PROFESSIONAL SKILLS IN HAEMATOLOGY BACHELOR OF MEDICINE: YEAR TWO 2000 P Prreeppaarreedd bbyy D Drr IIaann K Keerrrriiddggee D r T o Dr Tonnyy A Azzzzii D Drr S Saannddrraa D Deevveerriiddggee D r A Dr Arrnnoo E Ennnnoo D Drr P Phhiilliipp M Moonnddyy D r M i c h a e l S Dr Michael Seellddoonn D:\308870159.doc 1 CONTENTS I. The Haematological History ................................................................................. 1 II. The Haematological Examination ........................................................................ 3 III. The Abdominal Examination ................................................................................ 5 IV. Examination of Lymph Nodes .............................................................................. 7 V. Differential Diagnosis in Haematological Examination ..................................... 10 1. Causes of Lymphadenopathy ......................................................................... 10 2. Causes of Hepatomegaly ................................................................................ 10 3. Causes of Splenomegaly................................................................................. 11 4. Causes of Hepatosplenomegaly ..................................................................... 12 VI. 2 References/Further Reading ............................................................................... 13 D:\308870159.doc I. THE HAEMATOLOGICAL HISTORY Primary haematological diseases are uncommon, while haematological manifestations secondary to other diseases occur frequently. A wide variety of diseases may produce signs or symptoms of haematological illness. Haematological disease may produce signs or symptoms in essentially every organ system. Both hereditary and environmental factors should be carefully sought and evaluated. Signs and symptoms may reflect manifestation of both bone marrow failure and abnormal myelo- or lymphoproliferation. Presenting Symptoms 1. General Symptoms a. b. c. d. e. f. g. Performance status Weight loss Fever Rigors Night sweats Fatigue, malaise, weakness Anaemia: headache, tiredness, dyspnoea, postural dizziness, chest pain, myalgia h. Bleeding: GIH, menstrual, epistaxis, haematuria, petechiae, bleeding following surgery or trauma. i. Easy bruising: cutaneous, arthropathy, mucosal j. Thrombotic tendency k. Infection l. Bone pain m. Lymphadenopathy 2. Organ Specific Symptoms a. Nervous system - headache (anaemia, bleeding, infection) - paraesthesia (B12 deficiency, amyloidosis, paraneoplastic, chemotherapy) - confusion (anaemia, hypercalcaemia, infection, drugs) - impaired consciousness (haemorrhage, tumour, hyperviscosity, infection) b. Eyes - anaemia, polycythaemia, haemorrhage - diplopia (tumours, infection) D:\308870159.doc 1 c. Ears - tinnitis/roaring (anaemia) d. Naso- and oropharaynx - epistaxis (bleeding diathesis, thrombocytopenia) - anosmia (pernicious anaemia) - sore tongue (iron or B12 deficiency) - bleeding gums - dysphagia (iron deficiency) e. Neck - swelling (lymphoma, SVC obstruction) f. Cardiorespiratory - dyspnoea, angina, CCF (anaemia) - cough (infection, mediastinal lymphadenopathy) - chest pain (rib fractures, PTE, nerve root compression, VZV) - sternal pain/tenderness (leukaemia, myeloma) - dyspnoea, chest pain (pulmonary embolus) - oedema (VTE, hypoalbuminaemia, lymphoedema) g. Gastrointestinal - abdominal fullness (organomegally) - abdominal pain (lymphoma, retroperitoneal bleed, vincristine ileus, haemolysis, sickle cell crisis, PNH, AIP). - constipation (hypercalcaemia, vincristine) h. Genitourinary - priapism (leukaemia, sickle cell) - red urine (haematuria, intravascular haemolysis, myoglobinuria, porphyrinuria) i. Back/Extremities - back pain (haemolysis, bone or spinal infiltration) - arthritis/arthralgia (gout, myeloma, sickle cell, haemochromatosis, collagen vascular diseases, bleeding disorders) - bone pain - leg ulcers (sickle cell, Felty’s, hydroxyurea therapy) j. Skin - jaundice (haemolysis, pernicious anaemia) - pallor (anaemia) - bronze/grey pigment (haemachromatosis, iron-loading) - cyanosis (methaemoglobinaemia, abnormal haemoglobins, polycythaemia) - plethora (polycythaemia) - pruritis (Hodgkins disease, myeloproliferative disorders) - petechiae, purpura, echymoses 2 D:\308870159.doc