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We Practice What We Teach Milwaukee Symptoms and Special Circumstance in MPNs 2014 Florida Patient Symposium Laura C. Michaelis, MD Medical College of.

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Presentation on theme: "We Practice What We Teach Milwaukee Symptoms and Special Circumstance in MPNs 2014 Florida Patient Symposium Laura C. Michaelis, MD Medical College of."— Presentation transcript:

1 We Practice What We Teach Milwaukee Symptoms and Special Circumstance in MPNs 2014 Florida Patient Symposium Laura C. Michaelis, MD Medical College of Wisconsin, Milwaukee

2 We Practice What We Teach Milwaukee Spectrum of Symptoms –clinical conditions with high relevance for the duration and quality of the patients life, but with limited evidence to support sound diagnostic and therapeutic recommendations… –Tiziano Barbui. 2010

3 We Practice What We Teach Milwaukee Clone EMD Spleen Dyspoesis Clotting, Bleeding Cytokines Fevers, fatigue, NS Catabolic State Fatigue, Weight loss

4 We Practice What We Teach Milwaukee Spectrum of Symptoms Day-to-Day –Fatigue, Itching, Night sweats, Bone Pain, Fevers, Bleeding, Erythromelagia Life-Threatening –Arterial and Venous Clots, Bleeding Medication Associated –Side Effects, Anxieties, Financial Special Circumstances –Surgery, Contraception and Pregnancy

5 We Practice What We Teach Milwaukee Heterogeneous Presentations: Symptoms Mesa, Cancer 2007

6 We Practice What We Teach Milwaukee SX of Disease TX Tox Sx of Disease Risks and Benefits

7 We Practice What We Teach Milwaukee Case #1: Denise 46 yo woman with newly diagnosed PV –History of a blood clot in the left leg following her last pregnancy, 8 years ago –She has had 5 phlebotomies since diagnosis and her CBC demonstrates good control of her blood counts –She has been allergic to aspirin since childhood She tells you: Im still having a lot of itching after showering

8 We Practice What We Teach Milwaukee Aquagenic pruritus Often occurs with PV –Stinging, itching – often after contact with water –Majority of patients experience it Recent German study demonstrated 68% of PV patients reported about pruritus Can be relentless and may not always respond to treatment for the disease

9 We Practice What We Teach Milwaukee Treatment options for Pruritis Symptom-Oriented –Antihistamines –Paroxetine –Light therapy –Aprepitant Disease-Oriented –Cytoreduction: HU or IFN –Jak-Stat Pathway therapy –Aspirin

10 We Practice What We Teach Milwaukee Case #2: Carla 64 yo woman with ET –Diagnosed after a stroke at the age of 55 –Blood numbers are under good control –Taking HU to control platelet count But Im so tired at night – especially after eating.

11 We Practice What We Teach Milwaukee Managing MPN Fatigue Symptom-Oriented –Exercise (low-intensity as good as high intensity) –Healthy Lifestyle and Diet –Correction of Iron Deficiency When Possible –Stimulants: Ritalin/Provigil/ Nuvigil Disease Treatment –JAK2 Inhibitors

12 We Practice What We Teach Milwaukee Spleen-Related Symptoms: N=1433 PrevalenceSeverity Scherber Blood 2011

13 We Practice What We Teach Milwaukee COMFORT-1: Symptoms Verstovsek S et al. NEJM 2012; 366;

14 We Practice What We Teach Milwaukee Case #3: Jessica 42 yo mother Essential Thrombocythemia Diagnosed on routine blood testing at GYN office No risk factors WBC 12.3; Hgn 13; Plts years after diagnosis, reports foot pain. Occurs when walking or standing on her feet Burning, painful, reddish

15 We Practice What We Teach Milwaukee

16 We Practice What We Teach Milwaukee Case #3 Jessica Erythromelalgia –Neurovascular pain disorder –Can occur secondary to ET –Characterized by severe burning pain and redness –Can be debilitating Treatment –Aspirin, Cytoreduction –Gabapentin

17 We Practice What We Teach Milwaukee Spectrum of Symptoms Day-to-Day –Fatigue, Itching, Night sweats, Bone Pain, Fevers, Bleeding, Erythromelagia Life-Threatening –Arterial and Venous Clots, Bleeding Medication Associated –Side Effects, Anxieties, Financial Special Circumstances –Surgery, Contraception and Pregnancy

18 We Practice What We Teach Milwaukee Case #4: Gerald Gerald S. –56 yo man with newly diagnosed Polycythemia Vera Hgn 19.3 gm/dL Hct 58% WBC 12.4 k/uL Plts 338 k/uL –I recommend phlebotomy and starting a low- dose aspirin. He asks – how many treatments will I need and whats our goal?

