1 / 64

Pediatric Hematologic Case Study: Petechiae Presentation

This case study discusses a 6-year-old female presenting with petechiae on both forearms and lower legs, exploring differential diagnoses and treatment options for platelet disorders and anemia. The patient is evaluated through physical examinations, laboratory tests, and a detailed medical history.

laird
Download Presentation

Pediatric Hematologic Case Study: Petechiae Presentation

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. 1st Student Teleconference • Yeungnam Univ. – Kyushu Univ. • 26th March, 2012

  2. Case 1: Petechiae on both forearms & lower legs JeongHeo(M 6, YU medical college)

  3. Chief complaint • Petechiae on both forearms & lower legs

  4. Present illness • 6 years old female complaint petechiae on both arms & legs • Past medical history • Complete regular vaccination • No prior disease • Drug : Ibuprofen for 5 days • due to URI 2wks ago • No previous operation • No transfusion history • Atopic dermatitis

  5. Family history • Older brother (9 years old) has atopic dermatitis • Social history • N-S

  6. Review of system • General weakness(+) • Sweat(-) • Headache (-) • Gum bleeding (+) • Oynophagia (-) • Hemoptysis (-) • PND (-) • Anorexia (+) • Abd. discomfort (-) • Melena (-) • Stool color change (-) • Frequency (-) • Fatigue (+) • Wt. loss (-) • Dizziness (+) • Sorethroat (-) • Cough (-) • Cyanosis (-) • Palpitation (-) • Nausea (-) • Constipation (-) • Hematochezia (-) • Tenesmus (-) • Dysuria (-) • Fever (+) • Skin rash (-) • Tinnitus (-) • Neck pain (-) • Sputum (-) • DOE (-) • Chest pain (-) • Vomitting (-) • Diarrhea (-) • Bowel habit change (-) • Incontinence (-) • Polydipsia (-) • Chill (-) • Itching sensation (-) • Vertigo (-) • Dysphagia (-) • Dyspnea (-) • Orthopnea (-) • Chest discomfort (-) • Abd. pain (-) • Hematemesis (-) • Stool caliber change (-) • Nocturia (-) • Temperature tolerance (-)

  7. Physical examinations • General appearance • Relatively well being appearance • Alert mentality • Vital sign • BP 125/80 • PR 103/min • RR 20/min • BT 37°C • Weight 27kg (97%) • Height 123.5cm (97%) • Head&Eye&ENT • Icteric sclera • Pale Conjunctiva • No engorgemented neck vein • Gingival bleeding • Chest • Regular heart beat without murmur • Symmetric & clear breath sound with resonance

  8. Abdomen • Inspection • Not distended • Auscultation • Normoactive • Palpitation • Soft without tenderness • No splenomegaly & hepatomegaly • No palpable mass • Percursion • Resonance • Back & Extremity • No pitting edema • Petechiae (+)

  9. Problem list?? • Differential diagnosis???

  10. Platelet disorder • Platelet agenesis • Idiopathic thrombocytopenic purpura • Drug • Aspirin, clopidogrel • von-Willebrand disease

  11. Coagulation factor disorder • Hemophillia • Vitamin K deficiency • Liver disease • Anticoagulation drug

  12. Vascular disorder • Thrombotic thrombocytopenic purpura • Hemolytic uremic syndrome • Vasculitis • Systemic lupus erythematosus • Henoch-Schonleinpurpura

  13. Anemia • Malnutrition • Auto-immune Hemolytic anemia • Iron deficiency anemia • Aplastic anemia • Megaloblastic anemia • Leukemia • Bone marrow suppression

  14. Other impression?? • Physical abuse • Next step??

  15. CBC • Hb 6 g/dL/ WBC 5.5K / Plt 13,000 /uL • MCV 92.3 fL / MCH 33.5 pg/cell / RDW 16.2% • Reticulocyte 13.05%

  16. PB smear

  17. Total & Direct Bilirubin • 2.5 mg/dL / 0.4 mg/dL

  18. Coomb's Test • Direct (+) / Indirect (+)

  19. Iron study • Serum Iron 103 ug/dL / TIBC 310 ug/dLFerritin 20 ug/dL

  20. Serologic test • Antinuclear Ab (-) • Lupus anticoagulant (-) • Factor VIII Ab (-) • Factor IX Ab (-) • AntiplateletAb • Platelet-associated IgG (+) • Platelet glycoprotein Ab (+) • Anti-dsDNAAb (-) • Anti-SmAb (-)

  21. Urinalysis • No hematuria • No proteinuria • No pyuria • Urobilinogen (+) • Ketone (-)

  22. Coagulation screening test • BT 11min / PT 11 / aPTT 24 / PT INR 1.1

  23. Coagulation factor assay • Normal • vWF test • Normal

  24. Liver function test • AST 52 U/L / ALT 74 U/L / LDH 1283 U/L • GGT 13 U/L / TP 5.91 g/dL / Alb 3.73 g/dL

  25. Bone marrow examination

  26. Final diagnosis????

  27. Evan’s syndrome • Autoimmune disease • Idiopathic hemolytic anemia • Idiopathic thrombocytopenic purpura

  28. Treatment • IV immunoglobulin 1g/kg/d IV for 2days • Methylprednisolone 30mg/kg/d IV for 2-3days • Platelet transfusion

