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2023

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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HEPATITITS FOLLOWED BY MEDULLARY HYPOPLASIA – WAS LEPTOSPIRA THE CULPRIT?

Rute Baeta Baptista1, Raquel Costa2, Raquel Maia3, António Pedro Campos4, Flora Candeias1, Maria João Brito1

1- Unidade de Infecciologia, Área de Pediatria Médica, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa;
2- Pediatria, Hospital do Espírito Santo, Évora;
3- Unidade de Hematologia, Área de Pediatria Médica, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa;
4- Unidade de Gastrenterologia e Hepatologia, Área de Pediatria Médica, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa;

- 7th Excellence in Pediatrics Conference, 10 a 12 de Dezembro de 2015, Londres

Resumo:
Introduction - Leptospirosis is a spirochetal zoonosis which clinical presentation varies from subclinical to potentially fatal Weil’s disease. Pancytopenia has been reported as a rare presentation of severe leptospirosis in adult patients.
Clinical Case Report - A 12 year-old healthy boy developed fever, progressive asthenia and non-productive cough. Two weeks later, jaundice and hepatomegaly became apparent. Laboratory workup showed total bilirubin 16.34mg/dL, direct bilirubin 14.07mg/dL, aspartate aminotransferase 1566U/L, alanine aminotrasferase 2360U/L, g-glutamyltranspeptidase 318U/L. Blood cell count, electrocardiography and chest X-ray were normal. Abdominal ultrasound revealed homogeneous hepatomegaly with no signs of biliary dilatation or space occupying lesions. Leptospira were visualized in the urine by dark field microscopy and confirmed by real-time polymerase chain reaction (rt-PCR). The patient was treated with penicillin, with progressive clinical and liver function tests recovery. At four weeks after the onset of jaundice, progressive asymptomatic pancytopenia was noted (minimum values of haemoglobin 10.7g/dL, leucocytes 1.100/uL, neutrophils 400/uL, platelets 61x109/L). Direct coombs test was negative. Autoimmune screening was normal. A bone marrow biopsy showed medullary hypoplasia (20-30% cellularity). Marrow culture was negative. DNA from parvovirus B19 was detected in medullary blood by rt-PCR. The patient showed progressive spontaneous recovery of blood cell count with full recovery at 6 months after presentation.
Conclusion - Although pancytopenia has been reported in severe acute leptospirosis, in the reported case it is more likely to represent hepatitis associated medullary hypoplasia (immunological mediated marrow failure that follows the development of hepatitis). A concurrent parvovirus B19 infection may also have been implicated.

Palavras Chave: leptospirosis; hepatitis; medullary hypoplasia; parvovirus B19