1 / 37

Hem/Onc Board Review

Hem/Onc Board Review. June 20 th , 2010 TJ O’Neill. Question 23. What happened longest ago? A. Barry Bonds broke Hank Aaron’s HR Record B. The last Harry Potter book was released C. TJ last took care of a Med E patient in any capacity D. D/c summaries transitioned from paper to electronic.

cais
Download Presentation

Hem/Onc Board Review

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Hem/Onc Board Review June 20th, 2010 TJ O’Neill

  2. Question 23 • What happened longest ago? • A. Barry Bonds broke Hank Aaron’s HR Record • B. The last Harry Potter book was released • C. TJ last took care of a Med E patient in any capacity • D. D/c summaries transitioned from paper to electronic

  3. Question 23 • What happened 1065 days ago? ANSWER C • A. Barry Bonds breaks Hank Aaron’s Record • August 7th, 2007 • B. The last Harry Potter book was released • July 25th, 2007 • C. TJ last took care of a Med E patient in any capacity • July 23rd, 2007 • D. D/c summaries transitioned from paper to electronic • August 2007

  4. Question 1 • A 72 yo man is hospitalized because of dyspnea, anginal chest pain and new-onset anemia. CLL was diagnosed 2 years ago. He has not been treated or transfused. • On exam the pt is pale,bp 110/80, hr 112, rr 24. Diffuse cervical, axillary, and inguinal LAD s present. There is no JVD, lungs are clear, abdominal exam shows splenomegaly, no edema. • Hgb 6.0, WBC 55,000 w/ 90% lymphs, Plt 115,000, Retic count 12%. Tbili 2.8, Dbili 0.6. • Direct coombsPostive for IgG, T/S is A positive • Smear shows microspherocytosis. • Steroids are started and 2 type and crossed units are ordered but no compatible units are available

  5. Question 1 • Which is the most appropriate management at that time • A. Begin EPO • B. Schedule splenectomy • C. Transfuse one unit of A-positive erythrocytes • D. Transfuse one unit of O-positive erythrocytes • E. Withhold transfusion until compatible unit of blood is available

  6. Answer C- Transfuse A negative blood • Symptomatic anemia 2/2 CLL- hemolytic confirmed by Coombs test and microspherocytes • Given lack of pregnancy or prior xfusions very unlikely to have alloimmunization to erythrocyte antigens. Because of broad specificity of autoantibodies in patients with autoimmune hemolytic anemia most donor cells with be technically incompatible on cross-match • EPO not indicated given appropriate retic count • Rarely will splenectomy be needed • Steroids would be given as well but take time to work

  7. Question 7 • 42 y/o man is hospitalized because of hematuria. The pt has a mechanical mitral valve and has been taking warfarin 5mg/day • On exam he is alert and pale. BP 105/65, HR 96, rr 16, lungs are clear. • Labs: Hemoglobin 8.0, Plt 200,000, INR 7.0, aPTT 28, urinalysis shows gross blood • 3 units of FFP are transfused over the next 4 hours. Halfway through pt develops severe dyspnea w/o chest pain or cough. No fever, BP 115/70, HR 104, RR 28, sat 86%. • Pt has bibasilar crackles, no s3 or edema. BNP normal, cxr shows bilateral infiltrates

  8. Question 7 • Which is the most likely diagnosis • A. Anaphylaxis • B. Aspiration pneumonia • C. Pulmonary Embolism • D. Heart Failure • E. Transfusion related acute lung injury

  9. Answer E- TRALI • Occurs generally within 6 hours of plasma rich blood products. • Due to donor antileukocyte Ab reacting with recipient leukocytes causing leukocyte plugs to form in pulmonary capillary bed • Clinically improve in 2-3 days • Management is stop transfusion and give supportive care • Transfusion associated circulatory overload (TACO) unlikely given exam and normal BNP

  10. Other transfusion syndromes • Acute hemolytic transfusion reaction- within 24 hours. Usually due to ABO incompatibility • Delayed transfusion reaction within 7-14 days, more common than acute. Characterized by fever, jaundice, unexplained drop in Hgb, labs show hemolysis. Generally due to a remote transfusion event or pregnancy and low Ab levels • Transfusion related sepsis- Platelets • Febrile non-hemolytic- due to donor cytokines, consider leukoreduced • Allergic reactions- Generally mild unless recipient IgA negative • Transfusion associated GVHD- chemo, blood from 1st degree relatives, premature infants, need irradiated blood. Can be fatal

  11. Question 10 • Is this kid awesome? • A. Yes • B. No • C. Is that a young Kubal?

  12. Answer: Yes

  13. Question 8 • 58 y/o man is evaluated with increasing fatigue over 2 months. Pt has HTN, HLD treated with lisinopril, atorvastatin. A sister has hypothyroid • On exam, Temp normal, BP 135/80, HR 72, RR 18. No LAD or edema. The spleen is palpable. • Hgb 12.1, WBC 55,200, Plt 105,000. Smear shows increased granulocytic cells in all phases but no Auer rods. BM exam shows hypercellular marrow (80% cellularity) with marked granulocytic hyperplasia, left shift in the granulocytes and 3% myelocytes. Cytogenetic testing shows BCR/ABL translocation