19 We Practice What We Teach Milwaukee PV: What is the optimal hematocrit? January 2013

20 We Practice What We Teach Milwaukee Target Hematocrit Hct <45% Hct 45-50% High HctLow Hct All Events 18/ % 5/ % MF/MDS/A ML 38 BLEEDING Which group developed more arterial and venous clots? Which group experiences more bleeding episode? Which group develops fibrosis or leukemia more readily?

21 We Practice What We Teach Milwaukee Case #4: Gerald So – answers? –Phlebotomy goal should be a hematocrit of less than 45% –In women, generally aim for even lower than that, 42-43% Frequency varies – but as often as needed Sometimes medication also needed, but you have to give phlebotomy a chance

22 We Practice What We Teach Milwaukee Case #5: Kyle 57 yo man with Essential Thrombocythemia –Incidentally discovered two years ago –No symptoms, no history of blood clots –Platelet count of 1,380 k/uL –Now with found to occult + stools –Colonscopy normal, but stomach ulcers noted on endoscopy

23 We Practice What We Teach Milwaukee Bleeding vs. Clotting Not as common as clotting problems Often manifest with –Nosebleeds –Gum bleeding –Menorrhagia –Less likely to be deep tissue bleeding Rarely can be life threatening Risk increases with Platelets>1,000,000/uL

24 We Practice What We Teach Milwaukee Acquired VWD Normal Blood Vessel Increase in platelets

25 We Practice What We Teach Milwaukee Case #5: Kyle What can we do about his nose bleeds? –Normalization of platelet count –Medication vigilance combos in particular Anagrilide + Aspirin Plavix or Aspirin + heparin products –Predictable bleeding i.e. interventions to prevent menorrhagia –Special care in individuals with gastric ulcers or esophageal varices

26 We Practice What We Teach Milwaukee Case #6: Bonnie Surgery and VTE Increased risk for patients with MPN Likely due to differences in the –Blood vessels –Platelets –Clotting factors? 67 years old with PV TIA in her late 50s Treatment: HU and aspirin Recently diagnosed with small left-sided breast cancer, has opted for mastectomy What are my surgical risks?

27 We Practice What We Teach Milwaukee Modifying Surgical Risk Planning --Assessment by hematologist --Optimize blood counts --Especially platelets if splenectomy planned Preoperative --Discontinue ASA Postoperative --Anticoagulation – LMWH --Clinical vigilance re hemorrhage --US of abdominal veins

28 We Practice What We Teach Milwaukee Spectrum of Symptoms Day-to-Day –Fatigue, Itching, Night sweats, Bone Pain, Fevers, Bleeding, Erythromelagia Life-Threatening –Arterial and Venous Clots, Bleeding Medication Associated –Side Effects, Anxieties, Financial Special Circumstances –Surgery, Contraception and Pregnancy

29 We Practice What We Teach Milwaukee Gender-based differences Differences between the disease incidence in men and women Problems specifically faced by women Contraception Pregnancy/Fertility

30 We Practice What We Teach Milwaukee Cancer: Sex-based differences Breast Ovarian Cervical Testicular Prostate

31 We Practice What We Teach Milwaukee Cancer: Gender-based differences

32 We Practice What We Teach Milwaukee Gender and Cancer Does the disease occur more frequently in one sex vs. the other? –Diagnostic bias? –Due to exposure? –Due to genetic predisposition? Does the disease behave differently in one sex vs the other? –Modulated hormones? Gender-based lifestyle differences? –Interactions that we dont understand? Are there different consequences to the disease or treatment that depend on gender?

