morning report july 6, 2012. in the er… 18 month old male with bruising and a bloody nose
Post on 17-Dec-2015
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History
ROS: Fell off chair 3 days ago, now mother noticing increasing size of bruise with no other known trauma, +bruising, +epistaxis, +small spots on entire body when woke up this morning URI ~1 week ago now resolved No fevers, no night sweats, no weight loss, no n/v/d, normal
appetite and activity level, no gross hematuria
PMHx: Full term, no complications. Not circumcised. Immunizations UTD – received 12 mo vaccines ~6 months. No medications. No history of eczema.
Social Hx: LAHW mother, father, older sister, 1 cat.
Family Hx: No bleeding disorders
Physical Exam Temp 37.0 HR 115 RR 18 O2 sat 99% on RA BP 87/46 Gen: Well-appearing, well nourished, no distress. Running around
the room HEENT: Anicteric sclera. Scattered bruises throughout the scalp. No
rhinorrhea or congestion. Dried blood in right nare. MMM. Petechiae on OP and palate. Blood blister on upper lip mucosa. Scattered petechiae around mouth
CV: RRR, no murmurs, 2+ pulses throughout Resp: CTAB/L, no wheezing, no rhonchi Abd: Soft, NT, normoactive BS. No distension, no masses. No HSM. MSK: Normal ROM LAD: Shotty anterior and posterior cervical adenopathy, all <1 cm Neuro: No focal deficits Skin: Petechiae, purpura throughout extremities, torso and abdomen.
No jaundice or pallor
Platelets
Live 7-10 days
Thrombocytopenia <150K No symptoms >100K Minimal symptoms 50-100K Mild (cutaneous) symptoms 20-50K Moderate (cutaneous + mucosal)
symptoms 5-20K Severe symptoms (mucosal + CNS)
<5K
Kasabach-Merritt syndrome
Sequestration of platelets and coagulation activation in large vascular malformations
Fanconi anemia
Fanconi anemia ≠ Fanconi syndrome!
Autosomal recessive
Hypopigmented macules, café-au-lait macules
Abnormalities of thumbs
Microcephaly
Urogenital abnormalities
Short stature
Wiskott-Aldrich syndrome
X-linked recessive
Abnormal gene on proximal arm of X chromosome
Atopic dermatitis
Thrombocytopenic purpura
Increased susceptibility to infections
Thrombocytopenia-absent radii syndrome (TAR)
?defect of megakaryocyte Normal erythroid and myeloid
maturation
Bilateral absent radii
Normal thumbs
Skeletal, GU, heart anomalies
Bernard-Soulier syndrome
Autosomal recessive
Dysfunction/absence of platelet receptor for von Willebrand factor
Prolonged bleeding time
Easy bruising, severe hemorrhage with trauma/surgery
Increased destruction
Disseminated intravascular coagulation (DIC)
Hemolytic-uremic syndrome (HUS)
Thrombotic thrombocytopenic purpura (TTP)
Kasabach-Merritt syndrome
Immune thrombocytopenic purpura (ITP)
Drug induced
Mechanical platelet destruction
Platelet sequestration
Decreased production
Bone marrow failure or infiltrate Acquired aplastic anemia
Leukemia, infectious granuloma
Fanconi anemia
Infection
Nutritional
Thrombocytopenia and absent radii (TAR)
Wiskott-Aldrich syndrome
Bernard-Soulier syndrome
Cyanotic congenital heart disease
Back to our patient…
CBC 9.9>12.6/37.1<5, Smear: no schistocytes, +megakaryocytes
Type and Screen O+, Antibody negative, Coombs negative
ANA: pending
Autoplatelet antibody: pending
PT 12.6 PTT 27.5 INR 1.0
BMP normal
LDH normal
U/A 5-10 RBC, no WBC, no casts, no bacteria
CRP <3
Immune Thrombocytopenic Purpura
Isolated thrombocytopenia
Immune mediated destruction of normal platelets
Epidemiology
½ of cases occur in pediatric patients 2-10 year olds, peak 2-5 year olds
Most common cause of isolated thrombocytopenia in otherwise well children
60% of cases occur within 1 month of an infection
Seen following MMR vaccine
Diagnosis
History, physical, CBC, smear
Consider bone marrow biopsy if: Fever Bone/joint pain +family history HIV risk factors Skeletal or soft tissue morphologic abnormalities non-petechial rash Lymphadenopathy Abnormal Hgb, WBC, white cell morphology
