CASE DISCUSSION

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CASE DISCUSSION

  1. 1. CASE DISCUSSION 醫學七 楊琢琪
  2. 2. BASIC DATA • Mrs. 干 • Age: 33 • Gender: female • Room: 4B1 03 • Chart No.: 5167098 • Chief Problem: Dyspnea for 1.5 months
  3. 3. • Mrs. 干 , a 33 y/o woman, denied any systemic disease • G3P3, all healthy • Dyspnea since GA 28 wks • Dyspnea aggravated after C/S • Palpitation was also noticed • 04/26, to Cathay General Hospital BRIEF HISTORY
  4. 4. • 04/26 at Cathay General Hospital: • RBC 3.6 M, PLT 48 K, AST/ALT 188/199 • Fragmented RBC in peripheral blood • LDH 1230, FDP-DD 749 • CK/CK-MB 686/18.1 • U/A: gross hematuria, proteinuria 3+ bilirubin 3+, WBC >120 • Autoimmune study: no obvious positive finding BRIEF HISTORY
  5. 5. BRIEF HISTORY • At Cathay General Hospital: • HELLP syndrome or pulmonary hypertension was suspected. • Given with O2, Viagra, Herbesser, and then the condition improved. • Deterioration again on 2007/05/08, persistence of low platelet • Delirium once on 2007/05/18 • Transfer to our ICU on 2007/05/29
  6. 6. • In our ICU: • Initial presentation: drowsy consciousness • Vital sign: 36.8/114/20, BP: 132/85 • Non-rebreathing mask, FiO2 80%, SpO2 97% • PE & NE: Within normal limits • No arthralgia, no mucosal ulcer, no malar rash • No purpura, no ecchymosis, no hypertension • No gangrenes on extremities, no fever • No edema on lower legs BRIEF HISTORY
  7. 7. BRIEF HISTORY • CXR (2007/05/29): • Cardiomegaly and bulging pulmonary conus , increased bilateral lung marking and engorged pulmonary arteries. • Cardiac echo (2007/04/26): • Dilated RA & RV, LVEF 71%, moderate to severe TR, pericardial effusion, pulmonary hypertension.
  8. 8. • Chest CT (2007/04/26): • Dilated RA & RV with RVH, pulmonary trunk larger than aortic root, pleural effusion, small LAP (< 1 cm) at bilateral axillary region. • Lung perfusion scan (2007/06/01): • Little possibility of pulmonary emboli • Nuclear medicine study (2007/06/05): • LVEF 58% BRIEF HISTORY
  9. 9. BRIEF HISTORY • Lab data: • RBC 2.5 M, Hb 8.7, WBC 7.2 K, PLT 15 K • Reticulocyte: 7.96 % • AST/ALT: 25/29, BUN/CRE: 29.2/0.7 • BIL (T/D): 1.95/0.7 • Iron 54, TIBC 296, Ferritin 112 • DIC profile: 3P 1+, FDP 5.3, D-Dimer 1.16 • LDH 1476, BNP 1154, CRP 0.48
  10. 10. BRIEF HISTORY • Autoimmune study: (06/04) • Anti-Jo1, anti RNP, anti SS-A, anti SS-B: (-) • Anti scl-70, anti SM: (-) • ANA 1:40, centromere type • C3 64.5, C4 5.9, IgM & IgG↓(slightly) • Coombs' Test: Direct, positive, 1+, weak • RA factor: <20 • ADAMTS-13: normal • Anti-cardiolipin antibody: pending
  11. 11. BRIEF HISTORY • After consultation with hematologists and rheumatologists: • Thrombotic thrombocytopenic purpura (TTP) was suspected • Pulmonary hypertension, unknown cause • Should rule out autoimmune hemolytic anemia (AIHA), which may be secondary to SLE or Sjögren's syndrome . • Should also rule out antiphospholipid syndrome • No obvious evidence of SLE
  12. 12. CURRENT MANAGEMENT • Plasma exchange, FFP 27 U QD • Flolan (epoprostenol) 500meq QD • Viagra (sildenafil citrate) 0.25# BID
  13. 13. FOLLOW-UP DATA LDH PLT RBC Hb 05/29 1476 17 2.49 8.7 05/31 1311 15 2.5 - 06/01 - 14 2.68 9.3 06/03 - 7 2.39 - 06/04 - 3 2.24 8 06/05 - 9 2.2 8.1 06/06 1244 10 2.03 7.6 06/08 - 6.9 1.85 6.9 06/09 - 8.1 2.27 8.1 06/10 1008 13 2.19 7.7 06/11 935 16 2.72 9.2
  14. 14. SUMMARY • This is a 33 y/o female with progressively severe dyspnea after giving birth to her third child. It was accompanied by thrombo- cytopenia, anemia, gross hematuria, elevated LDH, pulmonary hypertension, and right heart failure. Her immunological studies and DIC profiles were of little contribution. Under the impression of TTP, plasma exchange was performed for 13 days. However, the benefit of this management is limited.
  15. 15. THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP)
  16. 16. INTRODUCTION • TTP syndrome: characterized by microangiopathic hemolytic anemia (MAHA) and platelet aggregation. • Platelet microthrombi form in the microcirculation throughout the body causing partial occlusion of vessels. • Organ ischemia, thrombocytopenia, and erythrocyte fragmentation (ie, schistocytes) occur.
  17. 17. INTRODUCTION • The thrombi partially occlude the vascular lumina with overlying proliferative endothelial cells. • The endothelia of the kidneys, brain, heart, pancreas, spleen, and adrenal glands are particularly vulnerable to TTP. • The liver, lungs, gastrointestinal tract, gallbladder, skeletal muscles, retina, pituitary gland, ovaries, uterus, and testes are also affected to a lesser extent. • Deep vein thrombosis can also occur. • No inflammatory changes occur.
