5. An IV line should be established and flushed with 0.9% saline solution before the pack is opened. 6. No drug or other infusion solution should be added to any blood component. 7. Monitoring of the patient involves recording temperature, pulse and blood pressure befor e transfusion, every 15 min for the first hour and hourly until transfusion is finished. 8. Adverse events should be recorded meticulously. 9. Major reactions require immediate ces sation of the transfusion and instigation of a full investigative protocol (see Emergencies : Transfusion reactions, p 502 \200\223504). 10. Minor febrile reactions are not uncommon, their occurrence should be recorded, simple m easures such as slowing the rate of infusion or administration of an antihistamine may deal with the problem; if not transfusion of the specific unit should be stopped. 649 11. An RBC pack should be given within 30 min of removal from the blood bank; the target in fusion time for an individual unit should be \211 4h. 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 650 Platelet transfusion May be given as prophylaxis against bleeding e.g. in patients undergoing intensive chemothe rapy or to arrest overt hemorrhage e.g. in DIC. Platelets may be required to cover surgery and dentistry. Indications for platelet transfusion 55 production due to BM failure/infiltration Acute and chronic leukaemias Myelodysplasia Myeloproliferative disorders and myelofibrosis Marrow infiltration with other malignant tumors Post-chemotherapy or TBI Aplastic anemia 44 platelet destruction in peripheral circulation hypertension Hypersplenism 2 splenic infiltration or portal Consumptive coagulopathies e.g. DIC Avoid in TTP (p530) Acute and chronic ITP (in emergenci es only) Alloimmune thrombocytopenias e.g. PTP and perinatal thrombocytopenia (need to be H PA typed) Sepsis Drug induced Platelet function abnormalities Aspirin and NSAIDs Myelodysplasia Rare congenital disorders e.g. Bernard \200\223Soulier Dilutional Massive blood transfusion \200\224in practice not usually required unless some other haemos tatic abnormality (e.g. consumption) Cardiac bypass Dilution and damage to platelets in extracorporeal circulation. Indications for irradiated platelets Recommended in immunosuppressed patients and haemopoie tic stem cell transplant recipients to prevent transfusion-associated GvHD. Indications for CMV negative platelets 1. Where CMV transmission may cause disease. 2. BM a nd PBSCT recipients who are CMV \200\223ve. 3. Solid organ transplant recipients who are C MV \200\223ve. 4. 5. Aplastic anemia. 6. GvHD. 7. Primary immunodeficiency syndromes. 650 In utero and neonatal transfusion. 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 651 Blood transfusion 651 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 652 Fresh frozen plasma (FFP) FFP is prepared by removing plasma from a single donor unit by centrifu- gation within 8h o f donation, snap frozen at \200\22380 C and maintained deep frozen until use. Serological testing excludes HBV, HCV, HIV and the product is ABO and Rh (D) grouped. Group AB Rh (D) \200\223ve FFP is suitable for all groups since it lacks anti-A and B, and will not sensit ize Rh(D) \200\223ve patients to Rh(D). Range and median concentrations of vitamin K dependent VII Factor coagulation factors in FF P Range (u/dL) Median II IX X 53 \200\223121 82.5 41 \200\223140 92.0 32 \200\223102 61.0 61 \200\223150 90.5 Indications for use 2 Warfarin overdose \200\224see p522. 2 DIC (common causes include obst etric hemorrhage, postoperative complications, following trauma, severe infection, septicae mic shock, acute blood loss \200\224see p512). 2 Liver disease and biopsy. 2 Massive blood transfusion \200\224use of prophylactic FFP (1 \200\2232 units FFP/10 units of blood) and platelets is not supported by documented clinica l benefit. Give as dictated by coagulation tests. 2 Isolated coagulation deficiencies where no specific concentrate is readily available. 2 Treatment of thrombotic thrombocytopenia purpura/haemolytic uraemic syndrome (see p468, 530). 2 Non-specific haemostatic failure in a bleeding patient e.g. following surgery, in intensi ve care with disturbed coagulation tests where no definite diagnosis is made. Instructions for use The average volume of 1 unit is 220 \200\223250mL. Half-life of infused coagulation factors in FFP <12h >12 <24h >24 <48h >48h Factors V, VII, VIII, and protein C Factor IX and protein S Factor X Fibrinogen, factors XI , XII, XIII, ATIII 652 2 Defrost the bag in a waterbath (5 min) or at room temperature (20 min). 2 Give as soon as possible and at least within the hour through a filter needle. 