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EMAD LAWENDY MDPGY2
October 2009
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Aim of this PowerPoint1. Discuss the incidence of neonatal
thrombocytopenia.2. Describe the risks of a low platelet count in
neonates .3. Develop DD for neonate who have
thrombocytopenia based on time of presentation risk factors , signs and symptoms .
4. Explain the potential mechanisms responsible for nonimmune thrombocytopenia.
5. Develop a management plan for neonates who have immune or nonimmune thrombocytopenia.
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Classification and incidence of neonatal thrombocytopeniaTraditionally defined as platelet count <
150.000 /mcL ( mild < 100.000 and moderate 50.000-99.000/mcL)
1. 18-35% of all neonates admitted to NICU develop thrombocytopenia at some point during their NICU stays.
2. The incidence of thrombocytopenia is inversely related to the gestational age.
3. 73% of ELBW infants has one or more record during the 1st postnatal week.
4. 85% in neonates less than 800 grams.5. 60% in neonates 801 – 900 grams.
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Neonatal platelet functions and risks of ThrombocytopeniaThere is developmental deficiencies in
primary hemostasis in preterm neonates during the first 1 and 2 weeks after birth .
The question of whether ( and to what degree) such haemostatic abnormalities contribute to the pathophysiology of IVH remains unanswered.
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Approach to the Neonate who has ThrombocytopeniaTime of presentation is one of the most
helpful pieces in evaluation ( pathologic process in 1st 72 hrs differ than after 72 hrs.
Early onset thrombocytopenia1- Severity of thrombocytopenia2-Clinical presentation 3- Maternal history
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If the mother has autoimmune thrombocytopenia -- the most likely diagnosis in an otherwise well appearing neonate is autoimmune thrombocytopenia mediated by the placental passage of maternal autoantibodies .
Mother with preeclampsia or chronic hypertension most common cause of mild thrombocytopenia in healthy appearing neonate . - can be managed with close observation
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Thrombocytopenia can be the first presenting sign of a serious condition many clinicians order blood cultures and consider administering antibiotics for well appearing neonates in whom the cause of the thrombocytopenia is not yet clearly defined.
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Differential diagnosis of Neonatal Early onset Thrombocytopenia
Clinical presentation
Degree of thrombocytopenia
Differential diagnosis
Ill-appearing Variable Sepsis ( bacterial, Viral)
TORCH infections (HSM)
Birth asphyxia (DIC)
Well appearing Mild - moderate Placental insufficiency
Genetic disorders ( cong. anomalies or dysmorphic features)
Autoimmune
Severe Neonatal alloimmune thrombocytopenia (NAIT)
Autoimmune.
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Differential Diagnosis of Neonatal Late-onset ThrombocytopeniaClinical presentation Differential diagnosis
Ill-appearing Sepsis ( bacterial, Viral, Fungal)
NEC
IEM
(Viral = HSV,CMV, enterovirus)
Well appearing Drug induced thrombocytopenia
Thrombosis
Fanconi anemia.
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Genetic Disorders Associated With Thrombocytopenia
Category Subtype Other clinical & labs
Chromosomal Trisomy13 Aplasia cutis , CHD, Cleft lip and palate , polydactly
Trisomy18 IUGR, CHD, rocker-bottom feet , overlapping digits, hypertelorism , small mouth , clinodactyly.
Trisomy21 CHD , transverse palmar crease , hypotonia , short neck with redundant posterior folds.
Turner S. CHD, Cutis vulgus ,webbed posterior neck, broad chest with wide spaced nipples , Lower extremity edema
11 q terminal disorder ( Jacobsen S, Paris Trousseau Thrombocytopenia)
CHD, genitourinary anomalies, abnormal brain imaging , Limb anomalies
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Category Subtype Other clinical & labs
familial May- Hegglin anomaly,Sebastian syndrome
Giant platelets , Neutrophilic inclusions
Fetcher syndrome Giant platelets , sensorineural hearing loss, cataracts, nephritis, neutrophilic inclusions.
Bernard- soulier Syndrome Giant platelets
X-linked macro thrombocytopenia due to GATA-1 mutation
Anemia, genitourinary abnormalities ( Cryptorchiadism)
Congenital amegakaryocytic thrombocytopenia
Abnormalities of head size and shape. Developmental delay ,CHD,cleft and high arched palate , Abnormal kidneys, optic atrophy, vulgus and varus deformities, Vertebral anomalies , Coloboma,Scoliosis,absent BM,megakaryocytes
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Category Subtype Other clinical & labs
familial Wiscott -Aldrich syndrome Immunodeficiency , small palates, eczema
Amegakaryocytic Thrombocytopenia and radioulnar synostosis
Restricted forearm pronation, proximal radioulnar synostosis in forearm, absent BM megakaryocytes
Fanconi anemia Hypopigmeted and hyperpigmented skin lesions. UT abnormalities , microcephaly , upper extermity radial side abnormalities involving the thumb , pancytopenia ( usually with onset in childhood)
Thrombocytopenia and absent radii
Shortened/absent radii bilaterally , normal thumbs, ulnar and hand abnormalities , abnormalities of the humerous.CH defects , Eosinophilia, leukemoid reaction
Neonatal primary hemophagocytic lymphohistiocytosis
Fever, HSM , Hyperferritemia , hypertriglyceridemia, hypofibrogenemia.
