Division of Family Practice Platelet and WBC disorders Adrian Yee MD FRCPC Clinical hematologist Assistant Dean, undergraduate education, IMP asyee@uvic.ca
When we understand that slide, we'll have won the war.
Conflict of Interest In the last 12 months, I have received honoraria from: Celgene Boehinger-Ingelheim Lundbeck
Learning outcomes At the end of this interactive workshop, you will be able to: Develop an individualized approach to manage patients with platelets and WBC disorder. Counsel patients on management plan and arrange appropriate follow up.
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
Questions: What are the differential diagnoses? What are you going to inform her?
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 Sometimes: 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 Iron studies, Retic count. ANA, anti-dna, RF, ANCA More contentious: bone marrow biopsy Abdominal ultrasound
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.