Neonatal alloimmune thrombocytopenia: Difference between revisions

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{{SK}}  NAITP;  NAIT; NAT; feto-maternal alloimmune thrombocytopenia ; FMAITP; FMAIT
==[[Neonatal alloimmune thrombocytopenia overview|Overview]]==


{{SK}}  NAITP;  NAIT; NAT; feto-maternal alloimmune thrombocytopenia ; FMAITP; FMAIT
==[[Neonatal alloimmune thrombocytopenia historical perspective|Historical Perspective]]==
==Overview==
 
'''Neonatal Alloimmune [[Thrombocytopenia]]'''  is a [[disease]] that affects [[fetus]]es and [[neonates|newborns]]. Genetic differences between the fetus and mother may result in the expression of certain antigens by fetal [[platelet]]s, not expressed by the mother. Fetomaternal transfusions result in the recognition of these antigens by the mother's immune system as non-self, with the subsequence generation of allo-reactive antibodies which cross the placenta. NAIT, hence, is caused by transplacental passage of maternal platelet-specific alloantibody and rarely [[human leukocyte antigen]] ([[HLA]]) allo-antibodies (which are expressed by platelets) to fetuses whose platelets express the corresponding antigens. NAIT occurs in somewhere between 1/800
==[[Neonatal alloimmune thrombocytopenia classification|Classification]]==
<ref>{{cite web
 
| url=http://www.orpha.net/data/patho/GB/uk-NAIT.pdf
==[[Neonatal alloimmune thrombocytopenia pathophysiology|Pathophysiology]]==
| work= Orphanet Encyclopedia
 
| title=Fetal and Neonatal Alloimmune Thrombocytopenia
==[[Neonatal alloimmune thrombocytopenia causes|Causes]]==
| author=Doctor Cecil Kaplan
 
| date=2003-11-01
==[[Neonatal alloimmune thrombocytopenia differential diagnosis|Differentiating Neonatal alloimmune thrombocytopenia from other Diseases]]==
| accessdate=2007-09-23
}}</ref>
and 1/5000
<ref>{{cite web
| url=http://bloodjournal.hematologylibrary.org/cgi/reprint/78/9/2276
| work= Blood Journal
| title=Prenatal Diagnosis of Neonatal Alloimmune Thrombocytopenia Using Allele-Specific Oligonucleotide Probes
| author=Janice G. McFarland, Richard H. Aster, James E. Bussel, John G. Gianopoulos, Rebecca S. Derbes, and Peter J. Newman
| date=1991-11-01
| accessdate=2007-09-23
}}</ref>
live births (more recent studies of NAIT seem to indicate that it occurs in somewhere between 1/800 and 1/1000 live births).


==Signs and Symptoms==
==[[Neonatal alloimmune thrombocytopenia epidemiology and demographics|Epidemiology and Demographics]]==
Frequently, the thrombocytopenia is mild and the affected neonates remain largely asymptomatic. In these cases, therapeutic interventions are not indicated. In case of severe thrombocytopenia, the neonates may exhibit hemorrhagic complication at or a few hours after delivery. The most serious
complication is intracranial hemorrhage, leading to death in approximately 10% or neurologic sequelae in 20% of cases.


==Diagnosis==
==[[Neonatal alloimmune thrombocytopenia risk factors|Risk Factors]]==
Although there are currently no widespread tests for NAIT, platelet antigen genotyping and platelet antibody identification can be performed on the maternal and paternal blood to determine the exact nature of the incompatibility.  The Blood Center of Wisconsin Platelet & Neutrophil Immunology Laboratory is currently recognized as a leader on research on NAIT and provides NAIT testing using maternal and paternal blood samples.


==Causes==
About 80% of cases of NAIT are caused by antibodies against platelet antigen [[HP-1a]], 15% by anti-[[HP-5a]], and 5% by other antibodies. Unlike the
hemolytic disease, NAIT occurs during the first pregnancy in to 50% of cases, and the affected fetuses may develop severe thrombocytopenia (<50,000 /μL) very early during pregnancy. Usually, the thrombocytopenia increases as gestation progresses. In utero intracranial hemorrhage occurs in about 10% of affected cases. This complication may also take place before 20 weeks of gestation. The recurrence of NAIT been estimated to be more than 80% in subsequent pregnancies with incompatible fetuses.