2 Must be group compatible; if blood group not known, give \200\230all groups \200\231. 2 If recipient Rh (D) \200\223ve 3 of child bearing age given Rh (D) +ve plasma give anti-D (250u). 2 Dose 10 \200\22315mL/kg body wt (usual starting dose in an adult = 2 \200\2234 units depending on the PT). 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 653 Blood transfusion 2 Check PT and APTT before and 5 min after infusion to assess response. 2 Note clinical response in bleeding patients; repeat as necessary, remember short half-life. 653 Makris, M. et al. (1997) Emergency oral anticoagulant reversal: the relative efficacy of in fusions of fresh frozen plasma and clotting factor concentrates on correction of coagulopat hy. Thrombosis Haemostasis, 77 477 \200\22380. 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 654 Cryoprecipitate 2 Prepared by slow thawing of FFP at 4 \200\2236 C. Fresh plasma taken from a single donor is snap frozen then thawed at 4 C and a cryoprecipitate forms. 2 Precipitate formed is cryoprecipitate which is then stored at \200\22330 C. 2 Rich in f actors VIII, XIII, fibrinogen and von Willebrand factor. Per unit (bag): \200\223 Factor VIII and vWF 80 \200\223100iu. \200\223 Fibrinogen 250m g. \200\223 Factor XIII and fibronectin. \200\223 Does not contain other coagulation fact ors. \200\223 May contain anti-A and anti-B blood group antibodies. 2 Formerly (but no longer) used for management of bleeding in factor VIII deficiency and von Willebrand \200\231s disease. 2 Main clinical use for cryoprecipitate is as additional support for the clotting defects induced by massive transfusion and DIC. 2 All donations are screened for HIV, HBV and HCV. Indications for use 2 Hemophilia A and vWD not treatment of choice where virally inacti- va ted concentrates are available. May still have a role in acquired vWD when purified factor VIII products are ineffective. 2 Hypo/dysfibrinogenaemia e.g. in DIC and liver disease/liver transplanta- tion is used to treat and prevent bleeding. 2 Of no proven value as empirical treatment in post-op or uraemic bleeding. Instructions for use 2 Keep frozen until required. 2 Thaw at room temperature/37 C (takes 5 \200\22310 min); use immediately. 2 ABO compatibility not required. 2 Give through filter needle. 2 Dose depends on the indications for use and desired increment severe 2 \200\2234 bags/10kg body wt less severe 1 \200\2232 bags. \200\223 Hypofibrinogenaemia: 2 Aim to keep fibrinogen >1g/L. 2 Factor VIII minimum adult dose 5 bags. Complications Viral transmission \200\224rare but reported. Reactions, fever, chills, alle rgic reactions. 654 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 655 Blood transfusion 655 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 656 Intravenous immunoglobulin Used as antibody replacement in 1 and 2 antibody deficiency states, and as immune modul ator. Preparations of intravenous IgG (IVIg) 2 Contain predominantly IgG (with small amounts of I gA and IgM). 2 Prepared from large pool of normal donors e.g. >1000. 2 Contain all subclass es of IgG encountered in normal population. Uses Antibody replacement 1 immune deficiency Transient hypogammaglobulinaemia 2 immune deficiency CLL Non-Hodgkin \200\231s lymphoma Multiple myeloma Post-BMT of infancy Common variable immune deficiency Sex-linked hypogammaglobulinaemia Late-onset hypogammaglo bulinaemia Hypogammaglobulinaemia + thymoma Immune modulation Autoimmune diseases ITP Autoimmune haemolytic anemia Autoimmune neutropen ia Red cell aplasia Coagulation factor inhibitors Post-transfusion purpura (PTP) Neonatal p latelet alloimmunisation Thrombocytopenia in pregnancy Antiviral activity Prophylaxis/treatment of CMV in BMT patients B19-induced red cell aplasi a Haemophagocytic syndromes (viral) Mechanisms of action Not fully understood: natural anti-idiotypic antibodies suppress antib ody production in patient; Fc receptor blockade on macrophages (thereby blocking RE functio n) and T/B lymphocytes (inhibits autoantibody produc- tion); suppression of production of i nflammatory mediators (e.g. TNF-a, IL-1) produced by macrophages. 656 Administration Usual dose 0.4g/kg/d 5d (e.g. ITP, etc) or 0.2 \200\2230.4g/d 1 day mo nthly (CLL, myeloma, etc). Check TPR and BP pre-infusion. With first infusion check TPR and BP 1 \201\2042 hourly for the first hour only. Side effects are more likely at the start o f an infusion and in the first hour. (See pack insert and BNF for details.) Complications 2 Fevers, chills. 2 Backache. 2 Myalgic symptoms. 2 Flushing. 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 657 Blood transfusion 2 Nausea vomiting. 2 Severe allergic reactions in IgA deficient patients (due to small a mount of IgA in IVIg preparation). 