Hematological Study Guide

HEMATOLOGICAL SYST Name Cause Thalassemia Minor Genetic producing abnormal Hgb Aplastic Anemia Destruction of stem cells inside bone marrow (radiation, drug, viral infection) Signs/Symptoms Majority asymptomatic. Discovered due to abnormal CBC results revealing microcytic/hypochromic RBCs ***Cooley’s anemia is Beta Thalassemia Major – transfusion dependent anemia (found early in infancy) Diagnostics ³ Hgb ³ Hct ± RBC ³ MCV ³ MCHC Normal RDW Normal to ± ferritin and iron Normal TIBC Treatments Diagnostic test: Hemoglobin electrophoresis Beta-thalassemia (abnormal) IDA, normal (Blood smear: microcytosis, anisocytosis, poikilocytosis) Do not treat thalassemia Genetic counseling prior to pregnancy Bone marrow production slows or stops Fatigue, weakness, pale color, tachycardia and systolic murmur; neutropenia, thrombocytopenia CBC w/ diff Platelet county Bone marrow biopsy (gold standard) Refer to hematologist Pernicious anemia Autoimmune causing destruction of parietal cells; gastrectomy, vegans, alcoholics, bowel disease Gradual onset of paresthesia on feet/hands, pallor, glossitis; numbness/tingling extremities, neuropathy, diff fine motor skills B12/folate levels; B12 levels may be nor mal in 5% of patients with B12 deficiency Antiparietal and anti-intrinsic factor (IF) antibody test + 24 H urine for methylmalonic acid, homocysteine level elevated, peripheral blood smear (macrocytosis) Dietary deficiency may > 5 years to occu r B12 sources: foods of animal origin (meat, poultry, eggs, milk, cheese) B12 via injections or nasal spray (1000mcg per week for 4 weeks then monthly for a lifetime) Oral 1000 -2000mg daily Multivitamin with iron since IDA commonly coexist Folic acid deficiency Inadequate dietary intake causing damage to DNA or RBCs Anemia, tired, fatigue, pallor, reddened sore tongue, glossitis, unexplained weakness, possible tachycardia, palpitations, angina or heart failure. Macrocytic normochromic, peripheral smear – macroovalocytes folate levels <4 Body ’ s supply last 2-3 months Lifestyle changes (dietary – leafy green vegetables, grains, beef, liver) PO folic acid 1-5 mg/day Pregnancy 400 mcg daily Sickle cell anemia Genetic hemolytic anemia; variations in RBC – sickle shaped and insufficient of oxygen carrying capacity Most asymptomatic; extreme anemia, frequent sickling episodes w/ pain, ischemic necrosis of bones or skin, renal/liver dysfunction, priapism, hemolytic episodes, hyposplenism, frequent infections – highly susceptible to infection. If fever give prophylactic PCN up to age 5 CBC Sickledex – screening Electrophoresis – gold standard Mean Hgb 8.0 RBC live 17 vs. 120 days Refer to hematologist, Sickle cell disease is part of newb orn screening; autosomal recessive (if each parent trait – one of four will have disease) prenatal screening available as early as 8-10 weeks via chorionic villus sampling or amniocentesis Concerns If someone comes up positive screen the family At risk ethnic groups Alpha thalassemia: Asian, African ancestry (AAA) Beta thalassemia: African, Mediterranean, Middle Eastern (more common in US) Pancytopenia (leukopenia, anemia, thrombocytopenia) Vitamin B12 deficien cy (pernicious ane mia, gastric disease, infections, antacids and metformin) Nerve damage from chronic B12 deficien cy ± incidence in older women 2-3x ± gastric cancer **All dementia or patients with neuropathy need B12 levels checked Elderly, infants, alcoholics, overcooked vegetables, low citrus intake, malabsorption (gluten). Drugs that interfere: Phenytoin (Dilantin), sulfa, metformin, methotrexate, zidovudine 1 out of 500 African