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206 Cards in this Set
- Front
- Back
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ANA
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SLE, scleroderma, polymyositis dermatomyositis, RA, sjogren’s, JIA
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anti-dsDNA
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SLE
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anti-smith
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SLE
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antihistone antibody
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drug induced lupus
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antiphospholipid, lupus antigen
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SLE
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anti-ribonucleoproteins:SS-A(Ro),SS-B(La)
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Sjogren’s, SLE, neonatal SLE
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rhemuatoid factor(IgM anti-IgG)
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RA,others
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anti-CCA
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RA(specific)
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anti-Jo-1(anti-tRNA synthase)
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polymyositis, dermatomyositis
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SCL-70(anti-topoisomerase 1)
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diffuse scleroderma
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anti-centromere
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CREST-scleroderma
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c-ANCA(PR3-ANCA)
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Wegener’s granulomatosis
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p-ANCA(anti-myeloperoxidase)
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microscopic polyangiitis, Churg-Strauss
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anti-U1-RNP
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mixed connective tissue disease
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anti-SRP
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polymyositis
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HLA-B27
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Ankylosing spondylitis, Reiters, Psoriatic arthritis
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IgA + an Upper respiratory infection + palpable purpura(legs/ass) + abdominal pain(intussusception)
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Henoch-Schonlein Purpura
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Hepatitis B + constrictions on arteriogram + foot/wrist drop
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polyarteritis nodosa
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trauma + migratory rash , periorbital swelling
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TNF-receptor associated periodic fever (TRAPS) ; Rx = etanercept
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most common autoinflammatory syndrome in children
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Periodic Fever w/ Apthous stomatitis, pharyngitis and cervical adenopathy
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HLA-DR4
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RA
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GI or chlamydia infection
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Reiter’s Syndrome (Reactive arthritis)
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asthma + eosinophils
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Churg-Strauss
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Upper resp destruction + pulmonary nodules + renal disease
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Wegener’s granulomatosis w/ polyangiitis
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strawberry tongue, hand/foot desquamation, persistant fever; lymphadenopathy(>1.5cm unilateral)
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Kawasaki’s disease (risk for coronary aneurysm) Rx = IVIG
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unilateral headache + jaw claudication + blurry vision
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temporal(giant cell) arteritis -->can lead to blindness
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polyarthritis, intermittent fever, rash, lymphadenopathy, HSmegaly, kids
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systemic Juvenile idiopathic arthritis (leading cause of mortality of all JIAs) - only autoinflammatory JIA type
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< 4 joints, progressive bone overgrowth, limb length discrepancy, anterior uveitis
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oligoarticular JIA
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apical lung fibrosis
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ankylosing spondylitis
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diffuse lung fibrosis
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diffuse scleroderma
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Raynaud’s phenomenon
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SLE, CREST syndrome, diffuse scleroderma, MCTD
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anti-Ro --> congenital heart block
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neonatal lupus
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dry mouth, dry eyes, dental caries, parotid enlargement
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Sjogren’s syndrome-->increased risk for B-cell lymphoma
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negatively birefringent (parallel=yellow)
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gout
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Mechanic’s hands
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Dermatomyositis (anti-synthetase syndrome)
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CD8+ destruction of endomysium
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Polymyositis
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CD4+ perifascicular atrophy
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Dermatomyositis
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symmetrical proximal muscle weakness; pharyngeal weakness; Gottron’s papules; heliotrope rash ; malar rash(not nasolabial sparing), shawl rash
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dermatomyositis
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asymmetric jug handle syndesmophytes
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reactive arthritis
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bilateral syndesmophytes(“bamboo spine” )
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ankylosing spondylitis
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comma shaped osteophytes; pencil in cup; sausage digits; DIPs; nail pitting
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psoriatic arthritis(Reiter’s)
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hyperparathyoidism/hemochromatosis
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pseudogout
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innate immunity, unprovoked inflammation
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auto-inflamatory syndromes
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NALP3-NACHT inflammasome mutation; increased IL-1b
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Familial Cold autoinflammatory syndrome, Muckel-Wells syndrome, Neonatal onset Multisystem inflammatory disease(NOMID)
