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Division of Family Practice
Adrian Yee MD FRCPC
Clinical hematologist
Assistant Dean, undergraduate education, IMP
Platelet and WBC disorders
Conflict of Interest
• Celgene
• Boehinger-Ingelheim
• Lundbeck
In the last 12 months, I have received honoraria from:
Learning outcomes
Develop an individualized approach to manage patients with
platelets and WBC disorder.
Counsel patients on management plan and arrange
appropriate follow up.
At the end of this interactive workshop, you will be able to:
Case 1: high platelets
38 year-old G1P1 F with long standing history of
thrombocytosis (~700). Initiated on oral contraceptives 3
months post-partum. Presented with abdominal pain,
jaundice.
CT abdomen/pelvis: portal vein thrombosis/mesenteric
venous thrombosis.
Approach to thrombocytosis
History: thromboembolic complication, bleeding, bruising,
constitutional symptom, adenopathy, abdomninal symptom
or alteration of bowel habits.
Physical: abdominal examination for ?splenomegaly.
Lab: Repeat CBC differential, peripheral smear, LFT,
Creatinine, Ferritin, Hep B/C/HIV and abdominal ultrasound
Consider JAK 2 mutation if thrombocytosis persists.
When is it an emergency?
No clear cut answer unlike thrombocytopenia.
It is a concern if patient has recent bleeding/thrombosis with
high platelet counts.
??Platelet over 1500.
??JAK 2 mutation
Janus kinase 2 mutation
“regulator” of hematopoiesis.
Mutation is present in 90% of patients with polycythemia
vera, 60% of patients with essential thrombocythemia.
Why is it important to know? New therapeutics…JAK 2
inhibitor (Ruxolitinib)
ASH education 2012
Management of Essential Thrombocythemia
Low risk group (age less than 60 and without thrombosis):
aspirin and cardiovascular risk management.
High risk group: hydroxyurea and aspirin.
Other option: Anagrelide
Novel therapy: Ruxolitinib (JAK 2 inhibitor)
B
l
o
o
d
Low platelet
32 year old M with recent viral illness. Now has easy
bruising. No other symptom.
On examination: no splenomegaly. Ecchymosis at the lower
extremities.
Lab: hemoglobin 132, WBC 7.2, Platelet 13.
Approach to low platelets
What are the differential diagnoses?
History: bleeding/bruising, constitutional symptom, recent
infection, joint symptom, rashes.
Physical examination: oral blood blister, skin, spleen,
lymphadenopathy, MSK
Lab investigations
CBC differential, Creatinine, Calcium, AST, ALT, Bilirubin, Hep
B, Hep C, HIV.
Abdominal ultrasound.
? ANA, Anti DNA, Rheumotoid factor.
?Bone marrow biopsy
Immune thrombocytopenia purpura
Treatment is recommended when platelet is less than 30.
First line treatment: Prednisone/Dexamethasone, IVIG
Second line: Splenectomy
Third line: Rituximab, Immunosuppressive Therapy and
Thrombopoietin agonist (Eltrompopag, Nplate)
Abnormal neutrophils
62 year old presented with fatigue and lightheadedness.
CBC diff: Hgb 112, WBC 300, Neutrophil 160 with left shift
and blast, Platelet 62.
When it is an emergency?
Hyperviscosity symptoms: headache, visual blurring,
confusion, tinnitus, unusual bleeding/bruising.
Total WBC > 50 if it is of myeloid lineage.
Blast in the peripheral film.
Severe cytopenia.
Lymphocytosis = seldom causes hyperviscosity.
Chronic myeloid leukemia
Rare disease: 1 in 100,000
Arises from single mutation (philadephia chromosome)
Excellent prognosis with treatment (Imatinib).
Neutropenia
23 year old F presented with fatigue. No other symptom.
Bloodwork: Hemoglobin 117 MCV 81
Platelets 190
WBC 0.7 and Neutrophil 0.2
Is this an emergency?
Patient is unwell with fever, symptom of infection, oral ulcer,
etc..
Neutrophil less than 0.5.
Pancytopenia.
Blast in the peripheral film.
Approach
History: fever, infectious symptom, unusual
bleeding/bruising, fatigue, constitutional symptom, MSK
symptom, connective tissue disorder screening questions.
Physical examination: oropharynx, lymphatic system,
abdominal, cardiopulmonary systems.
Differential diagnoses
Underproduction: Bone marrow failure- leukemias, lymphoma, myeloma, myelodysplasia, aplastic anemia, etc. Drugs: chemotherapy, methotrexate, imuran, etc. Cyclic neutropenia.
Destruction: Autoimmune disease Medications: penicillin, vancomycin, levamisole Sepsis Viral infection: Hep B, C, HIV, EBV, CMV
Investigations
Always:
CBC diff, peripheral smear,
creatinine, ca, LFTs, vitamin
B12, SPE, urine protein
electropheresis, Hep Bs Ag,
Hep B core antibody, hep C
and HIV
Abdominal ultrasound
Sometimes: Iron studies, Retic count. ANA, anti-DNA, RF, ANCA
More contentious: bone marrow biopsy
Does she need to be hospitalized?
Fever
Severe pancytopenia
Presentation is suggestive of Acute Leukemia.
When would you be less concerned about neutropenia?
Chronic neutropenia for years.
Intermittent.
Neutrophil is always over 1.0.
Lack of symptom/infection.