  18. 18. INTRODUCTION • Prevalence: > 3.7/1,000,000 (US) • Age of onset: 35 (median age), 40~49 (in average) • Sex: female-to-male ratio of 3:2 • Mortality/Morbidity: • Untreated: mortality rate is approximately 95% • Early diagnosis: survival rate is 80-90%
  19. 19. ETIOLOGY OF TTP • Primary/idiopathic TTP: ~ 80% • Decreased activity or amount of ADAMTS-13 (80%) • Persistence of ULVWF and abnormal platelet aggregation and microthrombi formation • Genetic or acquired (existence of antibody)
  20. 20. ETIOLOGY OF TTP • Secondary TTP: ~ 20% • Drugs: anti-platelet drug, eg. clopidogrel (Plavix) • Pregnancy: TTP can develop at any time during pregnancy even after the birth. • Infections: eg. HIV • Systemic lupus erythematosus (SLE) • Malignancy: cancer of any type may be complicated by TTP
  21. 21. CLINICAL PRESENTATION • Fever (60%) • Purpura: Nonpalpable small purpuric spots or petechiae occur with thrombocytopenia (platelet count < 50 K/μL). • Anemia, hemoglobin levels less than 10 g/dL • Altered mental status (36%) • Abdominal pain (24%) • Renal changes (88%) with gross hematuria (15%) • Heart failure, arrhythmias
  22. 22. LAB STUDIES • Thrombocytopenia and anemia • Fragmented RBCs (schistocytes) • LDH level > 1000 mg/dL • Indirect bilirubin level: Elevated • Reticulocyte count: Elevated • PT/aPTT: Normal • DIC panel (fibrinogen, D-dimer): usually normal • Urinalysis: Proteinuria and microscopic hematuria
  23. 23. TREAMENT FOR TTP • Plasma exchange with FFP: standard method • Steroid: of no proven additional benefit • Antiplatelet agents: controversial • Splenectomy is performed occasionally to treat patients who do not respond to plasma exchange. Some patients benefit from splenectomy. The response may be due to the removal of the site of sequestration of the RBCs and platelets. Another possibility is that the spleen is a major site of microvascular occlusive lesions in severe TTP.
  24. 24. TREAMENT FOR TTP • Vincristine, a second-line therapy with an un- known mechanism of action and with unproven benefit • Supportive care, anticonvulsants, platelet-depleted packed RBCs • Platelet transfusion is contraindicated because it is associated with rapid deterioration!
  25. 25. CONCLUSION • Thrombotic thrombocytopenic purpura is a rare condition associated with abnormal aggregation of platelets, intravascular destruction of RBCs, and the formation of microthrombi. The patient can present with some or all of the characteristics of the classic pentad including thrombocytopenia, fever, renal changes with gross hematuria, neurologic deficit, and hematologic changes. Some patients may exhibit dysfunction of ADAMTS-13. If treated early with plasma exchange, the prognosis is good.
  26. 26. DISCUSSION
  27. 27. REVIEW OF THIS CASE S/S of Typical TTP S/S of Our Patient Fever - Purpura - Altered mental status Delirium Hematuria Gross hematuria ? Dyspnea Abdominal pain - Anemia Anemia ? Pulmonary hypertension Heart failure BNP > 900 Arrhythmia ?
  28. 28. REVIEW OF THIS CASE Lab Data of Typical TTP Lab Data of Our Patient Anemia w/ Hb < 10 Anemia w/ Hb < 10 Thrombocytopenia PLT < 15 K; 3 K (lowest) Elevated LDH LDH > 1000 Schistocytes in PB Fragmented RBC Elevated indirect bilirubin - Elevated reticulocyte count Reticulocyte: 7.96% PT/aPTT normal PT/aPTT normal DIC panel usually DIC profile nearly normal ? Decreased C3, C4 level Proteinuria Urine protein 3+ (‘07/04/25)
  29. 29. DISCUSSION • Poor response to plasma exchange. Poor responder? Not yet responsive? Wrong diagnosis? • Obvious pulmonary hypertension while lung perfusion scan relatively normal. Pitfalls of perfusion scan? Causes of pulmonary hypertension? • If TTP, the relationship between TTP and pulmonary hypertension? Single etiology, or two combined disease?
  30. 30. TAKE HOME MESSAGE • Thrombotic thrombocytopenic purpura (TTP) • Platelet over aggregation • Platelet↓, hemolytic anemia, LDH elevation • Diffused microthrombi formation and occlusion of vessels • Multiple organ involvement, eg. kidneys, brain, heart, liver, lungs, gastrointestinal tract, etc. • Standard treatment: plasma exchange
  31. 31. THANK YOU VERY MUCH FOR YOUR ATTENTION! HAVE A GOOD DAY!

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