Hemophagocytic Syndrome Secondary to Infection Extrapulmonary Tuberculosis in the Pediatric Population, a Condition Difficult to Diagnose
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Hemophagocytic lymphohistiocytosis (HLH)
is characterized by activation and proliferation
of lymphocytes or histiocytes, which secondarily exhibit uncontrolled hemophagocytosis and
cytokine overproduction. Acute onset of fever,
generalized lymphadenopathy, hepatosplenomegaly, hypofibrinogenemia and hypertriglyceridemia characterized the syndrome. Its etiology may
be primary or secondary. The primary HLH is
related to genetic factors, and the secondary, associated with infectious, neoplastic and autoimmune processes. The HLH associated infection
occurs predominantly in immunocompromised
patients, and is usually fatal. The vast majority
of cases are reported with herpesvirus infections.
However, a lot of microorganisms are associated
with the etiology. Tuberculosis is an infection recently described as a cause of secondary HLH.
The association between HLH and tuberculosis
is difficult to diagnose. The possibility of finding
the M. tuberculosis in cultured tissue samples
and pleural fluid is very limited because the
extrapulmonary TB is a paucibacillary process.
Among the diagnostic tools to measure levels of adenosine deaminase in pleural fluid has adequate diagnostic sensitivity. Cut-off values are still a
matter of controversy.
hemophagocytic syndrome, extrapulmonary tuberculosis, adenosine deaminase.linfohistiocitosis hemofagocítica, tuberculosis extrapulmonar, adenosina deaminasa.
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