Hematology update 2013
Hospital Ampang
Toh See Guan
Approach to thrombocytopenia
Step 1: Exclude platelet clumping
Case 1
Madam Chin, 60 year-old lady
Admitted with acute myocardial infarction
Platelet count 38 x 109/L
Streptokinase not given
Request platelet transfusion prior to antiplatelet therapy
Patient succumbed from CCF 1 week later
Pseudo-thrombocytopenia
Platelet clumping
Normal FBP
Urgency for specific therapy
Step 2: Exclude TTP/ APML
Case 2
32 year old stockbroker, male
Good health
Petechiae and purpura LL for 1 week
TW 8 Hb 11.5 Plt 14
Bilirubin 19
Commenced prednisolone for ITP
J Suriar 2012
Initial blood film
J Suriar 2012
Case 2- cont’d
Hb done a year ago 15.6
Presented to ED 1 week later with status epilepticus
Intubated and transferred to ICU
TW 13 Hb 9 Plt 14
Bilirubin 42 ID 38
LDH 2450
J Suriar 2012
Repeat blood film
J Suriar 2012
What is the diagnosis?
Thrombotic Thrombocytopenic Purpura (TTP)
1. Fever
2. Petechiae/ purpura
3. MicroAngiopathic Haemolytic Anaemia (MAHA)
4. CNS Symptoms confusion, seizures, stroke
5. Renal failureTTP plasma
Normal plasma
Idiopathic TTP
Acquired deficiency of ADAMTS13
Accumulation of HMW vW multimers
Disseminated small vessel thrombi
CNS predilection
Diagnosis is clinical
Mortality is high without appropriate management
Moake J. N Engl J Med 2002;347:589-600
Case 2- Progress
Plasma exchange
1 – 1.5 plasma volume, daily
Corticosteroids
Aspirin when plts > 50
Discharged on Day 14
Case 3 34 year-old lady
c/o fever and petechiae LL x 2 weeks
Hb 8.0 TW 39.3 Plt 18
Diagnosis: APML in DIC
Acute ProMyelocytic Leukaemia
Acute Promyelocytic Leukemia
Case 3- cont’d Treatment started on 5th July 2008:
ATRA 40 mg bd
iv dexamethasone 8 mg tds
iv idarubicin D1, D3, D5
In remission since
Drugs
Sepsis
Step 3: Exclude secondary causes
Case 4
29 yr old IDU
native tricuspid valve endocarditis
Blood culture : MRSA, IV vancomycin
commenced
Baseline FBC : TW 3 Hb 10 plt 280
After 5 days …
c/o gum bleeding
Plt 13
HIV serology negative
PBF confirms thrombocytopenia
BMAT normocellular
What is the cause?
Vancomycin- induced thrombocytopenia
How?
reduced production
increased destruction
Rx: stop offending drug
Drug-induced thrombocytopenia
Updated list
http://www.ouhsc.edu/platelets/ditp.html
Gradual and progressive in most
Rapid and severe in fiban type (e.g.tirofiban,
eptifibatide)
Case 5
52 year old farmer
Poorly controlled diabetes
Infected diabetic foot ulcer
Admitted with fever, hypotension, confusion
Hb 9.4 TW 12 plt 120
BU 9 Creat 130
Case 5- cont’d
Blood culture : Pseudomonas aeruginosa
Intubated for resp distress Day 3
TW 20.2 Hb 9 Plt 46
Creat 180 BU 10
PT 21 s APTT 39 s TT 20 sec
Fib 0.9 D dimer 35 ug/mL
What is the diagnosis?
1. Septicemia
2. Sepsis associated thrombocytopenia
3. Sepsis associated DIC - likely
Organ dysfunction
Case 6
61 F
NSTEMI June 2012
Referred to IJN from Hosp PD
Admitted for heart failure
Noted thyrotoxicosis
free T4>100 TSH 0.01
Case 6- cont’d
Noted plt low 61 on admission
Referred to haematology
Discharged from IJN with
Plavix, aspirin
Bisoprolol, nitrate, vasteral
Carbimazole
But no diuretics
Case 6- cont’d
Seen at haematology
JVP raised AB displaced, PSM at LLSE
PR: 110 AF Bilateral gross leg edema
Lungs: dull R side
Ascites ++
Hb 10 TW 6.7 Plt 75
Crea 35 BU 3.3
Case 6- Blood film
What caused the low platelet count?
Congestive cardiac failure
Liver congestion- reduced TPO
Splenic pooling
Uncontrolled thyrotoxicosis- immune
Drugs
Step 4: Exclude inherited thrombocytopenia
Case 7 28 year-old lady
G1 P0 @ 32 weeks
Referred for ITP in pregnancy
Case 7- cont’d O/E: Cushingoid
Hb 9.0 MCV 65 Plt 54
Delivered SVD
Blood loss: 700 mL
Transfused 2 PC
Steroids tapered off
Khong SY 2012
Case 7- cont’d 2 years later…
G2 P1 @ 18 weeks
Hb 8.8 Plt 51
Blood film
Case 7- bleeding history* Menorrhagia since menarche
Duration: 10 days
Soaks 5- 6 pads/day; double protection
Easy bruising since childhood
Case 7- family history 3 siblings- all in India
No f/h of bleeding
Parents are first cousins
Case 7- coagulation tests PT 13.4s
APTT 32.1s
Fibrinogen 4.1 g/L
FVIII 145%
vWF Ag 150%
Case 7- platelet aggregation
Patient % platelet aggregated
Normal control % platelet aggregated
51 x 109/L 150 x 109/L 50 x 109/L
Ristocetin 1.5 mg/mL 0 101 48
Collagen 0.19 mg/mL 30 84 34
ADP 2.0 x 10-5M 42 96 52
Arachidonic acid 5.0 mg/mL
38 72 42
Case 7- diagnosis Bernard-Soulier Syndrome
Deficiency of glycoprotein Ib/IX/V
Unable to bind to vWF
Failure in adhesion and aggregation
Case 8
30 F
G1 P0 @ 24 weeks
Noted Plt 52 Hb 11.7 TW 8.6
No h/o bleeding
May-Hegglin anomaly
Dohle body
A diagnosis of exclusion
Step 5: Consider diagnosis of ITP
Case 9
23 year-old lady
Presented in Feb 2012
c/o:
easy bruising x 3 months
gum bleeding
heavy PV bleeding
Case 9- cont’d O/E:
Pale+
Gum bleeding
Bruises + Petechiae LL
BP 119/74 PR 90
Other systems: NAD
Case 9- cont’d Hb 8.7 TW 4.5 Plt 12
MCV 65
Case 9- blood film
Case 9- cont’d
Started on steroids
1 week later, plt 50
Discharged
Causes of thrombocytopenia
Acquired
Reduced production AA, Leukaemia, MDS, etc
Increased destruction
Immune
- Drugs, ITP, HIT, etc
Nonimmune
- DIC, sepsis, TTP, HUS
Splenic sequestraction
- hypersplenism
Inherited
Take a good bleeding
history
Screen family
Approach to thrombocytopenia: 5 steps
Step 1: Exclude platelet clumping
Step 2: Exclude TTP/ APML
Step 3 Exclude secondary causes
Step 4: Exclude inherited thrombocytopenia
Step 5: Consider diagnosis of ITP
Thank you
The end
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