657 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 658 Autologous blood transfusion Allows patient to be transfused with his/her own red cells, avoiding (some) problems associ ated with transfusion of allogeneic (i.e. donor) blood, e.g. immunological incompatibility, risks of transmission of infec- tion, and transfusion reactions. Useful for patients who w ish not to receive allogeneic blood or who have irregular antibodies that make cross- match ing difficult. Can be pre-deposit or intraoperative red cell salvage. Pre-deposit system Involves collection of 2 \200\2234 units of blood: the first unit is col lected 2 weeks before the operation and the second is taken 7 \200\22310d prior to surgery . Iron replacement usually given. Some pre-deposit programs use Epo (enables a larger numbe r of units to be collected but expensive). Advantages 2 Can store RBCs up to 5 weeks at 4 C. 2 May donate 2 \200\2234 units pre-op. 2 Avoids many problems associated with allogeneic blood transfusion. Disadvantages 2 Generally requires Hb \211 11.0g/dL. 2 Patients must be \200\230fit \200 \231 for pre-donation program (e.g. to donate 450mL 2 \200\2234 pre-op) and live near transfusion center. 2 Requires close coordination between surgeon, patient and transfusion lab and fixed date for surgery. 2 Little/no reduction in workload \200\224blood must be treated in same way as regular dono r units (including microbiological screening, grouping, compatibility testing, etc). 2 Cost is high. 2 Transfusion should be to donor only. 2 Bacterial contamination of blood u nits may still occur. 2 Patient may still require additional allogeneic units. 2 Blood may be wasted if operation canceled. 2 Patients with epilepsy excluded (risk of seizures). Intraoperative blood salvage Allows blood lost during surgery to be reinfused into patients using suction catheters and filtration systems. Expensive and not widely used in the UK at present. Intraoperative blood salvage useful in cardiovascular surgery but may be used for almost any surgical procedure (provided no fecal contamination or risk of tumor disseminat ion). 1. Single use disposable canisters (e.g. Solcotrans \204 ) where the patient is hepar inised and anticoagulated blood is collected into ACD anticoagu- lant in the canister. Red cells reinfused after filtration through a microaggregate filter. 658 2. Automated or semi-automated salvage (e.g. Hemonetics Cell Saver \204 ). Blood is collect ed, washed centrifugally, filtered and red cells held for reinfusion. Other physical methods \200\224pre-operative haemodilution Involves reducing the Hb concent ration prior to surgery. Reduces blood viscosity and red cell loss (through reduced haemato crit). Provides a bank of freshly collected autologous whole blood for return later. 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 659 Blood transfusion 2 2 \200\2233 units of blood are collected with replacement using crystalloids or colloid solutions. 2 Hb is reduced to 10 g/dL and haematocrit to 30% (0.3). 2 O2 transportation improves (inc reased cardiac output). 2 Used mainly in younger patients and in those with no pre-existing cardiopulmonary disease. Pharmacological methods of blood saving 2 Various drugs used to modify the coagulation and fibrinolytic systems, e.g. DDAVP. 2 Platelet inhibitory drugs e.g. prostacyclin. 2 Aprotinin widely used in cardiovascular su rgery, liver transplantation and other surgical procedures. 659 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 660 Jehovah \200\231s Witnesses 2 Religious sect numbering 120,000 in the UK. 2 Pose ethical and management difficulties du e to their refusal of blood transfusion, derived from a literal interpretation of a number of biblical passages (Acts 1 5:28 \200\22329). 2 Jehovah \200\231s Witnesses still die during both elective and emergency surgery due to their beliefs. Elective surgery \200\224discuss 1. Risks of surgery and the specific risk of refusing bloo d. 2. Extent of religious belief (preferably alone to prevent any external pressure). 3. What blood derived products they personally are willing to accept e.g. albumin, FFP, platelets, etc? If the surgeon agrees to an operation, communication then becomes para- mount and the Jehov ah \200\231s Witness should be referred to both an anesthetist and hematologist \200\224pre ferably when the patient is placed on the waiting list to allow time for any optimization a nd for further counseling with their family. Pre-operative considerations 2 Timing (e.g. liver transplantation before clotting function deteriorates). 2 Autologous blood transfusion \200\224not permitted but may be acceptable to some. 2 Morning list \200\224to allow post-op observations during \200\230office hours \200\231 . 