Transcript SENIOR CASE PRESENTATION

SENIOR CASE PRESENTATION
DEEPTHY DAMIEN
01/21/2010
Chief Complaint
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4 y/o with spontaneous bruising x 1 month
H/O Present Illness
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4 y/o previously healthy female brought in by mother
because multiple spontaneous bruising x 1 month.
Mostly upper and lower extremities involved. Seen at
another hospital and got referred to RCRMC
No fever /chills/weight loss/bone pain /joint pain/nose
bleed/head ache / blurry vision
Positive h/o URI almost 1 month ago
No similar problems in the past
Past Medical History
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No prior hospitalizations
Seen in the Ortho clinic for intoeing
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Surgical history : none
Medications: none
Allergies: none
Family History: none
Birth history: normal delivery ,no complications
Social history: Lives with parents and brother
Immunizations: up to date
ROS
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HEENT:No headache /congestion/epistaxis
Resp: No cough /wheeze
Cardiac: No chest pain /cyanosis/palpitation
Abdomen: No abd pain/diarrhea/constipation
GU:neg
Skin:pos for bruising
Neuro:No weakness/seizure
Physical Exam
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Vitals:T: 98.8 F,PR:90/min,RR:30/min,BP: 100/60,wt: 26.5 kg
Gen: active ,talking
Skin: ecchymosis R elbow, R knee, shin, L knee[3x4 cm], L ankle
[3x1cm],purpura multiple around knee.
ENT: normal
Neck: NAD
Heart: RRR, no murmur
Abd: soft,non-tender,no organomegaly, BS+
Extremities: no swelling/effusion, normal ROM
Neuro: no deficits
Labs
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CBC:6.7/12.9/34.6/7
P/S: decreased platelets. Normal morphology
of RBC,WBC
Diagnosis
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ITP/ IMMUNE(IDIOPATHIC) THROMBOCYTOPENIC PURPURA
Orders at Admission
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CBC with diff
CMP,Uric acid,LDH,ESR,PS
PT/INR/PTT
NO NSAIDS
NO STEROIDS
Monitor for bleeding
Restrict activities
DAY 2
Patient stable
 No new lesions/ bleeding
 LABS:
CBC:6.7/12.9/36.8/5
ESR:7
CRP: 0.3
PT:11.8/INR:1.1/PTT:26.6
CMP:142/3.5/104/27/11/0.5
ALP:170/AST:32/ALT:20/Bili:0.1
LDH:618
Uric acid:4.3
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Orders
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IV IG 1 g /Kg IV x1
DAY 3
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Patient stable
No new lesions
CBC:4.1/1.6/36.5/46
Discharged home with ,advised to f/u with
PCP in 1 wk,CBC prior to clinic visit
Take Home Message
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NO NSAIDS AS THEY INCREASE THE RISK OF
BLEEDING
DO NOT GIVE STEROIDS UNTILL OTHER
DIAGNOSIS ARE EXCLUDED,AS THE STEROIDS
CAN MASK EARLY LEUKEMIA.
If the ITP is not resolved in 4-6 wks ,they may need a
hematology referral/BM Bx
If BM BX comes neg it is ok to treat with steroids
Immune(idiopathic) Thrombocytopenic Purpura
In Children
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Thrombocytopenia (usually <20,000/microL) that is acquired and
generally benign
Unknown cause
Acute ITP is common in children
Also known as autoimmune thrombocytopenic purpura/
isoimmune thrombocytopenic purpura
Pathogenesis
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Auto antibodies (usually IgG) against platelet
membrane antigens ,such as glycoprotein
complex II b/III a. The antibody coated platelets
have a shortened half-life because of the
accelerated clearance by tissue macrophages
in the spleen and other portions of RES.The
net effect is a decrease in the platelet count.
In chronic ITP, T cell mediated cytotoxicity may
cause platelet destruction.
Epidemiology
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ITP is one of the most common causes of
symptomatic thrombocytopenia in children.
Incidence 3-8 /100,000 children/ year
Usual age 2-10 yrs ,peak 2-5yr
Slightly boys>girls
Clinical Presentation
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Sudden appearance of bruising and/or bleeding in an otherwise
healthy child.
History:
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in 60% , there is a history of prior infection. An increased risk of
ITP is associated with MMR immunization.
No systemic symptoms
Presence of systemic symptoms like fever ,anorexia,joint
pain,bone pain or weight loss usually points to other diagnosis
Drug induced(heparin,quinidine,sulfonamides) thrombocytopenia
is uncommon in kids
Physical Findings:
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Signs of cutaneous bleeding(dry purpura) like
petechiae,purpura and ecchymoses
Less often mucosal bleeding(wet purpura) assoaciated
with a platelet count <10,000/micro L
Conjuctival and retinal hemorrhages are uncommon.