Americans in US have sickle cell anemia ± risk Strep pneumo, H. influenzae due to hyposplenia MUSCULOSKELETAL SYS ORTHOPEDIC MANEUVERS ➢ Drawer Sign – knee instability/torn ligaments ➢ McMurray’s test – 50 with new onset back pain o Suspect spinal fracture (osteoporosis or chronic steroid use); Spinal stenosis (R/O ankylosing spondylitis); Symptoms worsen despite treatment ➢ Loss of posterior tibial reflex (L5) ➢ Loss of Achilles tendon reflex (L5 to S1) ➢ MRI ➢ Treatment depends on etiology o Uncomplicated – NSAIDs, warm packs for muscle spasms; ▪ Muscle relaxants ▪ After acute phase abdominal and core-strengthening ▪ Avoid bedrest due to deconditioning ➢ Spinal stenosis – pain improved when sitting down or leaning over ➢ Bulging disc – feels better when standing ➢ Complications – cauda equina syndrome ➢ Inspect, palpate, reflexes, strength, sensation, gait, straight leg raise = radiculopathy ➢ Waddell’s sign – overreaction during exam BENIGN VARIANTS ➢ Genu recurvatum – hyperextension or backwards curvature of knees ➢ Genu valgum – knock-knees o 35 yrs. are atherosclerotic CAD o Cardiovascular hx should include: ▪ Prior occurrence chest pain/discomfort, syncope ▪ SOB or fatigue with exercise ▪ Hx of heart murmur or elevated BP ▪ Family hx premature death, cardiovascular disease <50 yrs. of age o Cardiovascular physical examination ▪ Precordial auscultation supine and standing ▪ Assessment of femoral artery pulses to rule out coarctation of aorta ▪ BP sitting and standing HTN– avoid beta-adrenergic antagonist because of ability to blunt normal increase in heart rate; avoid diuretic if possible due to ± risk of dehydration Physiological murmur is ok – eval by cardiology Aortic Stenosis – play varies with degree Mitral Stenosis – play varies with degree Mitral regurgitation – mitral valve incompetency – regurgitation from left ventricle to left atria; commonly caused by rheumatic fever, endocarditis, calcific annulus, rheumatic heart disease, some mitral stenosis is usually present – play varies with degree of mitral regurgitation and ventricular chamber enlargement Mitral Valve Prolapse – most common valvular heart problem – common in pectus excavatum; ok to play if absence of symptoms of activity intolerance Hypertrophic cardiomyopathy – disease of cardiac muscle – can lead to sudden cardiac death; mid-systolic murmur that gets louder with standing Those with ICD should be aware of risk Septal/Atrial Defect – if repaired with little residual dysfunction, full sports is allowed; if no repair then degree should be assessed on individual basis; easily fatigued is a sign of atrial septal defect; child presentation can range from entirely well to heart failure A still murmur has a buzzing quality S2 split is occasionally found in uncorrected atrial septal defect Sinus Arrythmia should be encouraged to play Down syndrome needs cervical spine x-ray prior to sports (AAI) RANDOM PEARLES ➢ Pathological fracture – may be related to osteosarcoma or osteoporosis ➢ Stress fracture – overuse injury of bone o Take 6 weeks to heal ➢ DeQuervain’s Tenosynovitis – dorsal thumb pain o Use fingers a lot ➢ Contusion – bone injured but didn’t break ➢ Strain – injury to muscle ➢ Sprain – injury to ligament ➢ Cauda Equida syndrome – compression of spinal cord ➢ Vitamin D is recommended for all adults > 50 ➢ Risk factors for ankle sprain include: o Poor conditioning o Inappropriate footwear o Lack of warm-up period prior to exercising ➢ Mechanism unknown ➢ 4-7x more common in women ➢ More common in patients with autoimmune disease ➢ Widespread body aches, fatigue and cognitive changes ➢ Diagnosis