An 8-month-old male patient with a history of hypermelanoma pustulosa in the neonatal period presented with mild communication and CMPA.
She consulted for 5 days of high fever and abdominal distension.
A blood count with 5,800/mm3 leukocytes, ESR 9 mm/h, C-reactive protein 20 mg/l, and normal complete urine.
Examination revealed erythematous SLNB scar with left axillary lymphadenopathy of 1 cm in diameter.
The PCR for herpes virus 6 was positive, with the rest of the negative bacteriological and virological studies.
Abdominal ultrasound showed mild hepatomegaly.
Liver tests and bone scintigraphy were normal.
The immunoglobulin G, A and M counts were low.
Febrile fever, urinary infection caused by Proteus and Enterococcus, which is treated with cefoxime.
GEEV was administered to the ICU.
Episodes with abdominal disfunction and ultrasound guidance revealed ascitic fluid and increased lymph node size.
Having suspected immunodeficiency and BCG dissemination, it was decided to perform a pathological study of adenopathy month, identifying Candida lusitaniae, treated with antifungals for 3 weeks.
Because BCG cannot be ruled out, disseminated tuberculosis treatment was initiated.
Episodes with sepsis and abdominal obesity syndrome, need for MV and severe vasoactive drugs.
With reduced lymphocyte subpopulations in number, it is complemented by lymphoproliferative study and altered lymphocyte activation.
The patient presented decreased respiratory burst, characteristic pathological study of ganglionic granulomatous disease (CGE).
The study of respiratory burst performed to the mother, clinically healthy, is altered.
It is treated with serial VEGG and interferon gamma.
Subsequently, umbilical cord transplantation is performed, complicated by autoimmune hemolytic anemia that responds to rituximab.
It remains under regular monitoring until the current age of 3 years.