2 Admission to ITU for invasive monitoring if required. 2 Stop anticoagulants and NSAIDs. 2 Optimize Hb \200\224nutrition, B12, folate, Fe, Epo. Operative considerations 2 Surgeon Consultant Positioning of patient \200\224to prevent venous congestion Tourniquets if possi ble Speed Meticulous haemostasis Consultant Regional blocks Hypotensive anesthesia Hypother mia Isovolaemic haemodilution \200\223 permitted as long as blood remains linked to circul ation Hypervolaemic haemodilution Intraoperative blood scavenging e.g. cellsavers Blood sub stitutes (fluorocarbons) Pharmacological methods to improve clotting e.g. DDAVP, tranexamic acid, aprotinin Observation for re-bleed \200\224HDU, senior surgeon review Optimize Hb \200\224nutrition enteral or parenteral feeding, B12, folate, Fe, erythropoietin Severe ane mia \200\223 IPPV to reduce oxygen demand Reduce phlebotomy and use pediatric vials Acid s uppression to reduce GIT bleeding. 2 Anesthetist 660 2 Post-operative considerations 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 661 Blood transfusion Emergency surgery 2 Advanced directives. 2 Early investigations CT, USS abdomen, pelvis. 2 Low threshold to theater. Children 2 Communication with parent. 2 Judicial intervention if required. 661 Marsh, J.C. & Bevan, D.H. (2002) Hematological care of the Jehovah s Witness patient. Br J Haematol, 119, 25 \200\22337. 17-blood%2fproducts.qxd 10.12.2003 04.34 PM Page 662 This page intentionally left blank 662 18-phone numbers.qxd 10.12.2003 04.35 PM Page 663 Phone numbers and addresses 18 CancerBACUP 664 Leukemia Research Fund (LRF) 666 NCRI Leukemia trials 668 Birmingham Clinical Trial s Unit (BCTU) 668 British Socie ty for Hematology 669 Medical Research Council 669 18-phone numbers.qxd 10.12.2003 04.35 PM Page 664 CancerBACUP A major British cancer information and support charity; aims to provide clear, accurate, up -to-date information as well as sensitive and confidential support for patients and their f amilies. Also provides assistance to health care professionals with specific enquiries rela ting to patients in their care. CancerBACUP services for patient \200\231s and their families 2 A national freephone cancer information service (tel 0808 800 1234), staffed by specialist oncology nurses. 2 Cancer counseling service offering people the chance to talk through their concerns face to face (tel 0207 696 9003; fax 0207 696 9002) 2 CancerBACUP produces excellent written inf ormation e.g. booklets on specific cancers, treatments and aspects of living with cancer an d fact sheets on specific chemotherapy drugs, hormonal therapies, brain tumors and rare tum ors. Available from CancerBACUP \200\231s administra- tion (tel 0207 696 9003). CancerBACUP services for health professionals Medical Advisory Committee Statements produce d by an expert panel on controversial or complex oncology issues including 2 Clinical trial s. 2 Cancer screening \200\224cervical, breast, prostate, colorectal and ovarian cancer. 2 Breast cancer, the pill and hormone replacement therapy. 2 Information on Support groups Sources of help 2 Lists of booklets and factsheets. Internet CancerBACUP web site: (cid:27) www.cancerbacup.org.uk 664 18-phone numbers.qxd 10.12.2003 04.35 PM Page 665 Phone numbers and addresses 665 18-phone numbers.qxd 10.12.2003 04.35 PM Page 666 Leukemia Research Fund (LRF) The LRF is one of the major UK based research charities in the field of leukemia and relate d conditions. It supports leukemia research in major academic institutions; work supported ranges from basic science relating to the molecular genetics of leukaemogenesis to extensiv e case controlled studies into epidemiology of leukemia and related disorders. In addition to funding major research it provides information booklets on a range of (mainl y) malignant blood disorders which are suitable for patients, their relatives and carers an d also appropriate for non-specialist professional staff who become involved in specific as pects of the clinical care of the patient with leukemia or a related disorder. The stated aim of the fund is to improve treatments, find cures and prevent all forms of le ukemia and related cancers through a program of nationally funded, high caliber research ac tivities. Booklets are available on the acute and chronic leukaemias, lymphomas including Hodgkin \200\231s disease, myeloma and related disorders, myelodys- plasia myeloproliferative disor ders and aplastic anemia. A revised series of booklets was produced in 1997. Availability o f such information on the wards or in the clinic is vary helpful to patients and their