involves identifying multiple tender points throughout body 11/18 o Apply enough pressure so that nailbeds blanch ➢ Physical activity aimed at ± flexibility ➢ Trigger point injection may be helpful ➢ Acetaminophen, NSAIDs, Trazodone, antidepressants, antiepileptics TYPES OF SCANS ▪ ▪ ▪ X-rays – bone fractures/damage, OA, metal, dense objects MRI – gold standard for injuries to cartilage, meniscus, tendons and ligaments o No metal, pacemakers, aneurysm clips CT – costs less than MRI, views structures likes masses, trauma, fractures, bleeding. Forms 3-D picture Hook Test – Biceps Tear – MRI ORTHO TERMINOLOGY ➢ Abduction (varus) – movement away from body ➢ Adduction (valgum) – movement toward the body MUSCULOSKELETAL SYS Name Cause Signs/Symptoms Navicular Fracture (Scaphoid) Fall with outstretched hand Pain on palpation of 3 fingers/hands/wrist/ankles/feet/shoulders. Morning stiffness last longer than 1-hou r (lon ger than DJDs) w/ painful, warm and swollen joints. 3 mon) worse in upper back. Joint pain worse at night; low-grade fever, fatigue, chest pain w/ respiration, long-term stiffness improving with activity, some buttocks pain, loss of ROM, Uveitis/eye irritation/photosensitivity ±ESR, CRP, RF negative, 5 symptoms Minor depression: 2-5 symptoms r/o hypothyroidism, anemia, autoimmune, B12 def. S/S in teens: failing grades, acting out, avoiding socialization, moodiness. ANTIDEPRESSANT DISCONTINUATION ➢ SSRI, SNRI, TCA > 6 months then discontinued. Typically, last < 7 days. Taper over 6 weeks to reduce symptoms. Bothersome, not life threatening. ➢ F – Flu-like symptoms ➢ I – Insomnia ➢ N – Nausea ➢ I – Imbalance (dizziness, difficult coordination) ➢ S – Sensory disturbance ➢ H – Hyperarousal (anxiety/agitation) ➢ H – Headache ➢ ➢ ➢ ➢ ➢ ➢ ➢ ➢ ANXIETY SYMPTOMS W – Worry A– Anxiety T – Tension in muscles C – Concentration difficulty H – Hyperarousal or irritability E – Energy loss R – Restless S – Sleep disturbance ➢ > 3 of the following occurring on most days > 6 months TREATMENT GOALS ANXIETY/DEPRESSION ➢ Remission of symptoms for > 4-5 months aimed at elimination and restorative health o Most often achieved with psychologic, social services and medications o Slowly taper off meds ➢ Consider longer-term therapy if > 2nd episode QUESTIONS TO ASK PRIOR TO RX ➢ What are the most bothersome symptoms? ➢ What meds will help with these symptoms? ANXIOLYTICS ➢ Benzodiazepines (use long acting to avoid abuse) o ³ dose by 25% each week when dc ➢ Buspirone (BuSpar) -low abuse potential o Potentially helpful if taken 3x daily for 6 weeks or longer (useless as prn & for sleep) ➢ ➢ ➢ ➢ MAOI Food and drug interactions Phenelzine (Nardil) and tranylcypromine (Parnate) Do not combine with SSRI, TCA, triptans Elevates BP and risk of stroke when used with fermented foods such as beer, wine, cheese PSYCHOSOCIAL MENTAL HEA ALCOHOL SCREENING ➢ C: Do you feel the need to cut down? ➢ A: Are you annoyed when your friends/spouse comment about your drinking? ➢ G: Do you feel guilty about yourdrinking? ➢ E: Do you need to drink early in the morning? ➢ Positive response to 2/4 is highly suggestive of alcohol abuse ➢ Anyone feeling compelled to drink no matter what the consequences is addicted ANTIPYSCHOTICS SIDE EFFECTS ➢ Pill rolling, shuffling gait, bradykinesia ➢ Extrapyramidal symptoms: o Akinesia - inability to initiate movement o Akathisia - strong inner feeling to move, unable to stay still o Bradykinesia - slowness in movement when initiating activities that require successive steps such as buttoning a shirt o Tardive dyskinesia - involuntary movements of lips (smacking), tongue, face, trunk