Acute Life-Threatening Hemorrhage in Neonates With Severe Hemophilia A: A Report of 3 Cases.

Alvaro Moreira,Hrishikesh Das

doi:10.1177/2324709618800349

Abstract

Hemorrhagic shock is a rare, emergent condition that is often fatal in newborns. In this article, we report cases of 3 neonates presenting with acute, life-threatening hemorrhage who were subsequently diagnosed with severe hemophilia (<1% factor VIII). The first infant was tachycardic, pale, and had a precipitous drop in his hemoglobin secondary to a subgaleal hemorrhage. The second patient sustained a splenic rupture, a sequela that has been reported in only 4 other neonatal cases. The last infant presented with tonic-clonic seizures and respiratory distress. Head imaging demonstrated extracranial and intracranial hemorrhage, complications that can result in 20% mortality. All 3 patients were successfully treated with clotting factor concentrate and blood products. After normalization of factor VIII levels, the newborns did not develop any new hemorrhages and were discharged home within 3 weeks of birth. Pediatric providers should be aware that these signs and symptoms may be potentially lethal complications in neonates with severe factor VIII deficiency.

Highlights

Hemophilia A (HA) is an X-linked recessive blood disorder characterized by decreased levels of coagulation factor VIII (FVIII)
We describe cases of 3 neonates who sustain extracranial, splenic, and intracranial hemorrhage (ICH)
Assessing family history of bleeding disorders is crucial for rapid diagnosis, as 50% of all males born to HA-carrying mothers will inherit the disorder

Summary

Introduction

Hemophilia A (HA) is an X-linked recessive blood disorder characterized by decreased levels of coagulation factor VIII (FVIII). Children with severe FVIII deficiency have multiple episodes of spontaneous bleeding per month and can range from minor mouth bleeds to severe cranial hematomas from slight head trauma.[4] In this case series, we describe cases of 3 neonates who sustain extracranial, splenic, and intracranial hemorrhage (ICH). The initial physical examination in the newborn nursery was unremarkable; at 6 hours of age, the infant acutely developed a bluish discoloration to the skin diffusely throughout his body, became hypotensive, and had marked abdominal distention. His hemoglobin was 5.5 g/ dL, and his PTT was greater than 100 seconds. A CT of the head showed a subdural hematoma on the right side and a subgaleal hematoma involving the left and right frontoparietal region (Figure 3)

Discussion

Findings

Conclusion