care rs. Address for the Leukemia Research Fund 43 Great Ormond Street, London WC1N 3JJ Telephone 02 07 405 0101 e-mail: info@leukemia.research.org.uk (cid:27) www.leukres.demon.co.uk/lrfhome. htm 666 18-phone numbers.qxd 10.12.2003 04.35 PM Page 667 Phone numbers and addresses 667 18-phone numbers.qxd 10.12.2003 04.35 PM Page 668 NCRI Leukemia Trials Birmingham Clinical Trials Unit (BCTU) For randomization/entry of AML patients into MRC trials (AML14, AMLHR and AML15 trials). Contacts: BCTU Park Grange 1 Somerset Road Edgbaston Birmingham B15 2RR tel 0121 687 2310 fax 0121 687 2313 bctu@bham.ac.uk or H.Travers@bham.ac.uk M.Nixon@bham.ac .uk or N.H.Hilken@bham.ac.uk (cid:27) www.bctu.bham.ac.uk CTSU For randomization into: CLL4, CLL5, PT1, UKALL XII and childhood ALL CTSU Harkness Building Radcliffe Infirmary Oxford OX2 6HE tel 01865 240972 fax 01865 404849 Randomization@ctsu.ox.ac.uk (cid:27) www.ctsu.ox.ac.uk Northern and Yorkshire Clinical Trials and Research Unit 668 For randomization in: myeloma IX NYCTRU 17 Springfield Mount University of Leeds Leeds LS2 9NG 18-phone numbers.qxd 10.12.2003 04.35 PM Page 669 Phone numbers and addresses tel 0113 233 1476 fax 0113 343 1471 medseb@leeds.ac.uk (cid:27) www.ukmf.org.uk British Society for Hematology BSH 100 White Lion Street London N1 9PF tel/fax 0207 713 0990 (cid:27) www.blackwellpublishing.com/uk/society/bsh/ Medical Research Council 20 Park Crescent London W1N 4AL tel 0207 636 5422 (cid:27) www.mrc.ac.uk 669 18-phone numbers.qxd 10.12.2003 04.35 PM Page 670 This page intentionally left blank 670 19-hematology.qxd 10.12.2003 04.35 PM Page 671 Hematology on-line 19 19-hematology.qxd 10.12.2003 04.35 PM Page 672 Hematology on-line There are many Internet resources available for hematology, including organizations, journa ls, atlases, conference proceedings and newsgroups. The main difficulty with Internet resou rces is that they change so fre- quently and they are constantly being updated and outdated . It would be impossible to list every known site, and we have provided only URLs for those w e think are of most value. General websites Bloodline www.bloodline.net BloodMed www.bloodmed.com Hematologic Diseases (CliniWeb: Oregon Health Sciences University) www.ohsu.edu/cliniweb/C1 5/C15.378.html Hematology Case Studies (UC Davis School of Medicine) medocs.ucdavis.edu/IMD/420A/course.ht m International Medical News www.internationalmedicalnews.com Medical Matrix www.medmatrix.org/Index.asp Meducation www.meducation.com/ Medweb www.medweb.emory.edu/medweb MRC Molecular Hematology Unit www.imm.ox.ac.uk/groups/mrc_molhaem National Library of Medicine www.nlm.nih.gov Oncolink www.oncolink.com Oxford Hemophilia Center www.medicine.ox.ac.uk/ohc/ Oxford Regional Blood Club www.btinternet.com/ phm/BloodClub.html Sickle Hut www.sicklehut.com 672 The Hematology Site www.geocities.com/HotSprings/5340/ Atlases Atlas of Hematology www.medic.bgu.ac.il/mirrors/pathy/Pictures/atoras.html 19-hematology.qxd 10.12.2003 04.35 PM Page 673 Hematology on-line Atlas of Hematology (Nagoya) pathy.med.nagoya-u.ac.jp/atlas/doc/atlas.html Cells of the blood (University of Leicester: Department of Microbiology and Immunology) www -micro.msb.le.ac.uk/MBChB/bloodmap/Blood.html Digital Image Study Sets (UC Davis School of Medicine) medocs.ucdavis.edu/IMD/420A/dib Hematology Image Atlas www.hms.medweb.harvard.edu/HSHeme/AtlasTOC.htm Introduction to Blood Morphology www.hslib.washington.edu/courses/blood/intro.html Journals and books ASH Education Book www.asheducationbook.org Blood www.bloodjournal.org/ Blood Cells, Molecules and Disease www.scripps.edu/bcmd British Journal of Hematology www.blackwellpublishing.com/journals/bjh British Medical Journal bmj.bmjjournals.com Cancer caonline.amcancersoc.org Haematologica www.haematologica.it Lancet www.thelancet.com/ Nature www.nature.com/ New England Journal of Medicine content.nejm.org 673 Science www.sciencemag.org Societies and organizations American Association of Blood Banks (AABB) www.aabb.org 19-hematology.qxd 10.12.2003 04.35 PM Page 674 American Medical Association www.ama-assn.org American Society of Hematology www.hematology.org/ Aplastic Anemia Foundation of America www.medic.uth.tmc.edu/ptnt/00001045.htm Bloodline www.bloodline.net British Blood Transfusion Society (BBTS) www.bbts.org.uk British Committee for Standards in Hematology www.bcshguidelines.com British Society for Hematology www.b-s-h.org.uk CancerBACUP www.cancerbacup.org.uk/ European Bone Marrow Transplant Association www.embt.org Imperial Cancer Research Fund www.cancerresearchuk.org International Histiocytosis Organization (Histiocytosis Association of America) www.histio. org Leukemia