33 We Practice What We Teach Milwaukee Sex Ratio Hematologic diseases DiseaseMale:Female Ratio AML1:1 ALL1.3:1.0 HD1.3:1.0 Multiple Myeloma1.4:1 CLL2:1 CML3:2 ETFemale Predominance PV1.2:1.0 MF1:1

34 We Practice What We Teach Milwaukee Cartwright et al. British Journal of Hematology 2002, More women diagnosed than men More men diagnosed than women

35 We Practice What We Teach Milwaukee Clinical Trial Inclusion Trial Total Patients MaleFemale HU in High-Risk ET NEJM (32%) 77 (68%) ASA in PV NEJM (59%) 210 (41%) HU vs Anagrilide in high-risk ET NEJM (42%) 467 (58%) Ruxolitinib in MF (US Study) NEJM (54%) 142 (46%)

36 We Practice What We Teach Milwaukee Case #7: Jennifer 37 yo woman with a history of thrombosis in her right calf while on birth control Found to have JAK2 mutation and a slightly elevated platelet count She asks you: did the birth control or ET cause the blood clot? Can she take birth control again? Can she try and get pregnant?

37 We Practice What We Teach Milwaukee Challenges: Clotting ET – most common MPN in fertile women Hormonal contraception + ET = hypercoaguable state Pregnancy + ET = hypercoaguable state Thrombosis -- #1 cause of maternal death

38 We Practice What We Teach Milwaukee Challenges: Fertility Contraception –Combination hormones >progesterone only OCPs –General population have a 3–6-fold increased risk of venous thrombosis with OCPs One retrospective study of >300 patients. Subset on OCPs –ET + OCPs = 23% VTE –ET no OCPs = 7% VTE

39 We Practice What We Teach Milwaukee

40 We Practice What We Teach Milwaukee Challenges: Pregnancy Pregnancy outcomes likely impacted –Live birth rate 50-70% –First trimester loss 10-20% –Late pregnancy loss 10% –Increased rates of placental abruption, intrauterine growth restriction Can we change those outcomes?

41 We Practice What We Teach Milwaukee Preconception Counseling Risk Assessment –Prior VTE or arterial clot –Prior hemorrhage –Prior pregnancy complication –Diabetes or Hypertension requiring treatment –Platelet count of >1500 X 10 9 before or during pregnancy

42 We Practice What We Teach Milwaukee Preconception Counseling Multidisciplinary approach Discussion of teratogenic drugs Therapeutic options –Aspirin –LMWH –Cytoreductive therapy Delivery and post-partum plan Breastfeeding information

43 We Practice What We Teach Milwaukee Pregnancy: Low-Risk Patients Generally –Continue low-dose aspirin –Monitor platelet or Hct Keep HCT under 45% Consider venesection if necessary –Increased plasma volume of pregnancy means no set targets Antiplatelet agents reduce risk of VTE in ET patients Pregnancy is thrombotic Aspirin is likely safe in pregnancy (APLA pts)

44 We Practice What We Teach Milwaukee Pregnancy: High-risk patients Remove possible teratogeneic drugs –Taper off hydrea or anagrilide 3-6 months prior to conception –Hydrea likely contraindicated, men and women –Anagrilide crosses the placenta Cytoreduction –Interferon-alpha -- Case reports indicating likely safe Prevent Clotting –LMWH –Prophylactic or, in some cases, therapeutic doses

45 We Practice What We Teach Milwaukee Summary and Conclusions Some symptoms can be addressed with a palliative approach Some require that the disease be treated Target Hgn, PV Preventing Bleeding Undergoing Surgery Gender-specific issues: Contraception, Fertility and Pregnancy Modifying risk – lifelong effort for all patients –Cholesterol, Blood pressure, SMOKING

46 We Practice What We Teach Milwaukee Outcomes: Venous, Arterial Events like stroke, heart attack, VTE, bleeding MPN Smoking lipids Exercise Healthy Weight DM HTN control

47 We Practice What We Teach Milwaukee Conclusions Get involved in your care –Partner with your physician –Educate other physicians, care-providers Ask questions Participate in clinical trials Control what you can Any questions?

48 We Practice What We Teach Milwaukee Thank yous to All the patients Ann Brazeau MPN Research Foundation The Chicago MPN Roundtable Jamile Shammo Toyosi Odenike Brady Stein Damiano Rondelli My mentors Wendy Stock Richard Larsen Patrick Stiff Sucha Nand Mary Horowitz Ruben Mesa


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