Past History a. Family History - jaundice/anaemia/gallstones (Hereditary Haemolytic Disorders) - haemophilia (sex-linked) - thalassaemia - sickle cell - von Willebrands (AD or AR) - VTE (thrombophilia) b. Social/Sexual History - HIV risk - racial origin (thalassaemia, haemoglobinopathies) c. Occupational History - carcinogen exposure (aromatic hydrocarbons i.e. petrol derivatives, hair dyes, radiation, pesticides). II. THE HAEMATOLOGICAL EXAMINATION The following is meant as a guide only. Individual clinicians will all have their preferred techniques/processes and there is rarely a single correct examination technique. It is up to each student to learn an examination process that they can justify, that they can feel comfortable with and that they can reliably perform. Positioning is vital. The patient should lie comfortably in bed with one pillow. The legs, abdomen and thorax should be exposed. The patient’s breasts (if female) and genitals should remain covered to respect the patient’s privacy/dignity. 1. General Appearance a. overt symptoms/signs: breathlessness, diaphoresis, level of consciousness, pain b. position: are they in pain, siting, lying etc. c. therapy: venous access, supplemental oxygen, intravenous therapy, cytotoxics d. skin: jaundice, bruising, pigmentation, scratch markers, petechiae, oedema e. wasting: muscle bulk, cachexia f. alopecia: chemotherapy, connective tissue disease 2. Nails a. koilonychia: iron deficiency b. leuconychia: liver disease c. splinter haemorrhages: SBE d. Cyanosis e. Clubbing D:\308870159.doc 3 3. Hands a. gout b. Rheumatoid Arthritis: (associated with Felty’s Syndrome: leg ulcers, neutropenia, splenomegaly, thrombocytopenia, haemolysis) c. Palmar creases: pallor d. Palmar erythema/Dupytrens Contractures e. Hebedens nodes/Janeway lesions: SBE 4. Pulse 5. Forearms a. Petechiae b. Purpura, echymoses c. Palpable purpura: vasculitis, dysglobulinaemia, bacteraemia 6. While moving up the arms a. measure BP (or ask what the BP is). Do not measure BP if known bleeding disorder or thrombocytopenia. b. ask for temperature and urinalysis 7. Epitrochlear lymph nodes 8. Axillary lymph nodes (May also be performed after examination of the head and neck). 9. Face a. Eyes - scleral icterus (jaundice) - conjunctival pallor (more reliable than palmar or nail-bled assessment) b. Mouth (always remove dentures) - gum hypertrophy: phenytoin, monomyelocytic leukaemia, Vitamin C deficiency) - gum bleeding or infection - ulcers: neutropenia, HSV infection - infection: candidiasis, bacterial - atrophic glossitis: megaloblastic anaemia, iron deficiency (17%), malabsorption, alcoholism, enteritis - macroglossia: amyloidosis, trisomy 21, hypothyroidism - petechiae: palate, buccal mucosa - Waldeyer’s ring lymphatic enlargement: Non-Hodgkins Lymphoma - angular stomatitis: drooling, riboflavin deficiency, anaemia, candidiasis - black tongue: nicotine, antibiotics (Aspergillus niger) - teeth: caries, periodontal disease, infection - mucosal pigmentation: lead poisoning, Addisons 10. Lymph Nodes of the Head and Neck (patient sitting up) - can be examined from behind or while standing in front of the patient 4 D:\308870159.doc 11. Bony tenderness - with the patient sitting up tap over the spine with the fist for bony tenderness and press gently over the sternum and clavicles 12. Abdominal examination (see separate section) 13. Inguinal and Femoral Lymph Nodes 14. Legs - purpura or echymoses - palpable purpura - leg ulcers: Felty’s, Hereditary Spherocytosis, sickle cell disease, thalassaemia, polycythaemia, hyperviscosity, peripheral vascular disease - neuropathy: B12 deficiency, chemotherapy, lead poisoning, paraneoplastic, nerve root or spinal compression/involvement 15. Other a. Fundoscopy - hyperviscosity - retinal haemorrhage - papilloedema - bacterial endocarditis: Roth spots - retinal infection: CMV, candiasis, toxoplasmosis III. THE ABDOMINAL EXAMINATION 1. Position - patient lying flat with one pillow. Legs, abdomen and thorax exposed. 