Research Fund dspace.dial.pipex.com/lrf-/ Leukemia Society of America www.leukemia.org Medical Research Council www.mrc.ac.uk National Blood Service www.blood.co.uk National Guidelines Clearinghouse www.guidelines.gov National Heart, Lung, and Blood Institute www.nhlbi.nih.gov/ National Institutes of Health www.nih.gov 674 NHS Center for Reviews and Dissemination www.york.ac.uk/inst/crd/ Royal College of Pathologists www.rcpath.org 19-hematology.qxd 10.12.2003 04.35 PM Page 675 Hematology on-line Royal College of Physicians www.rcplondon.ac.uk Royal Society of Medicine www.roysocmed.ac.uk Sickle Cell Information Center Home www.scinfo.org Society for Hematopathology (Dartmouth College) www.dartmouth.edu/ nlevy/wwwx.html The Cochrane Library www.update-software.com/cochrane/ UK NEQAS Schemes www.ukneqas.org.uk Wellcome Trust www.wellcome.ac.uk/ World Federation of Hemophilia www.wfh.org/ Guidelines and trials cancerTrials (National Cancer Institute) www.cancertrials.nci.nih.gov Clinical practice guidelines: hematology (Canadian Medical Association) www.cma.ca/cpgs/hem a.htm Clinical trials: hematology (CenterWatch) www.centerwatch.com Comprehensive Sickle Cell Center at Grady Health System www.scinfo.org National Guideline Clearinghouse (Agency for Health Care Policy and Research (AHCPR)) www.g uidelines.gov 675 19-hematology.qxd 10.12.2003 04.35 PM Page 676 This page intentionally left blank 676 20-Charts%2fnomograms.qxd 10.12.2003 04.35 PM Page 677 Charts and nomograms 20 Karnofsky performance status 678 WHO/ECOG performance status 678 WHO hematological toxicity scale 680 Body surface area nomogram 682 Gentamicin dosage nomogram 684 The Sokal sco re for CML prognostic groups 686 20-Charts%2fnomograms.qxd 10.12.2003 04.35 PM Page 678 Karnofsky performance status Normal, no complaints; no evidence of disease 100% Able to carry on normal activity; minor signs or symptoms of disease 90% Normal activity with effort; some signs or symptoms of disease 80% Cares for self; unable to carry on normal activity or to do active work 70% Requires occasional assistance but is able to care for most of his/her needs 60% Requires considerable assistance and frequent medical care 50% Disabled; requires special care and assistance 40% Severely disabled; hospitalization is indicated although death not imminent 30% Very sick; hospitalization necessary 20% Moribund; fatal processes progressing rapidly 10% Dead 0% WHO/ECOG performance status 0 Fully active; able to carry on all pre-disease performance without restriction. 1 Restricted in physically strenuous activity, but ambulatory and able to carry out work of a light or sedentary nature, e.g. light housework, office work. 2 Ambulatory and capable of all self-care but unable to carry out any work activities; up and about more than 50% of waking hours. 3 Capable of only limited self care, confined to bed or chair more than 50% of waking hours. 4 Completely disabled; cannot carry on any self care; totally confined to bed or chair. Oken, M.M. et al. (1982) Toxicity and response criteria of the Eastern Cooperative Oncology Group Am J Clin Oncol, 5 649. 678 20-Charts%2fnomograms.qxd 10.12.2003 04.35 PM Page 679 Charts and nomograms 679 20-Charts%2fnomograms.qxd 10.12.2003 04.35 PM Page 680 WHO hematological toxicity scale Parameter Grade 0 Grade 1 Grade 2 Grade 3 Grade 4 Hemoglobin (g/dL) \211 11.0 9.5 \200\22310.9 8.0 \200\2239.4 6.5 \200\2237.9 <6.5 Leucocytes (x109/L) \211 4.0 3.0 \200\2233.9 2.0 \200\2232.9 1.0 \200\2231.9 <1.0 Granulocytes (x109/L) \211 2.0 1.5 \200\2231.9 1.0 \200\2231.4 0.5 \200\2230.9 <0.5 Platelets (x109/L) \211 100 75 \200\22399 50 \200\22374 25 \200\22349 <25 Hemorrhage none petechiae mild blood loss gross blood loss debilitating blood loss 680 20-Charts%2fnomograms.qxd 10.12.2003 04.35 PM Page 681 Charts and nomograms 681 20-Charts%2fnomograms.qxd 10.12.2003 04.36 PM Page 682 Body surface area nomogram 682 From Ramrakha, P. & Moore, K. (1997) Oxford Handbook of Acute Medicine (OUP), (with permis- sion). 20-Charts%2fnomograms.qxd 10.12.2003 04.36 PM Page 683 Charts and nomograms 683 20-Charts%2fnomograms.qxd 10.12.2003 04.36 PM Page 684 Gentamicin dosage nomogram 684 From Hope, R.A. et al. (1993) Oxford Handbook of Clinical Medicine, 3E (OUP) (with permissi on). 