Presentation on theme: "All that glitters is not gold. William ShakespeareWilliam Shakespeare, The Merchant of Venice Act II - Scene VII - Prince of MoroccoThe Merchant of Venice."— Presentation transcript:
All that glitters is not gold
William ShakespeareWilliam Shakespeare, The Merchant of Venice Act II - Scene VII - Prince of MoroccoThe Merchant of Venice All that glisters is not gold; Often have you heard that told: Many a man his life hath sold But my outside to behold: Gilded tombs do worms enfold. Had you been as wise as bold, Young in limbs, in judgement old Your answer had not been inscroll'd Fare you well, your suit is cold. The first record of this phrase is from French theologian Alain de Lille who said "Do not hold everything gold that shines like gold."
CC/History of present illness 50 yo Caucasian female with vertigo Sudden onset after lunch, never happened before, still symptomatic Worse with movement, associated with nausea room spinning, no tinnitus or hearing loss Better lying still Can not walk or change positions because of symptoms
Recent History Three separate clinic visits in last 9 months for shortness of breath, cough, wheezing attributed to allergies from cats, mold and improved after prednisone. Has established care in last 2 months with pulmonology and cardiology with diagnosis of asthma, chf, and obstructive sleep apnea
Past history Medical: heart failure, asthma, sleep apnea Surgical: hysterectomy performed 4 days ago and discharged home 2 days ago for dysfunctional uterine bleed, fibroids, prior right oophorectomy for cyst and laparotomy for adhesion and bowel obstruction Social: no tobacco, alcohol or illicit drugs, works as housewife, former furniture salesperson and secretary, husband is trucker, 2 healthy children Family: mother – liver cancer, father – Alzheimer, brother – diabetes mellitus
Exam #, bp 120/80, hr 85, resp 16, oxygen saturation 93% on 2L NC, temp 96.7 Neuro: Finger to nose, dysmetric left hand, any head movement reproduces vertigo and nausea HEENT: Nystagmus with leftward gaze Cardiac: S1S2 normal Pulm: clear but diminished on bases Extremities: trace pedal edema.
Labs Wbc 15, hemoglobin 15, platelet 270 with 90% neutrophils, 5% lymph, 5% monocytes, no bands. Sodium 140, potassium 2.8, chloride 98, bicarbonate 26, BUN 7, creatinine 1, glucose 124, calcium 8.2, magnesium 2, total protein 5.1, albumin 2.5, total bilirubin 0.8, AST 34, ALT 50, pro BNP 20443, troponin ranged from , TSH 3.3, UA – 1+ protein Uterine pathology – cervix had some nabothian cysts, endometrium was weakly proliferative, and myometrium showed intravascular leiomyomatosis, adenomyosis and adenomatoid tumor
Old labs Bnp 256 Echo – abnormal LV systolic function with LVEF 40%, mild mitral and aortic regurgitation, concentric LVH, mild elevation of right ventricle systolic pressure Bedside spirometry – FVC 2.23 L or 73% predicted, FEV L or 67% predicted, FEF 25-75% is 1.28 L/s or 46% predicted, FEV1/FVC 75% predicted, no change with bronchodilator Spirometry a month ago – FVC 2.53 L or 87% predicted, FEV L or 87% predicted with ratio 84% and FEF % predicted. Cardiolite – diminished LVEF 35% with global hypokinesis and akinesis of intraventricular septum, no wall motion abnormalities, no reversible defects to suggest myocardial ischemia Aggregate apnea/hypopnea index 5.1 but during REM sleep 19.8
Central MRI brain – showed acute left superior cerebellar distribution infarct. MRA brain – negative
Other investigations Carotid doppler – normal Echo – left ventricular ejection fraction 35-40%, moderate concentric left ventricular hypertrophy, 3+ MR, 1+ TR, grade 3 diastolic dysfunction, echogenic speckling of ventricular myocardium, moderate right and left atrial dilation, restrictive filling of LV CT thorax – no PE, moderate bilateral pleural effusion, right greater than left, heart enlarged, no mediastinal adenopathy, wedge shaped enhancement pattern on spleen, anasarca
Labs Thoracentesis – transudate, 700 mL removed with cytology negative for malignancy Negative hypercoagulability screen TEE showed thrombus in left atrial appendage 1 X 3 cm.
Restrictive Cardiomyopathy Restricted LV filling with rigid LV wall amyloidosis, endomyocardial fibrosis (equatorial Africa or less common in Asia and S America), eosinophilic or Loeffler endomyocarditis, hemochromatosis, glycogen storage disease, treatment from heart transplant, radiation fibrosis
Dilated Cardiomyopathy LV dilation diffusely From alcohol, peripartum, neuromuscular dystrophies, doxorubicin, cocaine, Takotsubo, arrhythmogenic right ventricuar cardiomyopathy/dysplasia, LV noncompaction, other drugs (trastuzumab, cyclophosphamide, imatinib)
Hypertrophic cardiomyopathy Asymmetric LV hypertrophy of interventricular septum with increased outflow tract pressure gradient Risk for SCD in young athletes Ekg shows LVH with widespread deep broad Q waves
Congo red staining of uterine tissue
Congo red staining of stomach tissue
Congo red stain of bone marrow
Further labs IgA normal, IgG 269 (nl ), IgM normal Serum protein electrophoresis shows hypogammaglobulinemia with immunofixation showing small monoclonal lambda Urine protein electrophoresis shows monoclonal band of free lamba light chains Beta 2 microglobulin 2.46 (nl ) Kappa light chain normal, Lambda light chain 13.7 (nl ) 24 hr urine protein 876 Bone marrow aspirate with 12% plasma cells and biopsy showed 10% CD38/CD138 cytoplasmic lambda monoclonal plasma cells identified, normal cytogenetics, multiple myeloma FISH negative for del chromosomes 13q and 17p and FGFR3/IgH and BCL-1/IgH translocations LC MS on peptides from uterine tissue - AL amyloid
Amyloidosis – deposition of amyloid protein fibril AA – serum amyloid A from inflammatory disorders, infections, occasionally neoplasms AL – light chain, monoclonal plasma cell disorder similar to multiple myeloma AH – heavy chain ATTR – transthyretin, senile cardiac amyloidosis Cryopyrin associated periodic syndrome Others – renal, CNS, localized Diagnosis – organ biopsy, subcutaneous fat pad biopsy, rectal mucosa biopsy Labs – Congo red, H and E, kappa/lambda light chain, LC MS, B2M
AL amyloidosis Renal - proteinuria Cardiac - diastolic/systolic dysfunction Nerve - Peripheral neuropathy GI - nausea, vomit, diarrhea, early satiety, macroglossia, splenic involvement Heme - easy bruising, may develop periorbital ecchymosis Skin - nail dystrophy
Treatment Prognosis is worse with multisystem involvement Not candidate for cardiac transplant due to renal and gastrointestinal involvement Not candidate for stem cell transplant due to elevated troponin Melphalan and dexamethasone is good choice but it is toxic to stem cells Recommendation -- bortezomib and dexamethasone for 6 cycles at 3 wk intervals.
References ci.yuma.az.us Miller AL, Falk RH, Levy BD, Loscalzo J. A Heavy Heart. NEJM. 2010;363: Fauci AS, Braunwald E, Kasper DL, et al. Harrisons Principles of Internal Medicine. New York: McGraw-Hill; ot_gold
Thank you ACP, Dr. Yturri Pathology: Drs. Sloop, Ausmus Cardiology: Dr. Galeo Neurology: Dr. Culcea Office: Ms. Hansen