  14. Question 8 • Which is most appropriate next step • A. Administer Imatinib • B. HLA typing of pt and his sister • C. Leukopheresis • D. Observation and monthly f/u

  15. Answer A • The pt requires Imatinib (Gleevec). • CML results from balanced translocation of chromosomes 9:22 creating BRC:ABL. Chronic CML pts have less than 10% blasts in marrow and blood. However eventually as disease progresses blast count may increase and progress to blast crisis • HSCT best reserved for very young or refractory • Leukopheresis for blast count > 50,000 • Would not observe because best chance to treat is during chronic phase • Dasatinib and nilotinib are new agents

  16. Question 11 • 66 y/o woman evaluated for 1 month of gradually increasing HA, blurred vision, and episodes of confusion. PMHx and family hx are unremarkable. No meds • She is afebrile, BP 150/80, HR 90. Retinal exam shows dilated, segmented, tortous retinal veins. She has an S3 and clear lungs. Abdominal exam shows hepatosplenomegaly. • Hgb 8.5, WBC 14,000 with 80% lymphs, IgM 4800 mg/dL, LD 80 • CXR shows cardiac enlargement. CT of chest and abdomen shows enlarged liver, spleen and many enlarged lymph nodes. BM biopsy reveals 70% replacement with lymphoplasmocytic cells

  17. Question 11 • Which is the next best therapeutic option • A. Blood transfusion • B. Furosemide • C. Plasma exchange • D. IVIG

  18. Answer C • Pt has lymphoplasmacytic lymphoma (Waldenstrom macroglobulinemia). • Sx include blurred vision, fatigue, mucosal bleeding, headache, heart failure, and altered mentation. Plasma exchange is indicated acutely followed by Ritux or fludaribine. • Fundoscopic exam may show engorged retinal veins • Blood transfusion is contraindicated

  19. Waldenstrom Macroglobulinemia • Proliferation of B lymphocytes that show maturation to plasma cells • Lymphoplasmacytic infiltration of BM • Elevated Igm, LAD, anemia, neuropathy, organomegaly, IgM monoclonal gammopathy and hyperviscocity syndrome

  20. Question 12 • 26 y/o woman admitted for DVT. No PmHx and only takes OCP. • Mild scleralicterus, 37.2, BP 110/67, HR 100, RR 16, abdominal exam shows mild splenomegaly • Hgb 10, WBC 2700, Plt 42,000, Retic 8%, MCV 70, Tbili 5.0, Dbili 0.8, LDH 1126 • AST, ALT, AP are normal. BM bx shows hypocellularmarow, absent iron stores and signs of early myelodysplasia. Direct and indirect cooms are negative. What next? • A. Flow cytometry • B. Hgb electrophoresis • C. Osmotic fragility study • D. Parvovirus B19 serology

  21. Answer A • Pancytopenia, hemolytic anemia, and thrombosis, concerning for PNH. • PNH caused by loss of GPI linked proteins on cell surface causing increased RBC sensitivity to complement • CD55 and CD59 deficiency can be detected by flow • Iron deficiency is common due to chronic iron loss in urine • Thrombosis may occur 2/2 loss of anticoagulants linked to GPI anchors • Eculizamab, Ab to C5, is a recently developed therapy

  22. Question 29 • Which evil organ system caused this problem? • A. Kidneys • B. Lungs • C. Blood • D. Rheumatological

  23. Answer C, blood

  24. Question 15 • 78 y/o woman has 3 month hx of increasing fatigue. No med hx, no meds. Afebrile, HR 72, BP 130/80, RR 16. Pt appears pale. • Hgb 7.8, WBC 2,800 ANC 1,200, Plt 560K, Epo 600 • BM bx shows hypercellular marrow with erythroid hyperplasia and dysplasia of the erythroid and granulocyte series. Megakaryocytes are increased with many hypolobulated cells. Iron stores are normal. Cytogenics studies show deletion of long arm of chromosome 5 (5q-) • Which is the most appropriate • A. Azacitidine • B. Danazol • C. Lenalidomide • D. G CSF and EPO

  25. Answer C • Dysplasia of erythocyte, granulocyte, or megakaryocyte lineages in the setting of hypocellular bone marrow suggest MDS. • Detection of clonal abnormalities in xsomes 3,5,7,8,17 support the diagnosis. • 5q- is characterized by elevated Plts and anemia, typically elderly women with a indolent course. Associated with xfusion dependant anemia, low incidence of neutropenia or thrombocytopenia. • This subtype responds well to leulinomide, thalidomide analogue • Azacitidine is better for other types of MDS • Danazol can treat MDS associated anemia but has many side effects • EPO level too high to give EPO

