Case Conference Presentation

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Case Conference Presentation

• Tania Kourtidou, MD
• PGY-1

10/19/2011
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Case Description

• 14yo girl with c/o b/l lower leg rash
  x1d

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Case Description

• 14yo girl with c/o b/l lower leg rash x1d
• Red spots, nontender, non-pruritic
• Applied Hydrocortisone cream -gtno 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

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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.

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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, antgtpost
• Rest of PE wnl

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Petechial - Porpuric rash
Petechea lt3mm
Porpura 3-10mm
Glass test
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Petechial - Porpuric rashDifferential diagnosis
Viral infections Enterovirus
Meningococcal infection
Leukemia Malignancy
HSP
ITP
Pressure Vomiting Trauma
D.I.C.
Simple porpura indicates a qualitative or
quantitative PLTs disorder.!
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Case Description

• Lab tests
• CBC, RET count, Peripheral smear
• PT, aPTT, INR
• ERS, CRP
• CMP
• Blood Cx
• UA could not be evaluated for blood

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Case Description

• Lab results

9.6
40.5
140
106
10
6.2
118
4.6
13.7
29
0.6
94
7.5
13
0.9
94
4.7
21
0.9
11.6
1.1
25.7
ESR 2 Peripheral smear (no schistocytes,
blasts)
CRP, Blood Cx Negative
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Case Description
THROMBOCYTOPENIA
Destruction
Production
Congenital Aplastic anemia Infections
(HIV,HCV,H.pylori) Paroxysmal nocturnal
hemoglobinuria Von Willebrands IIB Drugs
Immune
Non-immune
ITP Alloimune (neonate) Infections Drugs SLE,
Antiphospholipid syndrome
DIC TTP/HUS Kassabach-Merrit syndrome Hypersplenis
m
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Fleisher GA, LudwigS, et al., eds. Textbook of
pediatric emergency medicine. 3d ed. Baltimore
Williams Wilkins, 19934308.
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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

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Idiopathic Thrombopenic Porpura

• Porpura gt

???f??a (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. Emperors cloak in
the Empire of the Byzantium was always colored
with porpura. Justinian I
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Idiopathic Thrombopenic Porpura

• Outline
• Introduction
• Pathogenesis
• Diagnosis
• Clinical manifestations
• Therapeutic principals
• Latest treatment options

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Idiopathic Thrombopenic Porpura

• Isolated persistent thrombocytopenia
  PLTslt100x109/L
• Normal CBC
• Normal peripheral smear

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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

gt10y girls Insidious presentation
Chronic ITP gt6m
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Idiopathic Thrombopenic Porpura
The etiology is still unknown.
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Idiopathic Thrombopenic Porpura
Pathogenesis

• 1-4week after exposure to common viral
  infection or immunization (varicella, MMR).

Theories
Antibody cross-reactivity
bacterial lipopolysaccharides
H. pylori
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Idiopathic Thrombopenic Porpura
Pathogenesis

• Antibodies against viruses may x-react to PLT
  antigens?immune complexes on the PLT
  surface?removal 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

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Pathophysiology
B cells produce IgG autoantibodies against GP
IIb/IIIa and Ib/IX.
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Idiopathic Thrombopenic Porpura

• Clinical manifestations
• Sudden onset
• Healthy child
• Mucocutaneous bleeding
• epistaxis, gum bleeding, menorrhagia
• GI or CNS lt1
• 50 Minimal splenomegaly

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Idiopathic Thrombopenic Porpura
Diagnosis (of exclusion)!!!

• PLTslt100x109 /L
• Hb
• Normal aPTT and PT
• Prolonged BT

Peripheral smear Megathrombocytes
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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
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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
• Infection history
• Clinical features
• Physical exam
• Lab tests CBC and peripheral blood smear

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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.

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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
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Idiopathic Thrombopenic Porpura

• Treatment guidelines
• Consider treatment if PLTslt30x109/L
• The goal is to raise PLT count? hemostatically
  safe, not to cure
• 1st line treatment

Single dose of IVIG (0.8-1g/kg x 2d or
400mg/kg/d x 5d)
or
Short course of corticosteroids (Methylprednisolon
e 20-30mg/kg x3d or Prednisone 1-2.g/kg/d x14d)
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Idiopathic Thrombopenic Porpura

• Treatment guidelines
• IVIG
• Blocks Fc receptor on phagocytes ? PLT
  destruction
• Rapid elevation of PLT count gt20.000 within 48h
  ? Preferred to corticosteroids in severe disease
• Expensive, long effusion time (6-8h), allergic
  reactions, aseptic meningitis

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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

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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 PLTs?slow
  rise of PLTs
• Less allergic reactions than IVIG, no aseptic
  meningitis
• Hemolysis? Transient ? Hb

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Idiopathic Thrombopenic Porpura

• Treatment guidelines
• Splenectomy
• Reserved for children gt4 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.

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Idiopathic Thrombopenic Porpura

• Treatment guidelines
• Transfused PLTs
• Rapidly removed from the circulation ? only used
  in emergencies to control bleeding (PLTlt3x109/L)

No role in the routine management of ITP
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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

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Idiopathic Thrombopenic Porpura
Special Considerations 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.
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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
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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.

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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

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• 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
  19755568
• Evaluating the Child with Purpura, Leung et al.,
  Am Fam Physician 200164419-28
• Childhood Immune Thrombocytopenic Purpura
  Diagnosis and Management, Blanchette V.,
  Bolton-Maggs P., DM, Pediatr Clin N Am 55 (2008)
  393420
• www.uptodate.com
• www.aap.org
• www.hematology.org
• www.pdsa.org

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• 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.

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• Of the following, the MOST likely abnormal
  laboratory finding to expect for this boy is
• anemia
• hypoalbuminemia
• microscopic hematuria
• prolonged partial thromboplastin time
• thrombocytopenia

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• anemia
• hypoalbuminemia
• microscopic hematuria
• prolonged partial thromboplastin time
• thrombocytopenia

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