and extremities ➢ Increased risk of obesity, Type 2 DM, hyperlipidemia, metabolic syndrome and hypothyroidism SUICIDE ➢ Males represent nearly 80% of all completed suicides. ➢ Females attempt suicide 2-3 times more often. ➢ Highest rate of completed suicide is found in elderly males (75+). ➢ Inquiring about suicidal ideation DOES NOT lead to suicide. ➢ Risk Factors: o Older people who have lost a spouse o Plan involving gun or lethal weapon o Hx of attempted suicide o Mental illness: bipolar, depression o Hx of sexual, emotional or physical abuse o Terminal illness, chronic illness, pain o Significant loss o Bipolar is higher risk during depressive episode ➢ 3 important questions: o Are you thinking of hurting yourself? o If yes, do you have a plan? o If yes, do you have the means? ➢ Imminent risk o Immediate psychiatric referral, inpatient hospitalization ➢ Elevated risk but not imminent o Aggressive treatment SEROTONIN RECEPTOR SITES ➢ 5-HT1A – antidepressant ➢ 5-HT1C, 5-HT2C – cerebral spinal fluid production ➢ 5-HT1D – antimigraine effect (anti=defense) ➢ 5-HT2 – agitation, anxiety, panic (2 stressed) ➢ 5-HT3 – nausea, diarrhea (3 GI N/V/D) ALTERNATIVE MEDS FOR DEPRESSION ➢ St. John’s wort o Interacts with SSRI, TCA, MAOI o ³ Digoxin effectiveness o ³ effectiveness of birth control ➢ 5-HTP, L-tryptophan o Interacts with SSRI, MAOI, dextromethorphan, Triptans ➢ Omega-3 fatty acids o No major drug interactions o Highdoses may ± risk of bleeding o Stop 1 week before surgery ➢ Folate and vitamin B6 ➢ Exercise, yoga, massage, guided imagery, acupuncture, light therapy ➢ Kava-Kava and valerian root are both used for anxiety and insomnia. Do not mix with benzos, hypnotics or any CNS depressants ➢ Monoamine System o Serotonin (5-HT) ▪ Well being ▪ Calm ▪ ³ impulsivity ▪ ³ sex drive ▪ ³ aggression ▪ ±appetite o Dopamine ▪ Make you worry ▪ ± vigilance ▪ ± motivation o Norepinephrine ▪ Enhance concentration ▪ Enhance ambition ▪ Enhance productivity PSYCHOSOCIAL MENTAL HEA Name Cause Signs/Symptoms Bipolar Disorder Strong genetic component Type 1: classic manic episode: labile moods, euphoria, talkativeness, flight of ideas, grandiosity and less need for sleep; increased energy/activity, disinhibition. May have psychotic episodes. ± rates of substance abuse Type 2: hypomanic episode – hypomanic disorder, absence of mania and major depression Acute Serotonin Syndrome High levels of serotonin from new drugs or dosage change Acute onset high fever, muscular rigidity, mental status changes, hyperreflexia, uncontrolled shivering, dilated pupils, tachycardia, diarrhea Malignant neuroleptic syndrome Idiopathic Antipsychotics affect dopamine in brain. Sudden onset high fever, muscular rigidity, changes in mental status, fluctuating BP and urinary incontinence Depression 30-40% genetic 60-70% life events Sleep, interest, guilt, energy, concentration, appetite, agitation, suicide, mood (generally, complain of headache, back pain, chronic pain, 5 symptoms Minor: 2-5 sympto ms R/O hypothyroidism, ane mia, autoimmu ne, vitamin B12 CBC, CMP, TS H, Folate, B12, UA, UDS (EKG if giving something to prolong QT interval) GGT – lone elevation sign of abuse AST/ALT ratio – 2:1 ALT more specific for liver AST found liver, cardiac, kidneys, lungs and skeletal muscle MCV > 100 due to folate deficiency If patient is harm to self or others, refer to psychiatric hospital. 0.8% for driving Beer – 12 oz Wine 5 oz Liquor 1.5 oz High dose parenteral vitamins, especially thiamin e (vit. B1) Permanent due to thiamine deficiency can lead to Wernicke Encephalopathy (B1 is sugar for the brain ) PSYCHOSOCIAL MENTAL HEA Name Insomnia Cause Circadian rhythm disorders, psychic issues, mental illness, OSA, RLS, environmental factors, certain medications, idiopathic. 