SEVERE MIXED INMUNODEFICIENCY ASSOCIATED TO THYMIC APLASIA AND POLYMICROBIAL INFECTIONS NOT RESPONSIVE TO TREATMENT: A CASE REVIEW

Huerta JF, Pacheco E, Amaro L, Escobar V, Contreras A, Lopez G

Centro Medico Nacional ¡°20 de Noviembre¡± I.S.S.S.T.E. Mexico D.F., Mexico

 

Male infant four months old, from La Paz, Baja California, M¨¦xico. He was admitted on may 5, 1998 in Internal Medicine Department. Background: parents, cousins, brothers and a sister who died. His sister died when she was seen months old; she had Di George Syndrome diagnosis. Perinatal background was negative; he had oral moniliasis when he was 18 days old, faryngitis and conjunctivitis treated with ketoconazol, with evolution toward pneumonia treated with cefotaxime/amikacine during ten days. We found permanent lymphocytopnenia and when he arrived to the hospital he had clinical and radiological pneumonia¡¯s findings, and the blood sample showed leucocytosis with lymphocytos 8 %, and C-reactive protein (CRP) 7.4, reason why he was transferred to Infectology Department where he was treated with cefotaxime and amikacine. We found Gastroesophageal reflux III and the blood sample continued with lymphocytopenia and high CRP level.The CT Scan showed a consolidation area and brochiectasis diagnosis versus middle lobe cavitation was done. We started the treatment with gamma inmune globulin and transfer factor, with mixed inmunodeficiency versus common inmunodeficiency diagnosis, probably Di George Syndrome. The lymphocytic subpopulations were done, reporting not cellular sample. A new CT Scan showed a bullae and pachypleuritis. A decortication and right lobectomy was done reporting numerous abscesses, reason why we decided to change the treatment to imipenem and vancomycin. CD4, CD8, CD19 and CD21 markers were low with CD4/CD8 ratio inverted. Twenty days later he developed spontaneous pneumothorax treated with a seal water and dicloxacilin, getting a lot of purulent material, Staphylococcus epidermidis and Streptococcus inmitis were isolated from secretions. The blood sample showed leucocytosis and lymphocytopenia with CRP higher than 20. In thoracotomy secretion were isolated: Rodococcus sp, Staphylococcus epidermidis and Haemophillus influenzae III; then he wastreated with ceftriaxone and vancomycin, A new CT Scan revealed septum lung abscess and a directed CT Scan punction was done getting a lot of purulent material lymphocytosis was in 8%. On july 20 1998 he had meningeal and focussing findings in brain CT Scan, with numerous brain abscesses, ependymitis, aracnoiditis and obstructive hydrocephalus. He had a slow evolution until he died. Autopsy revealed thymic tissue less than one grame, that is the most important issue for supporting for Nezelof Syndrome diagnosis.

 

 
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