20-Charts%2fnomograms.qxd 10.12.2003 04.36 PM Page 685 Charts and nomograms 685 20-Charts%2fnomograms.qxd 10.12.2003 04.36 PM Page 686 The Sokal Score for CML prognostic groups Score = Exp[0.0116 (age \200\22343.4) + 0.0345 (spleen size \200\2237.51) + 0.188 ([platelets/700 ]2 \200\2230563) + 0.0887 (blasts \200\2232.1) <0.8 = 0.8 \200\2231.2 >1.2 Low risk Intermediate risk High risk 686 21-normal ranges.qxd 10.12.2003 04.36 PM Page 687 Normal ranges 21 Normal ranges (adults) 688 Hematology 688 Biochemistry 689 Immunology 689 Pediatric normal ranges 690 Full blood count 690 Haemostasis 690 21-normal ranges.qxd 10.12.2003 04.36 PM Page 688 Normal ranges (adults) Hematology (9) (3) (9) (3) (9) (3) Hemoglobin 13.0 \200\22318.0g/dL 11.5 \200\22316.5g/dL 0.40 \200\2230.52 0.36 \200\2230.47 4.5 \200 \2236.5 1012/L 3.8 \200\2235.8 1012/L 77 \200\22395 fL 27.0 \200\22332.0pg 32.0 \200 \22336.0g/dL 4.0 \200\22311.0 109/L 2.0 \200\2237.5 109/L 1.5 \200\2234.5 109/L 0. 04 \200\2230.4 109/L 0.0 \200\2230.1 109/L 0.2 \200\2230.8 109/L 150 \200\223400 109/L 0.5 \200\2232.5% (or 50 \200\223100 109/L) 2 \200\22312 mm/1st hour (Westergren) Haematocrit RCC MCV MCH MCHC WBC Neutrophils Lymphocytes Eosinophils Basophils Monocytes Platelets Reticulo cytes ESR (9) (3) Red cell mass 25 \200\22335mL/kg 20 \200\22330mL/kg Serum B12 Serum folate Red cell folate Serum ferritin 150 \200\223700ng/L 2.0 \200\22311.0 g/L 150 \200\223700 g/L 15 \200\223300 g/L (varies with sex and age) 14 \200\223200 g/L (premenopausal female) 0.8 \200\2231.2 INR 12.0 \200\22314.0s PT 0.8 \200\2231.2 APTT ratio 26.0 \200\22333.5s APT T 2.0 \200\2234.0g/L Fibrinogen 3s of control Thrombin time <250 g/L XDPs D-dimer <500n g/mL Factors II, V, VII, VIII, IX, X, XI, XII 50 \200\223150iu/dL 45 \200\223150iu/dL RiCoF 50 \200\223150iu/dL vWF: Ag 80 \200\223135u/dL Protein C 80 \200\223135u/dL Protein S 80 \200\223120u/dL Antithrombin III 2.12 \200\2234.0 APCR 3 \200\2239min Bleeding time 688 21-normal ranges.qxd 10.12.2003 04.36 PM Page 689 Normal ranges Biochemistry and immunology Serum urea 3.0 \200\2236.5mmol/L 11.5 \200\22316.5g/dL 60 \200\223125 mol/L 135 \200\223145mmol/L 3.5 \200\2235.0mmol/v 32 \200\22350g/L <17 mol/L 100 \200\223300iu/L 2.15 \200\2232.55mmol/L 200 \200\223450iu/L 0.7 \200\2231.5mmol/L 63 \200\22380g/L 0.18 \200\2230.42mmol/L 10 \200 \22346iu/l 14 \200\22333 mol/L 11 \200\22328 mol/L 45 \200\22375 mol/L 5 \200\22342iu/L 5 \200\22342iu/L 9 \200\22324pmol/L 0.35 \200\2235.5mU/L Serum creatinine Serum sodium Serum potassium Serum albumin Serum bilirubin Serum alk phos Serum calcium Serum LDH Serum phosphate Serum total protein Serum urate Serum g-GT Serum ir on (9) (3) Serum TIBC Serum ALT Serum AST Serum free T4 Serum TSH Immunology IgG IgA IgM 5.3 \200\22316.5g/L 0.8 \200\2234.0g/L 0.5 \200\2232.0g/L Complement C3 C4 C1 esterase CH50 0.89 \200\2232.09g/L 0.12 \200\2230.53g/L 0.11 \200\2230.36g/L 80 \200\223120% C-reactive protein <6mg/L Serum b2-microglobulin 1.2 \200\2232.4mg/L CSF proteins IgG Albumin 0.013 \200\2230.035g/l 0.170 \200\2230.238g/L Urine proteins Total protein Albumin (24h) <150mg/24h <20mg/24h 689 21-normal ranges.qxd 10.12.2003 04.36 PM Page 690 Pediatric normal ranges Full blood count Age Hb (g/dL) MCV (fL) Neuts Lymph Platelets 14.9 \200\22323.7 100 \200\223125 Birth 13.4 \200\22319.8 88 \200\223110 2 weeks 2 months 9 .4 \200\22313.0 84 \200\22398 6 months 10.0 \200\22313.0 73 \200\22384 1 year 10.1 \200\223 13.0 70 \200\22382 2 \200\2236 years 11.5 \200\22313.8 72 \200\22387 6 \200\22312 years 11. 1 \200\22314.7 76 \200\22390 Adult 9 12.1 \200\22316.6 77 \200\22392 Adult 3 12.1 \200\2231 5.1 77 \200\22394 2.7 \200\22314.4 1.5 \200\2235.4 0.7 \200\2234.8 1 \200\2236 1 \200\2238 1.5 \200\2238.5 1. 5 \200\2238 1.5 \200\2236 1.5 \200\2236 2 \200\2237.3 2.8 \200\2239.1 3.3 \200\22310.3 3.3 \200\22311.5 3.4 \200\22310.5 1.8 \200 \2238.4 1.5 \200\2235 1.5 \200\2234.5 1.5 \200\2234.5 150 \200\223450 170 \200\223500 210 \200\223650 210 \200\223560 200 \200\223550 210 \200 \223490 170 \200\223450 180 \200\223430 180 \200\223430 Neuts, neutrophils; lymph, lymphocytes and platelets (all 109/L) Haemostasis Parameter Neonate Adult level 150 \200\223400 109/L few sec longer than adult up to 25% increase as adult up to 1 week by 2 \200\2239 months as adult as adult as adult Platelet count Prothrombin time APTT Thrombin time Bleeding time Fibrinogen 2 \200\22310 min 2.0 \200\2234.0g/L Vit K factors Factor II Factor VII Factor IX Factor X 30 \200\22350% adult level 30 \200\22350% adult level 20 \200\22350% adult level 30 \200 \22350% adult level up to 6 months by 1 month up to 6 months up to 6 months Factor V Factor VIII vW factor Factor XI Factor XII Factor XIII as adult Variable: 50 \200\223200% adult level usually raised (up to 3 adult level) 20 \200\22350 % adult level 20 \200\22350% adult level 50 \200\223100% adult level 6 \200\22312 months 3 \200\2236 months 1 month FDP/XDP AT Protein C Protein S up to twice adult level 50 \200\22380% adult level 30 \200\22350% adult level 30 \200\22350 % adult level by 7 days 6 \200\22312 months up to 24 months 3 \200\2236 months 690 