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Bernard Soulier ( Giant Platelets)
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Fanconi anemia
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Thrombocytopenia and absent radii
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Category Subtype Other clinical & labs
Metabolic Propionic acidemia , methylmalonic acidemia
FTT, developmental delay, Ketoacidosis , hyperglycinemia, hyperammonemia
Isovaleric acidemia Odor of sweaty feet, poor feeing, hypotonia , hyperammonemia, metabolic acidosis
Gausher disease HSM, Gausher cells in BM.
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Mechanism of nonimmune thrombocytopeniaNeonatal platelet production
1.Production of thrombopiotin (Tpo).2.Proliferation of megakaryocyte progenitors.3.Megakaryocyte maturation .4.Generation and release of new platelets.
• Tpo concentrations are higher in neonates than adults.
• Neonates who have thrombocytopenia have lower Tpo concentration than similarly affected adults.
• Neonatal megakaryocytes generate fewer platelets than adults predisposition of ill neonates to develop thrombocytopenia.
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Mechanism of nonimmune thrombocytopeniaMeasurement of neonatal platelet production
• BM biopsy is the gold standard test for evaluation of thrombocytopenia.
• It is difficult and frequently postponed until the infant is out of the neonatal period.
• Plasma or serum Tpo concentration, circulating megakaryocyte, proginators, reticulated platelet percentages RP% , and glycocalicin concentrations ( research setting).
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Mechanism of nonimmune thrombocytopenia• Immature platelet fraction (IPF) part of CBC ( similar to RP% and to retic count in the evaluation of anemia.)
• IPF is elevated in thrombocytopenia associated with increased platelet destruction (e.g. ITP) and decreased in thrombocytopenia due to decreased platelet production (e.g. aplastic anemia , chemotherapy induced thrombocytopenia).
• IPF can predict recovery of the platelet count within the next 24 hours.
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Mechanism of nonimmune thrombocytopenia• Mechanisms of thrombocytopenia in specific neonatal illness.
• The use of several tests together can differentiate between disorder of increased platelet destruction and deceased production and clarify the mechanisms of common varieties of neonatal thrombocytopenia.
• Platelet underproduction is the primary mechanism for the thrombocytopenia seen in fetuses and neonates
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Mechanism of nonimmune thrombocytopenia1- Chronic intrauterine hypoxia (decreased concentration of megakaryocyte proginators + decreased number of megakaryocytes in bone marrow + lower plasma Tpo concentration)Megakaryocyte proginators from premature neonates are more vulnerable to chronic hypoxia induced suppression than those of term neonate.
2- Sepsis induced thrombocytopenia ( elevated Tpo concentration + increase megakaryocyte proginators concentrations + increase RP%)Neonates with gram negative sepsis have more thrombocytopenia and more sever illness but less thrombopoietic response ( down regulation of thrombopoietic response during sever illness to relative hypoproliferation.
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Management of neonatal thrombocytopeniaNeonatal Alloimmne Thrombocytopenia
NAIT should be Considered in any neonate with initial PLT < 50.000/mcl (50 x10^9/L), especially in the absence of other risk factors or clinical symptoms.
Combination of sever neonatal thrombocytopenia with a parenchymal ( rather than interventricular) intracranial hemorrhage is highly suggestive of NAIT.
When NAIT is suspected , rapid blood testing is very important for timely and accurate diagnosis.
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Blood should be collected from the mother and the father and submitted for confirmatory testing.
Antigen screening should include human platelet antigen HPA 1,3 and 5.
HPA 9 and 15 ( HPA 4 if parents are Asian ) should be done if the diagnosis is strongly suspected and the initial evaluation results are negative .
If results are positive antibody in the mothers plasma directed against the specific platelet antigen in the father .
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The neonates serum can be screened for platelet antibodies if blood from the parents is not available ( may give false negative result).
Imaging of the brain ( US , CT or MRI should be performed as soon as possible when NAIT is suspected .
Large number of infants with NAIT respond to donor PLT transfusion ( first line of therapy)
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If the patient is stable + no evidence of ICH PLT transfusion if the PLT count is < 30,000.
If the patient has ICH maintain PLT count > 100,000.
IVIG can be infused to increase the patient own PLT and protect the transfused PLT.
Matched ( antigen negative ) PLT must be given if there is no increase in the PLT count to a save concentration within 1-2 days .
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Matched PLT include. 1- Maternal PLT. 2- Donor PLT with known HPA 1b1b and 5a5a ( compatible in > 90% of cases of NAIT).3- Random donor matched PLT ( after the results of typing).
Methylprednisolone might be given on the days that IVIG is used.
NAIT often resolved within 2 weeks . ( frequent PLT count until normal count without treatment).
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Management of neonatal thrombocytopeniaAutoimmune thrombocytopenia
Should be considered in any neonate with
1- Early onset moderate to sever thrombocytopenia .
2- Maternal history of ITP or autoimmune disease with or without thrombocytopenia.
In mothers with ITP history 25% of neonates had thrombocytopenia at birth .
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Management of neonatal thrombocytopeniaAutoimmune thrombocytopenia
All neonates born to mothers with AID undergo screening PLT count shortly after birth.
1- Normal PLT count no further evaluation.
2- Mild to moderate thrombocytopenia repeat in 2-3 days.
3- PLT < 30,000 IVIG ( first line of therapy).
evidence of active bleeding IVIG + random donor PLT.
Cranial imaging should be done to rule out IVH.
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Management of neonatal thrombocytopeniaNon immune thrombocytopenia1- Determine the cause .
2- Provide specific therapy and supportive care.
3- PTs with moderate to sever thrombocytopenia PLT transfusion ( at different thresholds depending on the clinical picture and the signs of hemorrhage) .
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Thank You