11/7/2017 Management of the Hospitalized Patient Hematology Cases: Musings from the UCSF Hematology Consult Service Andrew D. Leavitt, MD October 19, 2017 Case #1: Unexpected Pre-op Labs 62 yo man admitted for bilateral Total Hip Arthroplasty has the following pre-op CBC that shows an unexpected anemia. CBC: WBC: 7.1 Hgb 7.8 Hct 29.2 Plt 288 Consult to Heme: 1. Why is he anemic? 2. Please evaluate ASAP, patient in pain and needs surgery. 3. Surgery cancelled until you give us an answer. 4. He is a physician How do you proceed? What do you want to know? 1
11/7/2017 Case #1: Unexpected Pre-op Labs MCV, Retic, Smear, and… Case #1: Unexpected Pre-op Labs …History Major Spine Surgery No Retic on record Ferritin 9 (low); Iron sat 3% (low) 2
11/7/2017 Case #1: Progression to Iron Def. Anemia * POINTS: 1. Microcytosis is late to the table. Trends can be useful. 2. Low ferritin should not be ignored when SI and %sat are normal Modified from Harrison’s 18 th ed *SI = serum iron Case 2: Anemia – Need a Marrow Biopsy? 64 yo woman: “Persistent microcytic anemia. Does she need a bone marrow biopsy?” Most recent CBC that prompted the referral: NORMAL VALUES 3 - 10 x 10 9 /L 4 - 5.2 x 10 12 /L 4.4 – 5.9 x 10 12 /L 12 - 15.5 g/dL 13.6 – 17.5 g/dL 36 - 46%; 41 - 53% 80-100 fL There was no retic & no smear to review when asked to see patient 3
11/7/2017 Case 2: Anemia – Need a Marrow Biopsy? The Mighty EMR 1. Stable MCV 2. Iron studies in early 2015 not c/w iron deficiency 3. Hemoglobinopathy eval in early 2015… Case 2: Anemia – Need a Marrow Biopsy? NORMAL <1.0 2.2 ‐ 3.2 96.7 – 97.8 Normal? Abnormal? Tells you what? 4
11/7/2017 Erythropoiesis: Hemoglobin Switching (Hgb: 2 alpha & 2 “ beta-like ” [aka, non-alpha; 3 options]) 50 So… Globin chain synthesis (%) No Bone Marrow Biopsy YES Alpha Gene Analysis Alpha: Chromosome 16 Beta-like: Chromosome 11 4 Alpha Genes: Silent c.) Trait) Trait) 0 Hg H) Adult Embryo Fetus Birth 6 mo. HgA (96-98) hydrops) HgF (75) HgA 2 (2-3) HgA (25) HgF (<1) Hoffman et al. Hematology 2000 Anemia? Then always MCV, Retic, Smear*, and… EMR trends *Gives insight into marrow and possible need for BM BX 5
11/7/2017 ANEMIA: Basic Outline MCV Retic NOT elevated Retic elevated Fe <80 Anemia of Inflammation Thalassemia (ACD) Pb 80-100 Anemia of Inflammation Bleeding (loss of blood cells): (ACD) 1. Outside the body Primary Marrow 2. Inside Multifactorial Retroperitoneum, body cavity, GI I o RBC: Hgb; enzyme; membrane 2 o RBC (the RBC environment): Immune (AIHA, AlloIHA) Not immune (DIC, TTP, HUS, Sepsis) >100 MDS Elevated Retics B12/folate Hypo-T4 Liver Dz/ EtOH Rx CASE #3: Anemia: Would you transfuse? 6
11/7/2017 Case #3: Does She Need RBC Transfusion? 34 YO woman with hx of Fe-deficiency anemia admitted for RLQ pain. Was found to be anemic and to have a R gonadal vein thrombosis The primary team wants to anti-coagulate for the gonadal vein thrombosis but is concerned because she (i) is so anemic and (2) is having her menses. Heme Consult WBC HGB MCV PLT RETIC Venofer: IV Iron Sucrose 200mg 100mg 200mg 100mg Transfusion Guidelines* - RBCs 2012 AABB Guidelines Hemoglobin Comment >10 RBC Tfx not indicated (typically) 8-10 Usually not indicated – be a doc 7-8 Should be considered (post-op; cardiovascular symptoms) <7 Likely indicated (but think…not always) <6 Indicated with rare exception *Guideline = A place to start your thinking, NOT a place to stop your thinking! 7
11/7/2017 Transfusion Guidelines* - RBCs 2016 AABB Guidelines • The AABB recommends a restrictive RBC transfusion threshold in which the transfusion is not indicated until the hemoglobin level is 7g/dL for hospitalized adult patients who are hemodynamically stable, including critically ill patients, rather than a liberal threshold when the hemoglobin level is 10g/dL (strong recommendation, moderate quality evidence). • For patients undergoing orthopedic surgery or cardiac surgery and those with preexisting cardiovascular disease, the AABB recommends a restrictive RBC transfusion threshold (hemoglobin level of 8g/dL; strong recommendation, moderate quality evidence).The restrictive hemoglobin transfusion threshold of 7 g/dL is likely comparable with 8 g/dL, but RCT evidence is not available for all patient categories. • These recommendations apply to all but the following conditions for which the evidence is insufficient for any recommendation: acute coronary syndrome, severe thrombocytopenia (patients treated for hematological or oncological disorders who are at risk of bleeding), and chronic transfusion–dependent anemia. *Carson et al. JAMA. 2016;316(19):2025-2035 Case #3: Does She Need RBC Transfusion? ANSWER: NO, BUT… Tell me more about your iron deficiency…. For years. Very heavy and prolonged menstrual periods. Struggled with oral iron on and off for years. Factor 8 55 (43-168) vWF Antigen 40 (42-191) Ristocetin Co-Factor 33 (42-191) 8
