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Case Conference Presentation

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

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Case Conference Presentation

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  1. Case Conference Presentation Tania Kourtidou, MD PGY-1 10/19/2011

  2. Case Description 14yo girl with c/o b/l lower leg rash x1d

  3. 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

  4. 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.

  5. 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

  6. Petechial - Porpuric rash Petechea <3mm Porpura: 3-10mm Glass test

  7. 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.!

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

  9. Case Description Lab results: 9.6 40.5 140 106 10 6.2 118 4.6 0.6 13.7 29 94 7.5 13 0.9 94 11.6 1.1 4.7 21 0.9 25.7 ESR: 2 Peripheral smear: (no schistocytes, blasts) CRP, Blood Cx: Negative

  10. Case Description THROMBOCYTOPENIA Destruction Production Congenital Aplastic anemia Infections (HIV,HCV,H.pylori) Paroxysmal nocturnal hemoglobinuria Von Willebrand’s IIB Drugs Immune Non-immune ITP Alloimune (neonate) Infections Drugs SLE, Antiphospholipid syndrome DIC TTP/HUS Kassabach-Merrit syndrome Hypersplenism

  11. Fleisher GA, LudwigS, et al., eds. Textbook of pediatric emergency medicine. 3d ed. Baltimore: Williams & Wilkins, 1993:430–8.

  12. 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

  13. 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

  14. Idiopathic Thrombopenic Porpura Outline • Introduction • Pathogenesis • Diagnosis • Clinical manifestations • Therapeutic principals • Latest treatment options

  15. Idiopathic Thrombopenic Porpura Isolated persistent thrombocytopenia: PLTs<100x109/L • Normal CBC • Normal peripheral smear

  16. 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 girls Insidious presentation Chronic ITP >6m

  17. Idiopathic Thrombopenic Porpura The etiology is still unknown.

  18. Idiopathic Thrombopenic Porpura Pathogenesis *1-4week after exposure to common viral infection or immunization (varicella, MMR). Theories Antibody cross-reactivity bacterial lipopolysaccharides H. pylori

  19. 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

  20. Pathophysiology *B cells produce IgGautoantibodies against GP IIb/IIIa and Ib/IX.

  21. Idiopathic Thrombopenic Porpura Clinical manifestations • Sudden onset • Healthy child • Mucocutaneous bleeding: epistaxis, gum bleeding, menorrhagia GI or CNS <1% • 50% Minimal splenomegaly

  22. Idiopathic Thrombopenic Porpura Diagnosis (of exclusion)!!! • PLTs<100x109 /L • Hb • Normal aPTT and PT • Prolonged BT Peripheral smear: Megathrombocytes

  23. 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

  24. 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

  25. 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.

  26. 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

  27. 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 (0.8-1g/kg x 2d or 400mg/kg/d x 5d) or Short course of corticosteroids (Methylprednisolone 20-30mg/kg x3d or Prednisone 1-2.g/kg/d x14d)

  28. 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

  29. 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

  30. 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

  31. 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.

  32. 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

  33. 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

  34. Idiopathic Thrombopenic Porpura

  35. 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

  36. 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.

  37. 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

  38. 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

  39. 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.

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

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

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