Portal vein thrombosis, livedo reticularis, polymicrobial sepsis and high antiphospholipid antibody titers in a newborn: A clinicopathological conference of antiphospholipid-associated neonatal syndrome
| Autor: | Murugan Sudhakar, Kirti Gupta, Gopalakrishnan Ezhumalai, Ritambhra Nada, Pooja Jindal, Vignesh Pandiarajan, Jogender Kumar, Himanshi Chaudhary |
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| Rok vydání: | 2020 |
| Předmět: |
Pathology
medicine.medical_specialty Umbilical vein Sepsis 03 medical and health sciences 0302 clinical medicine Fatal Outcome Rheumatology immune system diseases Antiphospholipid syndrome medicine Humans neoplasms Livedo reticularis Livedo Reticularis Venous Thrombosis business.industry Portal Vein Infant Newborn medicine.disease Antiphospholipid Syndrome Thrombosis Portal vein thrombosis Venous thrombosis Antibodies Antiphospholipid Female Autopsy medicine.symptom business 030217 neurology & neurosurgery 030215 immunology Rare disease |
| Zdroj: | Lupus. 30(1) |
| ISSN: | 1477-0962 |
| Popis: | Neonatal Antiphospholipid syndrome (APS) is a rare disease related to transplacental passage of antiphospholipid (aPL) antibodies from the mother or de novo production of aPL in a newborn. Neonatal aPL antibodies have rarely been associated with thrombosis. We describe a 5-week-old infant who developed fever, portal vein thrombosis and livedo reticularis like skin rash. Evaluation for thrombosis revealed high titers of antiphospholipid (aPL) antibodies (dual positive) in the child without any evidence of aPL antibodies in the mother, suggesting a de novo production in the child. Autopsy findings revealed umbilical vein sepsis with thrombosis of portal vein secondary to gram positive cocci which led to multiple liver and lung abscesses. Additionally, the baby had disseminated Cytomegalovirus (CMV) disease (acquired postnatally) involving walls of umbilical and portal vein, liver, lungs, adrenals, pancreas, thymus, and kidneys. Our case highlights the need for testing of aPL in every neonate with arterial or venous thrombosis even when the mother may have no features suggestive of an autoimmune disease. |
| Databáze: | OpenAIRE |
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