[CIS-PAGID] Peruvian patient with EBV associated lymphoproliferation

[Juan Carlos Aldave Becerra]

Good morning dear Doctors, I was presented this case from Peru: Female patient

4 years old

Parents are not blood relatives.

No previous medical history before 4 years
of age.

Normal psychomotor development



May 2011: First hospitalization
for Probable Infection by Epstein Barr Virus (fever; cervical, mesenteric and
mediastinal lymphadenopathy (4 mm); hepatosplenomegaly; hypergammaglobulinemia).



Bone marrow smear:

·        
 Lymphocytic marrow infiltration versus
reactive lymphoproliferative syndrome (June 2011)


Bone marrow biopsy

·        
90% cellularity,
presence of all three series, 40% CD8 cytotoxic T lymphocytes of mature
appearance (June 2011)

Liver biopsy:

·        
Extramedullary
hematopoiesis (June 2011)


Lymph node biopsy:

·        
Paracortical
hyperplasia with no evidence of malignancy  (June 2011)

 

September 2011: Second
hospitalization: sudden onset of fever, cough and respiratory distress, managed
in ICU for septic shock with respiratory focus, needing oxygen and inotropic
support; oliguria, anemia (received transfusion), hepatosplenomegaly.

Laboratory: 
mild anemia, lymphocytosis, elevated liver enzymes, and
hypergammaglobulinemia


Leukocytes: 11.400 cells/mm3

Lymphocytes: 8.430 cells/mm3

Neutrophils: 1999 cells/mm3


Hemoglobin: 10.1 g / L

Platelets: 343 000 cel/mm3

C reactive protein: 5.3 

Creatinine: 0.21 mg / dl

ALT: 142 IU; AST: 127

Alkaline phosphatase: 470 (normal up to 300)

Albumin: 3.32 g / dl

Total Protein: 9.14 g / dl

Urinalysis: normal

Haptoglobin: normal

IgA: 217 mg / dl

IgM: 369 mg / dl

IgG: 2401 mg / dl

IgE: 41.9 mg / dl

EBV EBNA IgG: positive

EBV EBNA IgM negative

EBV VCA IgG positive

Direct Coombs positive (1+)

Fibrinogen: normal

LDH 967 (high)

Electrophoretic proteinogram: hyperproteinemia, polyclonal increase of gamma
fraction 

Epstein Barr Virus Viral load (PCR):
1070 copies / ml

Cytomegalovirus viral load <600 copies

Bronchoscopy: thick secretions and fluffy whitish plaques

TCR clonality study: negative

Negative blood cultures

Autoantibodies (ANA, ANCA, ASMA, Anti KLM1, antithyroid): negative

Negative PPD

Negative viral markers for hepatitis



The patient has received IV acyclovir for 21
days; fluconazole for 10 days.
Good evolution from the respiratory standpoint. No fever. Persistent hepatosplenomegaly
(7 cm below the costal margin) and cervical microadenopathies. I have read about the PID that predispose to EBV infections and, because of the gender of the patient, I suspect of Itk deficiency. Please feel free to make me the suggestions that you have.Thank you very much, Juan Carlos AldaveAllergy and Clinical ImmunologyLima-Peru   		 	   		  
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