopathy and pharyngitis
lymphocytosis with reactive blood lymphocytes and other disease (see Table 82–3). 18,40,41 Fever almost always occurs and abdominal pain,
manifestations. The pharyngitis that develops is a result of the T-cell hepatomegaly, and abnormal liver function develops in most. Older
response to the infected B cells that are found in Waldeyer ring in the adults are less likely to develop lymphocytosis, they have fewer reactive
tonsils. Sometimes enlargement of the tonsils occurs to the extent that lymphocytes, and splenomegaly is less evident. This leads to the clinical
they touch each other in the midline. Blood lymphocytosis occurs in impression of infiltrative hepatic disease or cholecystitis. The illness at
response to the virus in the blood. Periorbital swelling, which occurs this age may be very protracted.
in mononucleosis, is an important clue to the diagnosis, even in young
adults. Other manifestations include lymphadenopathy, hepatitis and
splenic enlargement. (Table 82–2). 8,28 Although the liver is not an TABLE 82–3. Complications of Epstein-Barr Virus and
organ rich in lymphocytes and hepatocytes are not damaged by the Cytomegalovirus Mononucleosis
infection, CD4+ and CD8+ lymphocytes are trapped in that organ Complication Epstein-Barr Virus Cytomegalovirus
and their release of cytokines contributes to the inflammation in the Hemolytic anemia ++ +
liver and the changes in liver function that occur. However, hyperbi-
44
lirubinemia is very uncommon. The frequency of each clinical finding Thrombocytopenia + +
of the typical syndrome in newly infected patients is variable (Table Aplastic anemia + –
82–3). 28,29 The disease abates with the occurrence of a T-cell–mediated Splenic rupture + –
counterresponse to the virus-induced initial polyclonal B-cell prolif-
eration. During this time, dramatic clinical improvement can occur Jaundice (age ++ ++
in 24 to 48 hours. Subsequently, EBV remains in the patient’s B cells >25 years)
throughout life, but expresses only Epstein-Barr nuclear antigen-1 Guillain-Barré + ++
(EBNA-1), which does not elicit a T-cell response because of a gly- syndrome
cine-alanine repeat that inhibits its processing. 45 Encephalitis* ++ +/–
Group A streptococcus is found in the pharynx occasionally (3 to
4 percent of cases) in concert with an EBV primary infection. Although Pneumonitis* +/– +
treatment of the streptococcus eradicates the organism, the severe Myocarditis* + –
pharyngitis changes little, and the disease follows its usual course. B-cell lymphoma + –
Thus, treatment should be administered only if the test result for
β-streptococcus is positive. If a penicillin congener is used, quite often, Agammaglobulinemia + –
but not always, a rash develops 46,47 and the patient is labeled “penicil- ++, Common; +, less common; +/–, uncommon; –, not observed.
lin allergic.” The patient should be reevaluated after the mononucleosis
resolves to determine if the patient has a true allergy. *Can occur without the mononucleosis syndrome.

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