張貼日期:2018/1/8 上午 11:04:04
回覆模板:
A 70 y/o female was admitted due to
Chronic/recent onset/acute thrombocytopenia was found, and we are consulted for further evaluation.
[Major past history]
[PE]
E4V5M6
Mouth: several oozing blood clot at oral mucosa
Abdomen: splenomegaly(-)
Skin: petechiae over lower limbs
[Platelet and Hb course]
Hb: ____ (date) -> ____ (date) -> ____ (date) -> ____ (date)
Plt: ____ (date)-> ____ (date) -> ____ (date) -> ____ (date) . Transfusion refractoriness (+/-)
[PB smear]: schistocyte(-), giant platelet(+), platelet clumping(-), spherocytes(+)
[DIC profiles]
Fibrinogen/PT/aPTT/D-dimer:
LDH: ____ (date)
Bil-T: ____ (date)
[Infectious etiology]
HIV/HCV/H. pylori: (+)/(?)/(?)
[Medications history]
Tazocin: (Date starts) - (Date ends)
[Evidences of increased blood cell destruction]
05/02 Bil-T/-D (2.12/1.24)
05/03 LDH (305)
[PB smear]: not done
24-Corrected count increment(CCI): likely < 10000
[Etiologies of increased blood cell destruction]
DIC/MAHA
05/02 D-dimer (0.63), PT/aPTT (34.5/48.4)
05/04 Fibrinogen (527.7)
Infection
05/04 Fever(+)
05/04 Procalcitonin (1.35)
05/04 CRP (5.10)
HIV(unknown)
Autoimmune
05/02 Anti-ENA (0.09, negative)
Thyroid
05/04 TSH/free T4(0.14/1.07)
Cardiovascular destruction
05/05 UCG(Mild-to-moderate tricuspid regurgitation)
AV-shunts (not identified)
[Evidences of impaired BM production]
Reticulocyte production index: not checked
[Etiologies of impaired BM production]
Probable BM suppression agents: (+)
4/19- 4/27 Sevatrim
4/14-4/28 Brosym)
Nutrition status
B12/folic acid: not checked
BM disease evidence
05/02 SEPE/IFE (no monoclonal protein)
[Platelet sequestration]
Splenomegaly(not detected by 04/22 CT)
[Other special conditions]
hypothermia, hemodilution, cardiopulmonary bypass: nil
If MAHA + thrombocytopenia is present, following below flow chart
Probable mechanism of thrombocytopenia
Artifact
Decreased production
Increased destruction
Platelet sequestration
Special condition: hypothermia, hemodilution, cardiopulmonary bypass
If ITP is suspected (commonly 1 hour CCI < 10000), identify probable causes of secondary ITP:
Autoimmune disorder: SLE(?), Antiphospholipid syndrome(?)
Infections: HIV(?), HCV(?), Helicobacter pylori(?)
Drugs: heparin(-), antibiotics(penicillin, linezolid, rifampin, sulfonamides, vancomycin), NSAIDs(-), Platelet inhibitors(-), food(walnuts, milk, cranberry juice), beverage, herbs
Vaccines: measles(-), mumps(-), rubella(-), varicella(-)
Lymphoproliferative disorder: not evident at present
[Impression]
1. Severe thrombocytopenia, suspect immune thrombocytopenia(ITP)
2. HTN
[Suggestions]
1. Follow CBC QOD and component therapy with "減白加電血品" Plt 12u stat to keep platelet number > 10 k/uL.
2. 請血液室做PB smear
3. Check following blood exams "1hr after" platelet transfusion
CBC, Bil-T, LDH, IgG, direct Coomb's test (profile of Coombs’ poly-specific and monospecific DAT)/indirect Coomb's test, PT/aPTT, D-dimer, fibrinogen
4. Calculate 1 hr CCI:
Corrected count increment(CCI) = [post-transfusion platelet increment(/uL) x BSA (m2)] x 10^11/ number of platelets transfused
(each single donor platelet 12u = 3 x 10^11 platelets)
If 1 hr CCI < 10000, platelet transfusion refractoriness is confirmed (immune/non-immune factors related high consumption).
Prepare HLA-matched irradiated platelet
5. 根據臨床狀況及PB smear(isolated thrombocytopenia with giant platelet)判斷像不像ITP,若像ITP則加驗下列:
HIV combo test, HBsAg, anti-HCV, 碳13 尿素呼氣檢查(cash)
Anti-ENA screen, anti-dsDNA, C3/C4, ANA, anti-cardiolipin IgG/IgM, anti-beta2 glycoprotein 1 IgG/IgM, DRVVT
(anti-phospholipid IgG/IgM, RF, Anti-TPO)
Plateletantibody (血小板HPA抗體及HLA Class I抗體篩檢: optional)
6. Arrange CxR to exclude pulmonary tuberculosis & mediastinal lymphadenopathy.
7. Arrange abdominal echo to exclude splenic sequestration of platelets. (optional)
Treatment for ITP
1. Give IVIG for life threatening bleeding
- Vena 1 amp before IVIG infusion.
- IVIG (1g/kg) QD x 2 days, 第一瓶run 50ml/hr, 第二瓶run 100ml/hr, 若無不適,可上調流速至200ml/hr
2. Give dexamethasone 40mg PO QD x 4 days
3. Inform risk of rare severe bleeding (such as ICH or GI bleeding ~ 5%)
4. BM study (A+B+C) is suggested in elderly(> 60 y/o) ITP patient to exclude BM neoplasm/MDS.