Purpose
Neonatal immune neutropenia is observed in rare cases in newborns from mothers with idiopathic or autoimmune neutropenia, secondary to passive transfer of maternal granulocyte ...auto-antibodies.
Methods
We performed a literature review and report four supplementary cases from the French registry of neutropenia.
Results
Only 14 cases (11 mothers, 14 newborns) have been reported. Granulocyte aggregation (GAT) and granulocyte indirect immunofluorescence test (GIFT) are the recommended laboratory procedures for antibody detection. Monoclonal antibody-specific immobilization of granulocyte antigens (MAIGA)-confirmed antibody specificity.
Antibody detection in newborns is not generally possible owing to extreme neutropenia. In half of the cases autoantibodies against neutrophils (AAN) were positive in maternal sera (7 out of 11). In some newborns tested, IgG
+
AAN were also positive, with disappearance in parallel of spontaneous neutrophil count improvement. No correlation between maternal type of AAN and titer and neonatal neutropenia can be established. Neutropenia resolved spontaneously between 2 weeks and 4 months. Infections in newborns were observed in 43% of cases, with no deaths reported. Granulocyte colony-stimulating factor (G-CSF) was administered to some newborns (5 out of 14) in the case of infections. Low-dose G-CSF administered to childbearing women during pregnancy could be proposed to prevent neutropenia in newborns.
Conclusions
From the few cases reported so far it is impossible to draw any conclusions regarding frequency, risk factors, and outcome, but the overall prognosis for newborns seems good. Because it can be associated with potentially severe neonatal infections, autoimmune neutropenia in childbearing mothers should be closely monitored in collaboration with gynecologists and pediatricians.
Amyloidosis(A) prognosis is determined by cardiac involvement.The main types of A are immunoglobulin light chain(AL) and transthyretin-related(TTR), which can be mutated(TTRm) or senile(TTRwt). ...Specific treatments can’t be administrated unless A has been typed histologically.Literature suggests echocardiography,99mTc DPD scintigraphy and cardiac MRI could help typing A. We described these imaging modalities to assess these potential tools for an uninvasive typing.
We analysed these imaging modalities in patients examined at Cardiomyopathies Competence Center(CCC) of La Timone Hospital in Marseille, with an histologically proven diagnosis of cardiac A(CA).
We included 75patients examined between September 2006 and March 2014 at CCC, with a strongly suspected diagnosis of CA.CA could be histologically confirmed and typed in 45 patients(10 TTRm, 4 TTRwt, 6 TTR undeterminated; 19 AL;6 of other type).In 11 patients, CA was confirmed but untyped.No statistically significant difference was found between TTR and AL patients for the various imaging modalities.We observed 71% of men, aged 66, NYHA stage 2,4 on average.In all patients, cardiac biomarkers rates were increased. Myocardic mass and interventricular septal thickness were increased (199g/m² and 19mm), restrictive filling pattern was observed in 83% of patients.Despite a relatively preserved left ventricular ejection fraction, Global Longitudinal Strain was impaired at – 11%, with an apical sparing. Scintigraphy showed a frequent myocardic fixation (69%), slightly more intense in TTR patients.Cardiac MRI showed a constant late gadolinium enhancement, more extended in AL patients.
We didn’t observe the differences described between CA types, probably because of a lack of statistical power.This encouraged us to develop a protocol for multidisciplinary evaluation of CA, to improve the management of this disease, and to keep on evaluating the diagnostic accuracy of these imaging modalities.
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