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12870F with bad shoulder pain, pain on L shin and LE; PEx shows step off on shoulder, WBC nl, Hct low, plt nl, Ca 12.7, BUN and Cr elevated: multiple myelomaNBS: SPEP, MPEP Only transfuse if Hgb < 7! DEXA scan, bisphosphonates only if suspect osteoporosis (most of the question points towards multiple myeloma!)Sx: Calcium: hypercalcemia d/t plasma cell IL-1 secretion (osteoclast activating)Renal: light chains screw up kidneysAnemia: proliferation of cells that take up the bone marrow --| hematopoiesisBone pain: pathologic fracturesLytic lesions "LTR"(also seen in lung CA, thyroid CA, RCC)Vs. blastic (prostate, breast CA)DxRouleaux formation of RBCSPEP, MPEP: monoclonal spikeBone marrow biopsy: >10% plasma cells65M with pancytopenia, peripheral smear shows tear-dropped shape RBC ("dacrocytes"): primary myelofibrosis80F 6mo history of recurrent infections, WBC 87k, Hgb low, recurrent pneumococcal infections: CLLDx: smudge cellHeme malignancy algorithmLL lie at extremes of ageALL: very youngRF: Down syndrome*sx onset + decline can be suddenCLL: older (70-80Y)*classically presents with recurrent infections d/t haphazard B-cell production Middle ageAuer rods: Acute promyelocytic leukemiaSx: DIC d/t auer rods spilling into circulationPathophys: 15:17 translocationTx: all-trans-retinoic acid ATRA (vitA derivative)Bcr-Abl fusion protein, philadelphia chromosome, myelocyte proliferation: chronic myeloid leukemiaPathophys: 9:22 translocation (philadelphia)Tx: imatinib (tyrosine kinase inhibitor)??78M, RBC stuck like coins ("rouleaux"): multiple myeloma78M, pancytopenia, dry tap on bone aspiration: Primary myelofibrosisEssential thrombocythemia (a/w JAK2 mutation)Aplastic anemia 70F, 6mos recurrent infections, WBC 47k, many cells in different stages of aspiration that are MPO+, reduced ALP activity: chronic myeloid leukemiaVs leukemoid reaction: good WBC, elevated ALP (WBC marker)55M, s/p heme malignancy treatment, DIC: acute promyelocytic leukemiaDIC = low Plt, high fibrin degradation product, high PT/PTTTx: ATRA5F 6wks of weight loss, daily fevers, low Hgb/Plt, high WBC, cells +TdT: ALL50M, peripheral smear shows B-cells with bi-lobed nucleus: Hodgkin's lymphomaDx: "Reed-Sternberg cells" CD15+ CD30+Epi: @ young ppl + 40s-60sH/o CML, high fevers, weight loss, diffuse LAD for 3wks: progression of CML --> AML = blast crisis45F with 6mo history of intense pruritus, conjugated hyperbili, intrahepatic bile ducts dilated: primary biliary cholangitisDx: anti-mitochondrial AbTx: UDSA/ursodiolLiver transplant (diphenhydramine for itching)45M with h/o UC, 6mo history of pruritus, dilation of intra + extra hepatic bile ducts: primary sclerosing cholangitisDx: p-ANCA (anti-MPO Ab)Also seen in Churg-Strauss, microscopic polyangiitis, PSCTx:Liver transplant Newborn with conjugated hyperbiliDDxBiliary atresiaCholedochal cyst, Caroli's disease25M, recurrent hemoptysis, sinusitis, occasional hematuria: Wegener's/granulomatosis with polyangiitisPathophys: antigen-Ab complexes = T3HSSx: rapidly progressive glomerulonephritisVs. Goodpasture (no sinusitis) = T2HSTx: steroids, cyclophosphamide25M, new onset asthma, dysmorphic erythrocytes (RBC casts): Churg-Strauss/EGPADx: eosinophilia, p-ANCA35F, intermittent discoloration of fingers, diffuse skin thinking: sclerodermaLimited CREST: anti-centromere Ab, calcinosis, Raynauds, esophageal dysmotility, sclerodactyly, telangiectasiasDiffuse: anti-Scl70, anti-topoisomeraseC/b scleroderma renal crisis |-- ACEi C/I vasospastic drugs i.e. sumatriptan (5HT agonist triggers vasoconstriction) Also c/I for Prinzmetal angina35F, labs nl, PEx nl, up-to-date for vaccines, benign 2/6 diastolic murmur with opening snap at apex: MSTTE required for Systolic >= 3/6DiastolicSymptomatic Vs Influenza vaccine: required in fall V/Q scan: r/o PEStress testc/I pre-existing EKG abnormality (i.e. RBBB), cannot exerciseAlternative: pharmacological stress test via speeding up hearti.e. dobutamine (beta agonist), adenosine analog (ae: bronchoconstriction. c/I recent caffeine, theophylline, asthma) ................
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