Plasminogen 30 \200\22380% adult level 2 weeks 21-normal ranges.qxd 10.12.2003 04.36 PM Page 691 Normal ranges 691 21-normal ranges.qxd 10.12.2003 04.36 PM Page 692 This page intentionally left blank 692 22-OHC2e_index.qxd 10.12.2003 04.36 PM Page 693 Index 5HT3 antagonists 574 32P therapy 252 \200\2233 A- -lipoproteinaemia 114 ABO lineage infidelity 153 molecular analysis 154 prognosis 157 prognostic factors 157 relapse management 157 specific treatment 156 \200\2237 supportive treatment 155 World Health Organ ization classification 151 \200\2232 haemolytic disease of the newborn 441 incompatibility 316 \200\22317 abortion, recurrent 401 acanthocytosis 114 hereditary 97 addresses 664 \200\2239 adenosine deaminase deficiency 408 \200\2239 adrenal disorders 48 a driamycin 594, 596, 620, 622, 624 acquired conditions 114 -immune haemolytic syndromes 68, 69 (doxorubicin), BCNU (carmustine) cyclophosphamide, melphalan (ABCM) 278, 620 megaloblastic anemia 64 neutrophil function disorders 464 -non-immune haemolytic syndromes 68, 69 (doxorubicin), bleomycin, vinblastine, dacarbazine (ABVD) 596, 602 activated protein C resistance 396 acute lymphoblastic leukemia aggregants 370 albumin 425 alkalinisation 560 allergic reactions 585 allogeneic transplants 336 allografts 337 alloimmune thrombocytopenia, neonatal 448 \200\2239 158 \200\22363, 576 etiology 158 -allo and autografts 310 clinical features 159 cytogenetic analysis 159 emerge ncy treatment 161 immunological classification 158 immunophenotyping 158 incidence 158 inve stigations and diagnosis 159 \200\22360 minimal residual disease detection allopurinol 258, 560 amniotic fluid 442 amphotericin 330 amyloidosis (primary systemic) 32, 162 288 \200\22391 prognosis/prognostic factors 162, anemia 278, 456 \200\2237 aplastic 122 \200\2233 autoimmune haemolytic 116 \200\22317 of ch ronic disorders 50 \200\2231 congenital dyserythropoietic 450 \200\2231 dilutional 34 drug related 52 drug-induced haemolytic 108 in endocrine disease 48 in gastrointestinal disease 52 haemolytic of the neonate 434 hereditary 130 iron deficiency 56 \200\2238 in joint disea se 50 \200\2231 large bowel 52 leucoerythroblastic 120 163 relapse management 163 specific treatment 161 \200\2232 supportive treatment 161 acute myeloblastic leukemia (AML) 150 \200\2237 etiology 150 biphenotypic leukaemias 153 clinical features 154 \200\2235 cytochemistry 151 cytogenetic analysis 153 \200\2234 diagnosis 150 emergency treatment 155 immunophenotyping 152 incidence 150 22-OHC2e_index.qxd 10.12.2003 04.36 PM Page 694 anemia (continued) in liver disease 54 megaloblastic 64 microangiopathic haemolytic antiplatelet antibodies 378, 380 antithrombin concentrate 398 III deficiency 397 (MAHA) 112, 438 antiviral neonatal 430 \200\2231 oesophageal 52 pancreas 52 and peripheral blood movie 44 pernicious 60 in pregnancy 34 of prematurity 432 pure sideroblastic 228 refractory 220, 486 in renal d isease 46 sickle cell 72 small bowel 52 unexplained 10 \200\22311 prophylaxis 550 therapy 418 apheresis 584 aplasia acquired red cell 454 \200\2235 congenital red cell 452 \200\2233 pure red cell 126 aplastic crises 73 argatroban 521 arthropathy, chronic 353 aspirin 244, 253, 378 \200\2239 ataxia telangiectasia 410 atypical organisms 556 autohaemolysis test 98 autoimmune disorder s 32, 582 azacytidine 76 azo compounds 496 anagrelide 244, 252 analgesics 258, 538 \200\2239, 569, 585 simple non-opioid 538 aniline dyes 496 ankylostoma 495 Ann Arbor stating classification (Cotswolds modification) 210, 213 antenatal diagnosis 355 anti-arrhythmics 585 antibacterial prophylaxis 550 antibiotics B cells 408, 410 B lineage 158 babesiosis 96 bacteriology 302, 544 basopenia 142 basophilia 142 BEAM (BCNU, etoposide, cytarabine intravenous 552 \200\2233 prophylactic 569 antibodies 69, 441 antiplatelet 378, 380 anticoagulation 402 acquired 366 \200\2238 heparin 588 \200\2239 lupus 400 oral 590 post-pa rtum 404 \200\2235 prophylactic 401, 588 \200\2239 therapeutic 588 (ara-C), melphalan) 610, 612 \200\22313 benzodiazepines 574, 585 beta blockers 585 bilirubin 320 biochemistry 302, 471, 544, 689 bi rd-headed dwarfism 460 Birmingham Clinical Trials Unit 668 bisphosphonates 279 bleeding 51, 360 \200\2231, 588 anti-D prophylaxis 390, 441 antidepressants 585 antiemetics 574, 574 \200\2235 antiepilepti cs 585 antifungal active 365 disorders 344 neonate 448 \200\2239 prolonged after surgery 38 \200\2239 vWF-rel ated 348 \200\22350 see also hemorrhage bleomycin 594, 596, 602 blind loop syndromes 61 prophylaxis 550 therapy 330 \200\2232 anti-infective therapy 230 anti-inflammatory drugs 538 antiphospholipid syndrome 401 694 22-OHC2e_index.qxd 10.12.2003 04.36 PM Page 695 blood