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Case Conference Presentation. Tania Kourtidou, MD PGY-1. 10/19/2011. Case Description. 14yo girl with c/o b/l lower leg rash x1d. Case Description. 14yo girl with c/o b/l lower leg rash x1d Red spots, nontender, non-pruritic Applied Hydrocortisone cream ->no change - PowerPoint PPT Presentation
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Case Conference Presentation
Tania Kourtidou, MDPGY-1
10/19/2011
Case Description
14yo girl with c/o b/l lower leg rash x1d
Case Description
14yo girl with c/o b/l lower leg rash x1d
• Red spots, nontender, non-pruritic
• Applied Hydrocortisone cream ->no change
• Afebrile, no URI, GI, GU, musculoskeletal, visual disturbance
• On the 4th day of menstrual cycle
• 1st episode, no Hx of trauma, insect bite, pets, recent travel,
change in soaps, -sick contacts
Case description
PMHx: Tested for Marfan Syndrome – Negative
FH: Asthma, DM, HTN, Seizure disorder
Immunization status: Unknown
SHx: Denied smoking, sexual activity, illicit drug use, suicidal thoughts, both parents at home, attended high school.
Case Description
VS: T 98.2F, HR 98, BP 108/72, RR 20, SpO2 100%
Physical exam:
Skin: Flat, hemorrhagic, non-blanching, pinpoint, non tender, located only in lower extremities, ant>post
Rest of PE: wnl
Petechial - Porpuric rash
Petechea <3mm Porpura: 3-10mm
Glass test
Petechial - Porpuric rash
Differential diagnosis
Meningococcal infection
LeukemiaMalignancy
Viral infections
*Enterovirus
HSP
ITP
Pressure VomitingTraumaD.I.C.
*Simple porpura indicates a qualitative or quantitative PLTs disorder.!
Case Description
Lab tests:
1. CBC, RET count, Peripheral smear
2. PT, aPTT, INR
3. ERS, CRP
4. CMP
5. Blood Cx
6. *UA could not be evaluated for blood
Case Description
Lab results:
6.2 11840.5
13.7
140
4.6 29
106
0.6
10
7.5
4.7
13
21
0.9 94
0.9
9.6
94
11.6 1.1
25.7
ESR: 2Peripheral smear: (no schistocytes, blasts)
CRP, Blood Cx: Negative
Case Description
THROMBOCYTOPENIA
Production Destruction
Immune
Non-immune
CongenitalAplastic anemia
Infections (HIV,HCV,H.pylori)
Paroxysmal nocturnal hemoglobinuria
Von Willebrand’s IIBDrugs
DICTTP/HUS
Kassabach-Merrit syndrome
Hypersplenism
ITPAlloimune (neonate)
InfectionsDrugs
SLE, Antiphospholipid syndrome
Fleisher GA, LudwigS, et al., eds. Textbook of pediatric emergency medicine. 3d ed. Baltimore: Williams & Wilkins, 1993:430–8.
Case Description
Based on:
clinical presentation
PLTs
Normal CBC and peripheral smear
Pt d/c with the diagnosis of ITP
No indication for treatment
Recommended to f/u with hematology clinic
Idiopathic Thrombopenic Porpura
Porpura ~> Πορφύρα (porphyra)
Colouring substance produced with the treatment of shell Haustellum brandaris .Gives indelible deep red colour.
It was particularly precious because of its difficult production and the rarity of shells.
Therefore the use of clothing dyed with pupura represented a sign of wealth and power. Emperor’s cloak in the Empire of the Byzantium was always colored with porpura.
