Transcript Morning Report

Morning Report
July 6, 2012
In the ER…
18 month old male with bruising and
a bloody nose
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
One liner
 18 mo M with no significant PMH presenting with sudden onset
bruising and petechiae
Work-up
“Lab is calling to report a
critical lab…”
Platelets 5
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
Thrombocytopenia
 Increased destruction
 Decreased production
Name that syndrome!
thrombocytopenia
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
Clinical manifestations
 Cutaneous bleeding
 Mucosal bleeding
 Absence of other symptoms
Guidelines
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
IVIG
 Prevention of
reticuloendothelial
uptake of
autoantibody-coated
platelets
IVIG
 Interaction of the
autoantibodies with
anti-idiotype
antibodies in the
IVIG
Giving IVIG
 Single dose, 0.8-1 g/kg
 Side effects
 Fever
 Nausea, vomiting
 Headache
 Aseptic meningitis
 Anaphylaxis
 Renal failure
 Live vaccines must wait
Rhogam
 Hgb >10, Rho(D) positive
 Side effects: fever, chills, hemolytic anemia
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-idiopathicthrombocytopenic-purpura-in-children?source=search_result&selectedTitle=3%7E150
 http://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-immune-idiopathicthrombocytopenic-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):521527.
 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