A 16-year-old boy consults for presenting with pustular tonsillitis, fever up to 38.5°C, painful cervical lymphadenopathy, non-pruritic macular rash on the chest and mild hepatosplenomegaly, of 4-5 days of evolution. Paul-Bunnell test and Ig M for Epstein-Barr virus are positive. During his admission he developed continuous fever up to 40ºC, pancytopenia, icteric hepatitis and coagulopathy of progressive intensity. One week after admission, he was transferred to the ICU due to confusion and respiratory failure. Blood cultures and a urine culture were negative, CSF was normal and the chest x-ray showed no infiltrates. Procalcitonin is normal, but CRP and ferritin are very elevated. Of the following statements, which would be the most correct diagnostic and therapeutic approach?
Difficult question in which the correct answer could give rise to discussion. The patient is a 16-year-old male who is admitted for a fairly clear case of mononucleosis due to EBV infection that becomes complicated during admission. The data available to us are: - Pancytopenia. - Hepatopathy. - Coagulopathy. - Absence of infectious focus: negative blood cultures, negative urine cultures, normal X-ray, normal CSF. - CRP and Ferritin elevated / normal Procalcitonin. The possible complications that could have occurred (and that are considered in the answers) are sepsis or a bad evolution of mononucleosis. The clinical data could be consistent with a septic process, however, the main infectious foci have been reasonably ruled out and one of the most sensitive and specific markers of bacteremia (procalcitonin) is in the normal range, which should draw our attention. On the other hand, a complication of the EBV mononucleosis suffered by our patient should be evaluated, among the possible complications are hemolytic anemia, meningoecephalitis (normal CSF) or Guillain-Barré sd. Guillain-Barré syndrome, which are not very consistent with the clinical picture presented to us. Another possible complication of exceptional severity would be hemophagocytic syndrome; this is a rare entity that occurs in certain predisposed subjects and is characterized by an uncontrolled activation and proliferation of histiocytes and T lymphocytes, which produces a state of hypercytokinemia.  The diagnostic criteria, revised in 2004, include persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, hyperferritinemia, and hypofibrinogenemia. Hyperferritinemia >3000 in a suggestive clinical context necessitates treatment of the patient (mainly with immunosuppressants and corticosteroids) since mortality is very high and early treatment is essential. The definitive diagnosis is made by microscopic study of an OM biopsy.  Although in this question they do not quantify the value of ferritinemia, it is a remarkable data and given that the clinical picture is suggestive, meeting the diagnostic criteria, it seems to me that answer 5 would be the most accurate.
She has a bacterial sepsis of undetermined origin and should be given ceftriaxone and supportive care.
She has bacterial sepsis of undetermined origin and should be given vancomycin, ceftacidime and supportive care.
This is infectious mononucleosis with a severe course and glucocorticoids should be administered.
This is an infectious mononucleosis of severe course and treatment with Acyclovir should be initiated.
I would perform a bone marrow biopsy/aspirate and, if hemophagocytosis is confirmed, initiate treatment with immunosuppressants.
The clinical data could be consistent with a septic process, however, the main infectious foci have been reasonably ruled out and one of the most sensitive and specific markers of bacteremia (procalcitonin) is in the normal range, which should draw our attention.
The clinical data could be consistent with a septic process, however, the main infectious foci have been reasonably ruled out and one of the most sensitive and specific markers of bacteremia (procalcitonin) is in the normal range, which should draw our attention.
On the other hand, a complication of the EBV mononucleosis suffered by our patient should be evaluated, among the possible complications are hemolytic anemia, meningoecephalitis (normal CSF) or Guillain-Barré sd. Guillain-Barré syndrome, which are not very consistent with the clinical picture presented to us.
On the other hand, a complication of the EBV mononucleosis suffered by our patient should be evaluated, among the possible complications are hemolytic anemia, meningoecephalitis (normal CSF) or Guillain-Barré sd. Guillain-Barré syndrome, which are not very consistent with the clinical picture presented to us.
Hyperferritinemia >3000 in a suggestive clinical context necessitates treatment of the patient (mainly with immunosuppressants and corticosteroids) since mortality is very high and early treatment is essential. The definitive diagnosis is made by microscopic study of an OM biopsy.