Justinian I
Idiopathic Thrombopenic Porpura
Outline
1. Introduction
2. Pathogenesis
3. Diagnosis
4. Clinical manifestations
5. Therapeutic principals
6. Latest treatment options
Idiopathic Thrombopenic Porpura
Isolated persistent thrombocytopenia: PLTs<100x109/L
Normal CBC
Normal peripheral smear
Idiopathic Thrombopenic Porpura
The most common cause of thrombocytopenia in children
Prevalence: 4.0-5.3/100.000, 3500 new cases per year
85% Acute and self-limited
Between 2 and 10 years (peak age: 5y)
Equal gender/ethnic distribution
>10y girlsInsidious presentation
Chronic ITP >6m
Idiopathic Thrombopenic Porpura
The etiology is still unknown.
Idiopathic Thrombopenic Porpura
*1-4week after exposure to common viral infection or immunization (varicella, MMR).
Theories
Antibody cross-reactivity H. pylori
bacterial lipopolysaccharide
s
Pathogenesis
Idiopathic Thrombopenic Porpura
Antibodies against viruses may x-react to PLT antigensimmune complexes on the PLT surfaceremoval by reticuloendothelial system
Some strains of H. pylori may induce PLT aggregation
Bacterial products (ex.LPS) once adhered to PLTs, may induce increased phagocytosis or “clearance” of PLTs
Pathogenesis
Pathophysiology
*B cells produce IgG autoantibodies against GP IIb/IIIa and Ib/IX.
Idiopathic Thrombopenic Porpura
Clinical manifestations
• Sudden onset
• Healthy child
• Mucocutaneous bleeding:
epistaxis, gum bleeding, menorrhagia
GI or CNS <1%
• 50% Minimal splenomegaly
Idiopathic Thrombopenic Porpura
Diagnosis (of exclusion)!!!PLTs<100x109 /L
Hb
Normal aPTT and PT
Prolonged BT
Peripheral smear: Megathrombocytes
Idiopathic Thrombopenic Porpura
Diagnosis
Bone Marrow aspiration
Anti-PLT antibody studies
+ANA (adolescents)
EBV, CMV, Mycoplasma, H.pylori (cITP)
Normal or Increased number of megakaryocytes
Sensitivity and specificity
Idiopathic Thrombopenic Porpura
These laboratory tests are NOT recommended by the ASH practice guidelines to patients with the typical ITP presentation.
(American Society of Hematology, 2011)
Diagnosis of ITP should be based on :
1. Infection history
2. Clinical features
3. Physical exam
4. Lab tests: CBC and peripheral blood smear
Idiopathic Thrombopenic Porpura
Treatment
Most of the cases can be managed at home
“Most patients and their parents can live quite comfortably with petechiae and low platelets awaiting spontaneous
remission providing their physicians can.”
Dickerhoff 1994, Thrombocytopenia in childhood.
Idiopathic Thrombopenic Porpura
Treatment guidelines
If skin manifestations only Observation regardless of the PLTcount
Hospitalize if
Close monitor of CBC once a week
Once PLT begin to increase, it takes 2-3 weeks to normalize.
Severe, life threatening bleeding regardless of the PLT count
Idiopathic Thrombopenic Porpura
Treatment guidelines
Consider treatment if PLTs<30x109/L
The goal is to raise PLT count hemostatically safe, not to cure
1st line treatment:
Single dose of IVIG (0.80.8-1g/kg x 2d or 400mg/kg/d x 5d)
Short course of corticosteroids(Methylprednisolone 20-30mg/kg x3d or Prednisone 1-2.g/kg/d x14d)
or
Idiopathic Thrombopenic Porpura
Treatment guidelines
IVIG:
Blocks Fc receptor on phagocytes PLT destruction
Rapid elevation of PLT count >20.000 within 48h Preferred to corticosteroids in severe disease
Expensive, long effusion time (6-8h), allergic reactions, aseptic meningitis
Idiopathic Thrombopenic Porpura
Treatment guidelines
Corticosteroids:
Reduce capillary fragility, inhibit PLT destruction and antibody production
No evidence supporting long course vs. brief course
Cheap and convenient but side effects of long term use
Idiopathic Thrombopenic Porpura
Treatment guidelines
IV Anti-D therapy:
1st line for Rh+ with functional spleen
Induces mild hemolytic anemia RBC-antibody complexes saturate the macrophage Fc receptors Increased survival of antibody-coated PLTsslow rise of PLTs
Less allergic reactions than IVIG, no aseptic meningitis
Hemolysis Transient Hb
Idiopathic Thrombopenic Porpura
Treatment guidelines
Splenectomy:
Reserved for children >4 years of age with
persistent symptoms (bleeding) lasted longer than 1 year and lack of response to therapy and/or who have a need for improved quality of life.