11/7/2017 Case #4: Prolonged PT 28 yo woman G2P1 36 weeks pregnant admitted for hypertension and proteinuria, concern for pre-eclampsia. INR 3.1. You are called as they are concerned about her coagulopathy. PT 30.2 WBC 11.2 (ANC 8.8; ALC 1.8) INR 3.1 Hgb 11.5 aPTT 22.1 MCV 79 Fibrinogen 525 Plt 262 -She was feeling well prior to this admission -Eats a balanced diet and was not on any medications other than her prenatal vitamins. -Afebrile What is on your differential diagnosis for her prolonged PT? Isolated Prolonged PT (EXTRINSIC) Kallikrein PT XII XIIa HMWK XI XIa EXTRINSIC IX IXa VIIa VII TF VIIIa Ca ++ /PL INTRINSIC TF/VIIa X Xa Va • Early Liver Dz Ca ++ /PL • Early Vit K II IIa (thrombin) • Warfarin • Early DIC Fibrinogen Fibrin XIIIa • F VII def/inhib COMMON X-linked Fibrin 9
11/7/2017 Case #4: Prolonged PT Tests Ordered: Mixing Study: Factor VII activity: <6% (54-169%) Case #4: Prolonged aPTT 22 yo man admitted with abdominal pain going to OR for cholecystectomy. You are asked to evaluate for bleeding risk given lab results. Chemistries – fine CBC – all normal values PT/INR – 12.4/1.0 aPTT 34. On repeat 35 (normal: 22.4 – 32.7) Consult question: “Can we safely take this patient to surgery?” 10
11/7/2017 Case #4: Prolonged aPTT 22 yo man evaluated for possible living-related kidney transplant. ---- Most common cause of an isolated prolonged aPTT? Concern for bleed? Concern for clot? Neither? Disorders with isolated prolonged aPTT (INTRINSIC) Kallikrein XII XIIa HMWK XI XIa EXTRINSIC IX IXa aPPT VIIa VII TF VIIIa INTRINSIC Ca ++ /PL TF/VIIa X Xa Va • F VIII* Ca ++ /PL • vWD II IIa (thrombin) • F IX* • Heparin Fibrinogen Fibrin • F XI XIIIa • F XII COMMON X-linked Fibrin • Contact factors • Lupus anticoagulant (clotting)** 11
11/7/2017 Case #4: Prolonged aPTT 22 yo man evaluated for possible living-related kidney transplant. ---- Most common cause of an isolated prolonged aPTT? Concern for bleed? Concern for clot? Neither? Factor VIII 120 (nl) Factor IX 145 (nl) Factor XI 112 (nl) Factor XII 41 (low) Factor XII deficiency has no known clinical consequence A Brief Image Intermezzo Normal Iron Deficient Target cells Schistocytes 12
11/7/2017 Case #5: Young Man Feeling Fatigued 23 yo man experienced 4 days of progressive fatigue, mild headache, and occasional palpitations. He then had difficulty focusing at work and co-workers said he looked pale. Went to local urgent care. CLINIC EXAM: T 37.1 HR 117 BP 144/68 RR16 100%RA Pale, no acute distress, no scleral icterus Hear, lung and abdominal exams all normal Labs drawn at clinic: CBC: WBC 13.4, Hg 5.1, Hct 15.9 , Platelets 264 Chem: 137/3.6/102/28; Bun 13, Creatinine 0.74 PMH: No prior surgeries or significant medical problems. No GI or GU complaints. The patient was sent to the UCSF ED for evaluation. Case #5: Young Man Feeling Fatigued AT UCSF ER: Patient is as described. BP 144/68, HR 117, Temp 98.8 (37.1), Res 16 O2 Sat 100% (room air) WBC 13.8, Hgb 5, Hct 15.9 , MCV 113, Plt 271 MORE LABS BACK: Reticulocyte count 487K/uL LDH 413, AST 26, ALT 14, Alk Phos 55, T.bili 2.1, D.bili 0.2 Additional labs pending… 13
11/7/2017 Case #5: Young Man Feeling Fatigued You sent Type and Crossmatch to the Blood Bank. You ask them to set up 4 units of blood and you want two units STAT. 20 minutes later blood bank says that his antibody screen is positive, there could be a delay. AND… 25 minutes later they say that he has an antibody and they are working to identify it so that they can provide compatible units. They ask: What do you want to do? You want to ask: Auto or Allo Ab? How many more tubes do you need for a phenotype? Case #5: Young Man Feeling Fatigued Lets discuss the EMR Blood Bank info Direct Coombs = DAT 14
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