  26. Question 18 • 54 y/o man evaluated for increased lethargy and vague abdominal symptoms x2 weeks. No PMHx, FHx. No smoking, no meds • BP 136/82, P 90, RR 18, Sat 99% RA. He has no clubbing or cynanosis. Abdomen is soft, no hepatosplenomegaly. • Hgb 20.2, Plt 312,000, WBC 8200, EPO 35 • Which is most appropriate next diagnostic test? • A. Abdominal ultrasound • B. Echocardiogram • C. JAK2 mutation analysis • D. Erythrocyte mass study

  27. Answer A • Pt with elevated EPO levels in the setting of erythrocytosis usually either exogenous EPO or chronic hypoxia • Given normal sat, RCC is the most likely dx. Only 1-5% of pt with RCC have erythrocytosis, 20-80% have anemia. • P vera would have a low EPO level

  28. Causes of Secondary Polycythemia and Elevated EPO Levels • Tumor related increased serum EPO levels • RCC, hepatocellular carcinoma, uterine fibroids • Hypoxemia • COPD, OSA, altitude • Increased carboxyhemoglobin • Smoking • Abnormal hemoglobin • High oxygen-affinity hgb

  29. Question 24 • 19 y/o AAM evaluated 2 days of arm, leg and back pain. PMhx positive for similar episodes of pain but not this severe and anemia of unclear cause. FHx positive for anemia. ROS + poor exercise tolerance, no meds, no allergies. • Exam +scleral icterus, 99.0, 126/62, 112, 18, BMI 22. No splenomegaly or hepatomegaly • Hgb 10.2, WBC 8600, MCV 70, Tbili 5.7, Dbili 0.3. Smear shows rare sickles erythrocytes and target cells • Hgb Electrophoresis. Hgb S 67%, Hgb F 3%, A 25%, A2 5% • Diagnosis? A. Sickle cell trait • B. Sb+ thalessemia • C. Hgb SC disease, • D Hgb S w/ hereditary persistence of fetal hemoglobin

  30. Answer B • Sb+ thalessemia is characterized by hemoglobin S> 60%, elevated A2 and microcytosis. Generally milder than SS disease • Sickle cell trait, AS, have S level <50% and higher A level as well as normal sized RBCs • SC disease would be 50:50 Hgb S to C • SS disease have <70% S and >30% Hgb F

  31. Question 36 • 27 y/o man evaluated for new onset anemia found during routine CBC. Pt is s/p kidney xplant for hereditary nephritis (Alport’s) 2 months ago. Meds are cyclosporine, mycophenolate, prednisone, Bactrim. No other medical hx • On exam pale conjunctiva, 37.5, 136/82, 112, 18. Petichiae on lower extremities. R pelvic kidney is nontender with healed scar • Hgb 8.1, Plt 120,000, WBC 8200, LDH 1430, Cr 3.2, direct and indirect Coombs are negative • A smear is obtained.

  32. Which medication is most likely responsible • A Cyclosporine • B Mycophenolate • C Prednisone • D. Trimethoprim- sulfamethoxazole

  33. Answer A • Pt has cyclosporine induced HUS • MAHA with negative Coombs. • Would stop cyclosporine, PLEX is not well studied for this syndrome • Mycophenolate and Bactrim would cause myelosuppression but not MAHA

  34. Question 47 • 57 y/o woman evaluated for 1 week of swelling and pain in LLE. 2 normal pregnancies, no miscarriages. No PMhx or hx of clots • DVT is confirmed on u/s and pt is started on unfractionated heparin infusion. Warfarin 5mg/day is also started • Which is the most appropriate heparin therapy? • A. Minimum 5 days, 2 INR measurements >2, 24 hr apart • B. Minimum 2 weeks, with 4 INR measurements >2, 24hr apart • C. Min 3 days with 1 INR measurement >2 • D Min 24 hours with 1 INR measurement of >2

  35. Answer A • Need >24 of fully therapeutic Warfarin to allow further reduction of prothrombin, the Vit K dependant clotting factor with the longest half-life (60hr)

  36. Question 50 • 50 y/o woman with advanced multiple myeloma diagnosed 6 months ago comes for follow up. Treatment includes daily thalidomide and pulse dexamethasone. She feels well. • Labs show serum monoclonal protein concentration of 3.0 g/dL. Hgb concentration, serum calcium, and renal function are normal. BM aspirated shows reduction in plasma cells from 50% to 10% • Which of the following is most appropriate trx to optimize this patients disease free and overall survival. • A. Autologous stem cell transplantation • B. Continuation of oral thalidomide • C. Initiate parenteralbisphosphonates • D. Initiate oral melphan

  37. Answer A • Thalidomide plus Dex is first line for pts less than 65 who are candidates for xplant. Response to chemo is 65-75% which should be followed by stem cell transplant. • Oral melphan is used for MM but can impair collection of stem cells

More Related