No enlarged LN/spleen/liver
Serious hemorrhages requiring blood transfusion and
ICH are rare in children with ITP
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Disease course: 70% of children have the acute form of ITP, which is
defined by recovery (platelet count >150,000/ micro L) in 6 months of
presentation with or without treatment. Treatment do not affect the long
term outcome,but minimize the risk of significant bleeding
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Diagnosis:2 criteria
1.Isolated thrombocytopenia,with otherwise normal blood counts and
peripheral smear.
2.No clinically apparent associated conditions that may cause
thrombocytopenia
Exclude concurrent inf/autoimmune disorders/malignancy/drugs/genetic
bleeding disorders/ marrow failure
Most chldren:2-10 yrs ,with abrupt bruising in otherwise healthy child
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Labs:
CBC:
thrombocytopenia , usually the only abnormality detected,usually <20,000/microL
Peripheral smear:
no morphologic abnormalities in WBC/RBC.Platelet decreased in number,often large in
size.
Other studies :
Coags,coombs’ test,retic count,HIV,studies for collagen vascular/rheumatoid disorders.
Anti platelet ab testing-not routinely indicated in kids
Bone marrow exam:
increase number of megakaryocytes,may appear large and immature
Routinely performed in the past to r/o marrow failure or malignancy(acute
lymphoblastic leukemia).New guidelines –BM exam is unnecessary in the “typical”
case of childhood ITP
Indication for BM exam:
-Atypical presentation
-Persistent thrombocytopenia beyond 6 months
-subsequent clinical course that is not consistent
with ITP
- Before Steroid treatment in typical ITP is
recommended by some .(not by UpToDate)
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Differential Diagnosis
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Viral infection(IMN,Hepatitis,HIV- 1)
Drug exposure(Heparin, Quinidine, Sulfonamide)
Autoimmune Disorders(SLE)
Leukemia (ALL)
Acquired marrow failure syndrome (aplastic anemia)
Inherited thrombocytopenic
disorders(thrombocytopenia-absent radius syndrome,
Wiskott-Aldrich syndrome,mutation of MYH 9 gene)
Treatment
Initial medical management:
-Ideal management still unclear -observation
alone vs observation with pharmacologic
intervention
-restrict contact sports/physical activity
-avoid medications with antiplatelet or
anticoagulant activities
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Pharmacologic Intervention
Presence of severe life threatening bleeding,risk of significant bleeding
(going for procedures/count <10,000/microL and cutaneous bleeding),
concomitant / preexisting conditions(hemophilia) need intervention
 Corticosteroids:
Reduce Ab production,RES phagocytosis of antibody coated
platelets,improve vascular integrity, improve platelet production
Prednisone 1-2 mg/kg/day(max 60 /day) in 3 divided doses x 2-4 wks,
followed by a taper of 4mg / kg/day ,divided into 3 doses for 4 days
OR
Methylprednisolone( 30-50 mg /kg/day) for 3-7 days
Some cases may need repeat courses
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IV IG:
Mechanism of action is multi-factorial,inhibition of
antibody adsorption to platelets,prevention of RES
uptake of auto ab coated platelets, interaction of auto
abs with idiotype abs in IV IG.
Works better than steroids but higher cost
Dose:
400mg /kg/day x 5 days
OR
Single dose of 1g/kg
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Anti-Rho(D) immune globulin: has been shown to be effective
Platelet transfusion: used in case of life threatening
bleeding(ICH)
The therapy is targeted to increase count to >20,000.In risk of life
threatening bleeding , IV IG could be repeated or combined with
steroids
Monitoring: the patients getting pharmacologic intervention the
usual hospital stay is 2 days.In the ambulatory setting ,platelet
count monitored 1-2 times /wk ,interval can increase as the
platelet increase .monitoring is necessary until the count return to
>150,000/microL(50% in 1 mont,70% in 3 months)
Chronic ITP: persistent
thrombocytopenia(<150,000/microL) for > 6 months
20-30% will have chronic ITP
1/3 rd of the cases will have spontaneous remission in
months to years
In chronic ITP the platelet count ranges between
20,000-75,000/microL.usually do not require any
treatment
Management: decrease the risk for bleeding.
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Pharmacologic therapy is used when they have
significant bleeding/or need to go for procedures
CORTICOSTEROIDS-short course/pulse course
IV IG – 500 mg /kg/day x 2 days ,and may be repeated
if the symptoms recur
SPLENECTOMY- may be needed in patients needing
repeated /continuous pharmacologic intervention even
12 months after diagnosis
Rituximab,Danazol,Interferon,Cyclosporin,Cyclophosp
hamide,Romiplostim,Eltombopag,Azathioprine
Bibliography
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Up To Date, Treatment and prognosis of ITP in
children, clinical manifestation and diagnosis of
ITP in children, Evaluating purpura in children
AAFP, August 1 ,2001, Evaluating the Child
with Purpura.