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.