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2019

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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IMMUNOLOGICAL RECONSTITUTION INFLAMMATORY SYNDROME AND THROMBOTIC MICROANGIOPATHY - SEVERE COMPLICATIONS IN A CHILD WITH AIDS

Ana Paula Rocha1, Tiago Silva1, Telma Francisco2, Conceição Neves3, Vera Brites4, Flora Candeias1

1 - Unidade de Infecciologia, Área da Criança, Adolescente e Mulher, Hospital Dona Estefânia, CHLC
2 - Unidade de Nefrologia, Área da Criança, Adolescente e Mulher, Hospital Dona Estefânia, CHLC
3 - Unidade de Imunodeficiências Primárias, Área da Criança, Adolescente e Mulher, Hospital Dona Estefânia, CHLC
4 - Unidade de Cuidados Intensivos Pediátricos, Área da Criança, Adolescente e Mulher, Hospital Dona Estefânia, CHLC

- 36th Annual Meeting of the European Society for Paediatric Infectious Diseases, Malmo, Sweden, sob a forma de poster

Resumo:
Introdução: Although rare in developed countries, immunological reconstitution inflammatory syndrome (IRIS) is still a severe problem in advance immunosuppression caused by HIV infection. This condition is associated with many complications, including secondary thrombotic microangiopathy (TMA).
Relato de caso: Ten-month-old boy diagnosed with HIV/AIDS in the context of a Pneumocystis jirovecii pneumonia, with an initial viral load (VL) of 1460000 copies/mL and CD4 9/uL. Highly active antiretroviral therapy (HAART) was started (lamivudine, zidovudine, lopinavir/ritonavir). About 2 weeks after starting HAART, he developed fever, diarrhea that became mucous and bloody. At the time he had a VL of 3280copies/mL and 109 CD4/uL (1200% increase). He also showed anemia (8,6x10g/L), thrombocytopenia (68x109/L), hepatic tests elevation (AST 141U/L, ALT 218U/L), hypertriglyceridemia (190mg/dL), hypofibrinogenemia (0,8g/L), hyperferritinemia (1194ng/mL) and low sedimentation rate (4mm/h). Soluble CD25 was 22167 pg/mL.Macrophagic activation syndrome in the context of IRIS was assumed and he was started metilprednisolone (2mg/Kg/day) and iv immunoglobulin with temporary clinical and analytical improvement, although with persistent diarrhea. Despite extensive investigation, including colonoscopy with biopsy for opportunistic infectious agents, none was identified. Clinical deterioration was noted after one month with worsening of thrombocytopenia (minimum 8x109/L), anemia (minimum 4,2x10g/L), presence of schizocytes on the blood smear, LDH elevation (1748U/L), low haptoglobin (<0,07g/L) and low ADAMTS activity (0,17). Assuming TMA secondary to HIV and with no improvement after plasma infusion, plasmapheresis was initiated with sparse response, and the patient died in disseminated intravascular coagulation with massive intestinal blood loss.
Conclusões: This child had a very severe course after HAART initiation, with development of IRIS and TMA, which are in agreement to the dismal outcome. Severe persistent diarrhea may have been caused by HIV intestinal lymphatic tissue infection or intestinal microangiopathy.

Palavras Chave: HIV; Immunological reconstitution inflammatory syndrome; Thrombotic microangiopathy