Idiopathic Thrombopenic Porpura
Treatment guidelines
Transfused PLTs
*Rapidly removed from the circulation only used in emergencies to control bleeding (PLT<3x109/L)
No role in the routine management of ITP
Idiopathic Thrombopenic Porpura
Life threatening hemorrhage:
1.PLT infusions (10ml/kg expect to PLT by 50.000/L)
2. IV Methyprednisolone 30mg/kg (max 1g) over 20min, repeat daily up to x3
2.IVIG (1g/kg over 4-6h, repeat daily up to x5)
3.Emergent splenectomy
4.Plasmapheresis, RBCs transfusion, antifibrinolytics
Idiopathic Thrombopenic Porpura
Special Considerations
TPO Mimetics and Receptor Agonists(Romiplostim, Eltrompobag)
No published data to guide the use of these agents in children
High-Dose Dexamethasone
Rituximab
A-Interferon
Adolescents with significant ongoing bleeding and/or need for improved quality of life despite conventional treatment.
Altenative to splenectomy or in those who have failed splenectomy.
Immunosuppression(AZA,CTX,VCR)
Multiple agents have been reported; however insufficient data for specific recommendations.
Idiopathic Thrombopenic Porpura
Follow up:
Spontaneous recovery:
10-20% chronic ITP adolescent girls
monitor platelet count and clinical status daily to weekly, depending on the severity and treatment
Once platelet count has normalized, recurrence is rare and follow-up platelet counts are unnecessary
-60% by 3months-80% by 6months-90% by 1year
Idiopathic Thrombopenic Porpura
Parent education.
Avoidance of contact sports, wearing protective headgear, lining the crib with protective padding
MMR should be given regardless PMHx of ITP
Discontinue medications that suppress platelet production
There should be a low threshold for prompt evaluation of child that has sustained blunt trauma with ITP.
Idiopathic Thrombopenic Porpura
ITP is often an acute and self-limiting disease in children
Most of the times no treatment is required
Goal = prevention of complications
Therapy needs to be tailored to the individual patient
Parent and patient education is very important
References:
2011 Clinical Practice Guideline on the Evaluation and Management of Immune Thrombocytopenia (ITP), The American Society of Hematology
Idiopathic Thrombocytopenic Purpura in Children: Diagnosis and ManagementP. D. McClure Pediatrics 1975;55;68
Evaluating the Child with Purpura, Leung et al., Am Fam Physician 2001;64:419-28
Childhood Immune Thrombocytopenic Purpura: Diagnosis and Management, Blanchette V., Bolton-Maggs P., DM, Pediatr Clin N Am 55 (2008) 393–420
www.uptodate.com
www.aap.org
www.hematology.org
www.pdsa.org
A 9-year-old boy presents to your office with purple spots on his legs and mild swelling of his scrotum of 1 day's duration. He has had no vomiting, diarrhea, or constipation. He is afebrile, alert, and active. On palpation, he reports mild abdominal discomfort. He has no edema of the lower extremities or presacral area. His weight is 1 kg more than his weight at his health supervision visit 6 months ago.
Of the following, the MOST likely abnormal laboratory finding to expect for this boy is:
A.anemia
B. hypoalbuminemia
C. microscopic hematuria
D.prolonged partial thromboplastin time
E. thrombocytopenia
A. anemia
B. hypoalbuminemia
C. microscopic hematuria
D. prolonged partial thromboplastin time
E. thrombocytopenia