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Neonatal Alloimune Thrombocytopenia (NAIT)

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Low platelet count in an otherwise healthy term newborn is due to NAIT until proven otherwise. Urgent matched platelet transfusion should be discussed with specialist on call at all times.

Background

  • Neonatal Alloimmune Thrombocytopenia (NAIT) results from maternal human platelet antibodies (HPA) against fetal platelet antigens inherited from the father. In contrast to rhesus haemolytic disease, platelet allo-immunization can occur during the first pregnancy.
  • NAIT presents commonly in the newborn with unexpected bruising and purpura or can be the cause of severe intracranial haemorrhage.
  • The possibility of allo-immunization during pregnancy is particularly high for HPA-1a, -5b and -15b among Europeans, for HPA-2b and HPA-3a among Maori, for HPA -4b among Asians, and for HPA-6b among Polynesians. The picture becomes more complex for families with mixed ethnic backgrounds.1
  • The incidence of NAIT is reported to be between 1:800 and 1:1000. In clinically presenting cases, the rate of intracranial haemorrhage is ~20% followed by death in 10% and neurological sequelae in 20% of these newborns.2

Presentation

The commonest mode of presentation is the well neonate with bruises or petechiae, but the spectrum of disease ranges from sub-clinical moderate thrombocytopenia to catastrophic intracranial haemorrhage and death. A high index of suspicion is essential in all cases of active bleeding, but also in asymptomatic laboratory diagnosed thrombocytopenia. A history of thrombocytopenia in a previous sibling makes the diagnosis almost certain.

Modes of presentation

  • Bruising/bleeding neonate
  • Excessive haematoma at injection site
  • Previously affected sibling
  • Recurrent fetal loss and stillbirth
  • Antenatal ICH/hydrocephalus
  • Disseminated intravascular coagulopathy
  • Postnatal ICH (e.g.: silent, full fontanel, seizures)

Differential Diagnosis

Also see Neonatal Thrombocytopenia  guideline

Timing Most Common Aetiology
Fetal Allo-immune (e.g. NAIT)
Congenital infection
Aneuploidy
Autoimmune (e.g. ITP, SLE)
Inherited (e.g. Wiskott-Aldrich syndrome)
Early-Onset Placental insufficiency
Perinatal asphyxia
Early onset sepsis
Congenital infection
DIC
Allo-immune
Autoimmune
Kasabach-Merritt syndrome
Metabolic disease
Inherited
Late-Onset Sepsis
NEC
Infection
Autoimmune
Kasabach-Merritt syndrome
Metabolic disease
Inherited
Thrombus

Investigation

Further testing should not delay platelet transfusion if required urgently

Infant FBC to confirm platelet count
If no paternal blood available or paternity is uncertain, send 1 x infant EDTA (purple, 1 mL) tube for genotyping
Mother FBC: Normal platelet count during pregnancy
4 x CPDA (yellow) tubes for genotyping and 1x plain (red) tubes for anti-platelet antibodies.
Father 4 x CPDA (yellow) tubes for genotyping
  • If platelet transfusion is needed, ring haematologist on call to discuss.
  • Definitive diagnosis of NAIT depends on parental testing.
  • Ideally, matching with maternal antibodies is preferred prior to platelet transfusion.
  • Phone laboratory with specimen queries (523 5731).

Management

Platelet Count (x109 Action
<30 Transfuse
30-49 Transfuse if any bleeding or high risk
50-99 Transfuse if major bleeding
>99 Do not transfuse

Transfusion

  • If transfusion is needed, discuss the case with a clinical haematologist.
  • Urgent transfusion of 10 mL/kg of platelets (over 30-60 min) is needed if the infant is bleeding or at high risk of bleeding. Infants are at significant risk for ICH in the first days of life.
  • Matched platelets are first choice, but may not be available in a timely fashion.
  • If matched platelets are not available in a timely fashion, unmatched platelets (consider addition of IvIg) should be given.

IvIg

  • Consider IvIg (1-2 g/kg) in combination with unmatched platelets if matched platelets are not available in a timely fashion.5
  • IvIg as stand-alone treatment may be considered, but response can be delayed by 24-36 hours, leaving a window of risk for ICH.

Other considerations

  • Ensure Vitamin K has been administered
  • Urgent head ultrasound
  • Infants are at significant risk of ICH in the first days of life.
  • If the platelet count fails to rise in response to matched platelets consider alternative diagnoses, and check for rarer maternal antibody types.
  • Affected infants should have a FBC checked daily until platelet count is stable over 100x109/L.
  • Maternal antibody levels fall towards term, so repeat testing 6 weeks post-partum is required in some cases.
  • It is essential that parental investigation is initiated prior to discharge and adequate follow up arrangements made.
  • The risk of a subsequent pregnancy being affected is 100% if the father is homozygous for the reacting antigen, and 50% if heterozygous.
  • In general, subsequent pregnancies are at least as severely affected as the first. Antenatal therapy in subsequent pregnancies remains contentious, but options include intrauterine platelet transfusion, maternal immunoglobulin and steroids.5 If NAIT is confirmed, future pregnancies should by managed by the Maternal Fetal Medicine Team.

References

  1. Bakchoul T, Bassler D, Heckmann M, Thiele T, Kiefel V, Gross I, et al. Management of infants born with severe neonatal alloimmune thrombocytopenia: the role of platelet transfusions and intravenous immunoglobulin. Transfusion. 2014;54:640-5.
  2. Kaplan C. Foetal and neonatal alloimmune thrombocytopaenia. Orphanet J Rare Dis. 2006 Jan;1:39.
  3. Roberts I, Murray N a. Neonatal thrombocytopenia. Semin Fetal Neonatal Med. 2008;13:256-64.
  4. Edinur H a, Dunn PPJ, Lea R a, Chambers GK. Human platelet antigens frequencies in Maori and Polynesian populations. Transfus Med. 2013;23:330-7
  5. Transfusion Medicine Handbook. 2008;New Zealand Blood Service, www.nzblood.co.nz

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Document Control

  • Date last published: 31 August 2015
  • Document type: Clinical Guideline
  • Services responsible: Neonatology
  • Owner: Newborn Services Clinical Practice Committee
  • Editor: Sarah Bellhouse
  • Review frequency: 2 years