7-8 hours of sleep is the ideal amount. Difficulty falling asleep or waking up during night too early and unable to go back to sleep; daytime drowsiness, fatigue, headache, irritability, difficulty concentrating Diagnostics Primary – not caused by disease or environment Secondary – caused by disease or environment Short term – less than 3 months, pain, stress, grief Chronic – at least 3 months, occurs at least 3 nights per week Delusions and paranoia (disorganized speech and behavior). Hallucinations are common (usually auditory) with loss of ego boundaries; flat and restricted affect with poor social skills, ability to plan and organize day-to-day activities (executive skills) are poor Schizophrenia Anorexia Nervosa Signs/Symptoms Irrational preoccupation with intense fear of gaining weight with distorted perception of body shape and weight Secretive, perfectionistic and self-absorbed. Marked weight loss (BMI <18.5), lanugo on face, back, shoulders, amenorrhea for 3 mon or longer, abdomin al distention with hepatomegaly, cheilosis, oral and gum disease, coarse dry skin, hypotension with bradycardia and hypothermia If purging - loss of dental enamel. Engagein severe food restriction or binge eating and purging. Some use laxatives, vomiting and excessive daily exercise. Onset teens to early 20 ’s • • • DSM V: Inability or refusal to maintain body weight at or above minimum normal weight for age and height Intense fear of gaining weight and becoming fat despite low body weight Disturbance in perception of body weight and shape Treatments Sleep hygiene (regular time, avoid caffeine, Refer to sleep lab (polysomnography is gold standard for sleep apnea) Antihistamines (careful w/ Benadryl in elderly) Benzo hypnotics: Short acting: Alprazolam, Triazolam, Midazolam Intermediate acting: Lorazepam, temazepam Long acting: Diazepam, clonazepam, chlordiazepoxide (Halcion and temazepam are more sedating) Non-Benzo hypnotics: adverse effects include agitation, hallucinations, nightmares, suicidal ideations. Awakening and cannot recall event. Zolpidem (Ambien) – sleep onset or inability to stay asleep Eszopiclone (Lunesta) – sleep onset or inability to stay asleep Ramelteon (Rozerem) – sleep onset insomnia (melatonin agonist) Complementary: Kava-Kava, Valerian root (do not give to children or lactating/pregnant & do not mix with benzos or hypnotics), Melatonin, Chamomile tea, meditation, yoga, Tai-Chi, acupuncture, regular exercise (avoid 4 hours prior to bedtime) Refer to psychiatrist Use of typical antipsychotics can increase sudden death Antipsychotics can prolong QT intervals (EKG needed) and can cause torsade de pointes – clozapine, thioridazine, ziprasidone, haloperidol and others 1st SSRI line Wellbutrin contraindicated– increases seizure threshold Chvostek’s sign – contract of facial muscles when facial nerve is tapped briskly – associated with hypocalcemia which could further develop to tetany Concerns Risk factors: depression, anxiety, GERD, female, illicit drug use, musculoskeletal illness, pain, chronic health problems, shift work, alcohol, caffeine and nicotine. (certain medications – SSRI, cardiac, BP, allergy and steroids can cause insomnia) Onset is usually 16-30’s Onset usually during adolescence Osteopenia/osteoporosis due to prolonged estrogen depletion from amenorrhea and low calcium intake, peripheral edema due to low albumin from low protein intake, cardiac complications – arrhythmia, cardiomyopathy, hypokalemia