2. Inspection Observe abdomen from the foot of the bed – asking the patient to take slow, deep breaths and looking for symmetry and masses. Observe for: - scars - generalised distention (obesity, ascites, tumor, organomegaly, flatus, pregnancy) - prominent veins (Caput Medusae, IVC obstruction, SVC obstruction) - pulsations (AAA, transmitted cardiac pulsation, normal thin person) - visible peristalsis (normal thin persons, intestinal obstruction) - umbilical nodule (Sister Joseph nodule) - periumbilical discolouration (pancreatitis, haemoperitoneum) - striae (weight loss, pregnancy, ascites, Cushings) D:\308870159.doc 5 3. Palpation Reassure patient. Ask them if there are any areas that are painful or tender and examine this area last. Kneel down or sit down to examine the abdomen (in this position your head is level with the patient’s head and your MCP joints are at 180o, permitting greater sensitivity of palpation). Look at the patient’s face Begin in RUQ: a. light palpitation: note presence of tenderness, guarding, rigidity or rebound tenderness b. deep palpation: for any mass describe site, tenderness, size, shape, surface, edge, consistency, pulsatility and movement with inspiration c. liver – ask patient to breath normally through their mouth. With hand parallel to right costal margin or with fingers pointing towards the RCM – start palpation in RIF or 2-3cm below where you have felt the liver on light palpation and advance to RCM by 1-2cm with each expiration. Do not move your hand during inspiration – feel for the liver edge as it moves down with inspiration. - percuss upper border of liver (usually at 61CS MCL) - measure span (normal 10-12.5cm) d. spleen – spleen only palpable when enlarged 1½ -2 times normal size - enlarges inferomedially or inferolaterally therefore examine from RIF and LIF - splenic notch (hilus) may be palpable c. kidneys - bimanual palpation (ballotment) f. fluid thrill 4. Percussion a. Ascites (shifting dullness) - commence in midline with finger pointing towards the feet – the percussion note is tested out towards the flanks on each side - percuss to flank until dullness is reached. The finger should be kept at this point and the patient asked to roll onto their right side. After 30-60 seconds percussion is repeated over this point and shifting dullness is present if the percussion note has become resonant (>80% sensitivity and accuracy) - while the patient is on their right side (right lateral decubitus position) the abdomen may then be examined for splenomegaly using the left hand/forearm to splint the left ribs 6 D:\308870159.doc b. Spleen (unreliable): 40-50% sensitivity - Percuss in the lowest left intercostal space in the midaxillary line during deep inspiration and expiration. Enlarged but nonpalpable spleens will cause dullness of the percussion note during deep inspiration. 5. Auscultation a. b. c. d. e. f. Bowel sounds (present, absent, tinkling, borborygmi) Splenic rub (infarct) Hepatic rub (hepatoma, hepatic metastases, infarcts, inflammation) Hepatic bruit (hepatocellular carcinoma, alcoholic hepatitis, AV malformation) Renal bruit (renal artery stenosis) Venous hum (portal hypertension, 4% of normal people) 6. Other a. Hernial orifices b. Rectal examination c. Femoral and inguinal lymph nodes IV. EXAMINATION OF LYMPH NODES Lymph nodes can be examined sequentially as part of the general/haematological/gastroenterological examination or specifically as an independent examination. If specifically examining for lymphadenopathy begin with the patient sitting on the edge of the bed facing you. During palpation of lymph nodes consider the following features: a. b. c. d. e. f. g. site (palpable nodes may be localised or generalised) size consistency tenderness fixation (fixation suggests non-haemopoeitic malignancy, chronic inflammation or sarcoidosis) overlying skin (tethering suggests carcinoma, inflammation suggests infection) matted/non-matted The palpable lymph node areas are: D:\308870159.doc 7 1. Epitrochlear - use Osler’s method/”politician’s handshake” - usually considered to be pathological if enlarged - enlargement occurs with local infection, lymphoma, syphilis, cocaine abuse 2. Axillary - examine the right axilla by supporting the patient’s right arm on your own right arm and placing your left hand in the patient’s axilla (vice-versa for left axilla) - for descriptive purposes the axillary lymph nodes may be “grouped” in to 3 main groups (anterior, posterior and apical) or five main groups; central apical, lateral, medial (pectoral), infraclavicular (anterior) and subscapular (posterior) 3. Supraclavicular - Valsalva manouver may bring out inapparent nodes - left SCF node (Virchow’s node) is a “sentinel node” for intra-abdominal malignancy 4. Posterior Triangle (Posterior Cervical) 5. Anterior Triangle (Anterior cervical, Jugular chain) 6. Submandibular 7. Jugulodigastric 8. Submental 9. Preauricular 10. Postauricular 11. Occipital 12. Inguinal 13. Femoral 8 D:\308870159.doc 14. Paraumbilical (Sister Joseph’s node) - suggestive of intra-abdominal or pelvic neoplasms The Axillary Lymph Nodes: A: B: C: D: E: Central Lateral Pectoral Infraclavicular Subscapular Lymph Nodes of the Head and Neck 1: 2: 3: 4: 5: 6: 7: 8: 9: D:\308870159.doc 9 Submental Submandibular Jugular Chain Supraclavicular Posterior Triangle Postauricular Preauricular Occipital Jugulodigastric V. DIFFERENTIAL DIAGNOSIS IN HAEMATOLOGICAL EXAMINATION 1. Causes of Lymphadenopathy Generalised Lymphadenopathy: Lymphoma (rubbery and firm) Leukaemia (e.g. chronic lymphocytic leukaemia, acute lymphocytic leukaemia) Infections: viral (e.g. infectious mononucleosis, cytomegalovirus, HIV, measles, rubella); bacterial (e.g. tuberculosis, brucellosis, syphillis), protozoal (e.g. toxoplasmosis) Connective tissue diseases: e.g. rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis Infiltration: e.g. sarcoid, amyloidosis Drugs: e.g. phenytoin (pseudolymphoma) Metabolic: Gaucher’s disease, hyperthyroidism, Niemann-Pick disease Other: scabies, serum sickness, heroin or cocaine abuse Localised Lymphadenopathy: Local acute or chronic infection Metastases from carcinoma or other solid tumours Lymphoma, especially Hodgkin’s Disease 2. Causes of Hepatomegaly Hepatomegaly 1. Massive: Metastases Alcoholic liver disease with fatty infiltration Myeloproliferative disease Right heart failure Hepatocellular cancer 2. Moderate: The above causes Haemochromatosis Haematological disease – e.g. chronic leukaemia, lymphoma, myeloproliferative disorders Fatty liver – e.g. secondary to diabetes mellitus Infiltration – e.g. amyloid 10 D:\308870159.doc 3. Mild: The above causes Hepatitis Biliary obstruction Hydatid disease Human immunodeficiency virus (HIV) infection Firm and Irregular Liver Hepatocullular carcinoma Metastatic disease Cirrhosis Hydatid disease, granuloma (e.g. sarcoid), amyloid, cysts, lipoidoses Tender Liver Hepatitis Rapid liver enlargement – e.g. right heart failure, Budd-Chiari Syndrome (hepatic vein thrombosis) Hepatocellular cancer Hepatic abscess Biliary obstruction/cholangitis 3. Causes of Splenomegaly Massive Common: Chronic myeloid leukaemia Myelofibrosis Rare: Malaria Kala azar Primary lymphoma of spleen Moderate The above causes Portal hypertension Lymphoma Leukaemia (acute or chronic) Thalassaemia Storage diseases e.g. Gaucher’s disease D:\308870159.doc 11 Small The above causes Other myeloproliferative disorders: polycythaemia rubra vera essential thrombocythaemia Haemolytic anaemia Infection: viral (e.g. infectious mononucleosis, hepatitis) bacterial (e.g. infective endocarditis) protozoal (e.g. malaria) Connective tissue diseases: e.g. rheumatoid arthritis systemic lupus erythematosus polyarteritis nodosa Infiltrations: e.g. amyloid, sarcoid Megaloblastic anaemia (rarely) NB: Secondary carcinomatosis is a rare cause of splenomegaly 4. Causes of Hepatosplenomegaly Chronic liver disease with portal hypertension (therefore signs of liver disease/portal hypertension are critical features in assisting with DDx of hepatosplenomegaly) Haematological disease: - lymphopopliferative disorders: lymphoma, ALL - myeloproliferative disorders: AML, CML, etc - other: pernicious anaemia, sickle cell, thalassaemia Infiltration: amyloid, sarcoid Gauchers Infection: viral hepatitis, EBV, CMV Connective tissue disease: SLE Acromegaly Thyrotoxicosis 12 D:\308870159.doc VI. REFERENCES Sackett DL. A primer on the precision and accuracy of the clinical examination. JAMA 1992, 267, 2638. Sapira JD. The Art and Science of Bedside Diagnosis. Williams and Wilkins, Baltimore, 1990. Handin RI, Lux SE, Stossel TP eds. Blood: Principles and Practice of Haematology. J.B. Lippincott Company, Philadelphia, 1995. Talley NJ, O’Connor S. Clinical Examination. Maclennon and Petty, Sydney, 1996 Talley NJ, O’Connor S. Examination Medicine. Maclennon and Petty, Sydney, 1997. D:\308870159.doc 13