==Treatment==
==[[Neonatal alloimmune thrombocytopenia screening|Screening]]==  


===During Pregnancy===
The use of [[Intravenous immunoglobulin]] ([[IVIG]]) during pregnancy and immediately after birth has been shown to help reduce or alleviate the affects of NAIT in infants and reduce the severity of thrombocytopenia.  The most common treatment is weekly [[IVIG]] infusions at a dosage of 1g/kg beginning at 20 weeks of pregnancy and continuing until the birth of the child<ref>{{cite web
| url=http://www.emedicine.com/med/topic3256.htm
| work= eMedicine
| title=Immune Thrombocytopenia and Pregnancy
| author=Lynnae Millar, MD
| date=2006-06-29
| accessdate=2007-09-19
}}</ref>. In some cases this dosage is increased to 2g/kg and/or combined with a regiment of [[Progesterone]] depending on the exact circumstances of the case.  Although this treatment has not been shown to be effective in all cases it has been shown to reduce the severity of thrombocytopenia in some.


===After Birth===
==[[Neonatal alloimmune thrombocytopenia natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
The most rapidly effective treatment in infants with severe hemorrhage and/or severe thrombocytopenia (<30x10<sup>9</sup>/L) is the transfusion of compatible platelets (i.e. platelets from a donor who, like the mother lacks the causative antigen). Additionally if the thrombocytopenia in the infant at birth is not severe enough to warrant a transfusion of platelets (>30x10<sup>9</sup>/L) an infusion of [[IVIG]] (1g/kg/day for two days) in the infant has been shown to rapidly increase platelet count and reduce the risk of related injury.


==Resources==
==Diagnosis==
As of September 23, 2007 there is an active Yahoo group that is dedicated to helping those that are affected by NAIT. The group can be found at http://health.groups.yahoo.com/group/NAIT/
[[Neonatal alloimmune thrombocytopenia history and symptoms| History and Symptoms]] | [[Neonatal alloimmune thrombocytopenia physical examination | Physical Examination]] |[[Neonatal alloimmune thrombocytopenia laboratory findings|Laboratory Findings]] | [[Neonatal alloimmune thrombocytopeniachest x ray|Chest X Ray]] |  [[Neonatal alloimmune thrombocytopenia CT|CT]] | [[Neonatal alloimmune thrombocytopenia MRI|MRI]] | [[Neonatal alloimmune thrombocytopenia ultrasound|Ultrasound]] | [[Neonatal alloimmune thrombocytopenia other imaging findings|Other Imaging Findings]] | [[Neonatal alloimmune thrombocytopenia other diagnostic studies|Other Diagnostic Studies]]


==References==
==Treatment==
{{reflist|2}}
[[Neonatal alloimmune thrombocytopenia medical therapy|Medical Therapy]] | [[Neonatal alloimmune thrombocytopenia surgery|Surgery]][[Neonatal alloimmune thrombocytopenia primary prevention|Primary Prevention]] | [[Neonatal alloimmune thrombocytopenia secondary prevention|Secondary Prevention]] | [[Neonatal alloimmune thrombocytopenia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Neonatal alloimmune thrombocytopenia future or investigational therapies|Future or Investigational Therapies]]




==Case Studies==
[[Neonatal alloimmune thrombocytopenia case study one|Case#1]]


[[Category:Hematology]]
[[Category:Hematology]]
[[Category:Obstetrics]]
[[Category:Obstetrics]]
[[Category:Neonatology]]
[[Category:Neonatology]]
 
[[Category:Disease]]
{{WH}}
{{WH}}
{{WikiDoc Sources}}
{{WikiDoc Sources}}

Revision as of 16:05, 21 September 2012

Neonatal Alloimmune Thrombocytopenia
ICD-10 P61.0
ICD-9 776.1
DiseasesDB 33767

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: NAITP; NAIT; NAT; feto-maternal alloimmune thrombocytopenia ; FMAITP; FMAIT


Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Neonatal alloimmune thrombocytopenia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination |Laboratory Findings | Chest X Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | SurgeryPrimary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies


Case Studies

Case#1 Template:WH Template:WikiDoc Sources

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