To Treat or Not to Treat
Children with no or mild bleeding (skin manifestations only) be managed with observation* alone regardless of platelet count
Essential components: anticipatory guidance, follow-up, reliable parents
All patients: restrict activity (no contact sports), avoid medications with antiplatelet or anticoagulant activity
*can be done as an outpatient
Treat…
Any child with significant bleeding (mucosal bleeding) regardless of platelet count
Children with platelet count < 10K and cutaneous bleeding
Choices: IVIG Anti-D immunoglobulin Steroids
IVIG
IVIG can be used if a more rapid increase in the platelet count is desired
Meta-analysis comparing IVIG to steroids Primary outcome: platelets > 25K Steroids 26% less likely to achieve outcome
Giving IVIG
Single dose, 0.8-1 g/kg
Side effects Fever Nausea, vomiting Headache Aseptic meningitis Anaphylaxis Renal failure
Live vaccines must wait
Steroids
Reduce antibody production
Reduce reticuloendothelial system phagocytosis of antibody-coated platelets
Improve vascular integrity
Improve platelet production
Prognosis
20% go on to have chronic ITP (> 6-12 months)
Intracranial hemorrhage is rare – incidence of 0.1-0.5%
Compare
5 yo M with ALL s/p chemotherapy with a platelet count of 5K
5 yo M with ITP with a platelet count of 5K
PREP 2007
In examining a 4-year-old girl who is new to your practice, you discover that she has rudimentary thumbs and is well below the 5th percentile for both weight and height. You also observe irregular hyperpigmentation on the trunk and anogenital areas.
Of the following, the MOST likely hematologic disorder associated with these findings is:
A. Acute lymphoblastic leukemia
B. Bloom syndrome
C. Diamond-Blackfan anemia
D. Fanconi anemia
E. Thrombocytopenia and absent radii (TAR) syndrome
PREP 2005
An otherwise well 4-year-old boy is brought to your office because his mother has noticed bruising over the past 2 weeks. On physical examination he appears well and has no hepatosplenomegaly or adenopathy. There are scattered petechiae on the right upper arm, resolving bruises on the legs, and no evidence of new bruises. A complete blood count shows a platelet count of 50 x 103/mcL. The white blood cell and differential counts, hemoglobin, and hematocrit are normal
Of the following, the BEST next step in the management of this patient is to recommend:
A. administration of intravenous immunoglobulin therapy
B. administration of oral corticosteroid therapy
C. avoidance of sulfonamides
D. hospitalization for observation
E. performance of a bone marrow examination
Goals and Objectives
Review the differential diagnosis for thrombocytopenia and syndromes associated with thrombocytopenia
Review the pathophysiology of ITP
Review the current approach to diagnosis and management of ITP
References
http://www.uptodate.com/contents/treatment-and-prognosis-of-immune-idiopathic-thrombocytopenic-purpura-in-children?source=search_result&selectedTitle=3%7E150
http://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-immune-idiopathic-thrombocytopenic-purpura-in-children?source=search_result&selectedTitle=4%7E150
Beck CE, Nathan PC, Parkin PC, Blanchette VS, Macarthur C. Corticosteroids versus intravenous immune globulin for the treatment of acute immune thrombocytopenic purpura in c hildren: a systematic review and meta-analysis of randomized-controlled trials. J Pediatr. 2005; 147 (4):521-527.
Buchanan GR. Thrombocytopenia during childhood: what the pediatrician needs to know. Peds in Review. Nov 2005; 401-409.
Consolini DM. Thrombocytopenia in infants and childrewn. Peds in Review. April 2011; 135-151.
Nuenert C, Lim C. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood (2011) 117: 4190-4207
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