  29. If not response? • Splenectomy • Vaccines for Pneumococcus, H. influenzae, Meningococcus

  30. Splenectomy failure patients?? • Search for accessory spleen • Diagnosis and eradication of Helicobactor pylori infection • IV immunoglobulin • Anti-D • High-dose dexamethazone • Danazol • Vincristine • Vinblastine • Cyclophosphamide • Azathioprine • Rituximab • Cyclosporine A

  31. More information??

  32. Disease review : Approach to Bleeding disorder Yeungnam – Kyushu Teleconference Hyun Jung Chung, YUMC

  33. Components of hemostasis • Vascular endothelium • Platelet • Coaguation factor

  34. Vascular endothelium Antithrombotic Prothrombotic Von Willebrand factor Tissue factor Fibrinolysisinhibitors • Antiplatelet effects • Anticoagulant properties • Fibrinolytic properties

  35. Platelet • Adhesion • To ECM after vascular injury with vWF • Secretion • Release of calcium and ADP. • Calcium : coagulation cascade • ADP : platelet aggregation • Aggregation • Via glycoprotein IIb/ IIIa • Primary hemostatic plug. • Secondary plug • Platelet contraction + fibrin

  36. Coagulation factor (-) surface charge Tissue injury Most critical step

  37. Bleeding? Platelet and vessel wall Coagulation pathway ↑Prothrombin time(INR>1.2) Extrinsic pathway Vt. K deficiency WarfarinTx Liver disease ↑activated partial thromboplastin time(>22-38s) Intrinsic pathway Heparin therapy Hemophila A, B ↑Both Liver disease(advanced) DIC • ↑Bleeding time (>2-7min) • Henoch-Schonleinpurpura • Immune Thrombocytopenic Purpura • Von Willebrand’s disease • Multiple myeloma

  38. Platelet disorders • Thrombocytopenia = Reduced platelet number • Causes • Decreased production of platelets • vitamin B12 or folic acid deficiency • Decreased platelet survival • Immunologic or Nonimmunologic etiology • Sequestration : Hypersplenism • Dilutional • Massive transfusions

  39. Idiopathic (Immune) Thrombocytopenic Purpura • Thrombocytopenia • Absence of other blood cell abnormalities • No clinically apparent conditions causes thrombocytopenia • Incidence of 22 million/year in one study

  40. Clinical Manifestations • May be acute or insidious onset • MucocutaneousBleeding • petechiae, purpura, ecchymosis • epistaxis, gum bleeding • Menorrhagia • GI bleed, CNS bleed : rare

  41. Etiology of ITP • Often after infection >80% • Preceding URI history • Theories • Antibody cross-reactivity • Cross-react to PLT antigens • Secondary PLT clearance • H. pylori • Some strains, induce PLT aggregation • Bacterial lipopolysaccharides • LPS adherence to PLT • Secondary PLT clearance • Adults • Lesser clear than children • No prodrome. • Chronic, recurrent form

  42. Childhood (acute) ITP Extravascular destruction Adult (chronic) ITP

  43. Diagnosis • “Diagnosis of exclusion” • Rule out other causes: • PLT clumping : Pseudothrombocytopenia • Drug : Acetaminophen, Bactrim, Quinidine • Infections : HIV, Hepatitis C • Thyroid : hyper/hypo • Autoimmune disease : SLE • Destructive-consumptive : TTP-HUS • Bone marrow disease : Leukemia, MDS

  44. P/E : no splenomegaly, lymphadenopathy • Bleeding time prologation • PT, aPTT : Within normal limits • Peripheral blood smear • Normal RBC, WBC • Normal or slightly enlarged PLT • Bone marrow study • Ix : >60yo, refractory, suspicious other hematologic disorder (AA, leukemia, MDS) • Megakaryocyte : increased • Confirmatory study • Serum antiplatelet antibody assay • Antiplatelet glycoprotein antibodies • Confirmatory test is not necessary in typical cases!

  45. ITP : Relatively BENIGN DISEASEA PROSPECTIVE STUDY OF ITP IN 245 ADULTS • Only.. • 12% Bleeding • 12% Needed splenectomy • 1.6% died • 28% Asymptomatic • 18% needed no treatment • 63% remission(PLT>100K) • 87% at least partial remission(PLT>30K, Asx.)

  46. Management • Indications of treatment • Platelet < 20K & persistent bleeding • High risk of severe bleeding : wet purpura, retinal bleeding • Chronic bleeding >6mon • PLT< 20K or PLT < 50K + mucocutaneous bleeding • Management for children • 30-70% recover within 3 weeks • Single dose IVIg 0.8-1g/kg as effective as repeated • Vaccination schedule : delay 8-11 mon • Splenectomy • Chronic ITP (> 12 mo) • Refractory with life-threatening bleeding • Vaccination : encapsulated bacteria • Pneumococcal, H. influenzae and meningococcal vaccines

More Related