Division of Family Practice

Adrian Yee MD FRCPC

Clinical hematologist

Assistant Dean, undergraduate education, IMP

asyee@uvic.ca

Platelet and WBC disorders

When we understand that slide, we'll have won the war.

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.

Management

Initiate on Anticoagulation.

Stop OCP

Investigate causes of high platelets

What are the differential diagnoses?

What are you going to inform her?

Questions:

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.

Differential Diagnoses

1. Acute myeloid leukemia.

2. Chronic myeloid leukemia.

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.

Summary

Develop an individualized approach to manage patients with

platelets and WBC disorder.

Counsel patients on management plan and arrange

appropriate follow up.