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generalized cold exposure, fever, profuse sweating, extreme thirst
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Familial cold autoinflammatory syndrome
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urticaria-like rash, non pruritic, temp change, hearing loss
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Muckle-Wells syndrome
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non-pruritic rash, CNS-meningitis, low IQ, epiphyseal plate overgrowth,deaf
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NOMID
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pyrin-mutation, fever, inflammation of skin,serosal membranes, joints; neutrophils
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Familial Mediterranean Fever
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↑IgD, trigger = vaccinations, ↑mevalonic acid in urine
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hyper IgD syndrome (a. recessive)
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decrease tubular re-uptake of uric acid
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probenecid(contraindicated for people with kidney stones)
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inhibits xanthine oxidase (indicated for overproduction, renal insufficiency)
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allopurinol
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most commonly used drug for gout
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NSAIDs
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acute gout attacks/prophylaxis
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colichine(administer within 10-12 hrs)
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lupus flair suppression; inhibits TLR-7
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hydroxychloroquine(anti-malarial)
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IL-1 receptor antagonist
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Anakinra
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anti-IL-1 Mab
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canakinumab
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IL-1 false receptor; fusion protein
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rilonacept
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alkylating agents(x-link DNA); cell cycle non specific
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carmustine, cyclophosphamide, ifosfamide, cisplatin, carboplatin
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M phase specific( “M”icrotubules)
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paclitaxel/docetaxel , vincristine/vinblastine
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hemorrhagic cystitis(acrolein)
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cyclophosphamide
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hand-foot syndrome
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5-FU
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nephrotoxic + hearing loss
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cisplatin
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pulmonary fibrosis
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bleomycin(single/double stranded DNA breaks)
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cardiotoxicity(lifetime dose of 550mg)
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doxorubicin(intercalates b/w DNA)
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leucovorin rescue
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methotrexate
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↑ activity with leucovorin
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5-fluorouracil
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acute and delayed diarrhea; topoisomerase inhibitor
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ironotecan “i run to the can”
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hypersensitivy rxn + prevents microtubule dissasembly
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Paclitaxel(must pre-medicate w/ anti-histamines & steroids)
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fluid retention
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Docetaxel(pre-medicate with steroids)
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neuropathy + constipation
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Vincristine (inhibits microtubule polymerization & inhibits pooping)
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cerebellar neurotoxicity
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cytarabine
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all-trans retinoic acid
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acute promyelocytic leukemia
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acneform rash
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erlotinib(EGFR targeted therapy)
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VEGF-Mab
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bevacizumab
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Cardiotoxicity
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Trastuzumab + doxorubicin
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Tumor Lysis Syndrome/ Cytokine Release Syndrome
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Rituximab (Tx for Burkitt’s)
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Fatal anaphylaxis
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cetuximab
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last stage of granulocyte development which can still undergo mitosis
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myelocyte(nucleoli gone at this stage) (3rd stage)
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last stage of erythrocyte development which can still undergo mitosis
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polychromatophilic erythroblast (3rd stage)
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T Cell mediated destruction of hematopoietic BM cells
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Aplastic anemia
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only oncologic emergency
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Neutropenic Fever (ANC<500 and fever >101F). Host infection (pseudomonas). Tx with cefepime.
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epinephrine, stress, exercise, cortisol
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neutrophil demargination
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endomytosis
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megakaryocyte
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CD34+
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hematopoietic stem cell
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erythroid islands
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macrophages
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leading cause of cancer death
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lung cancer
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chemo sensitivity highest
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log-phase
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basophils
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CML
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TdT+(dna polymerase) cell marker
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Acute lymphoblastic leukemia(ALL)
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large mediastinal mass(w/ TDT+)
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T-cell ALL/lymphoma
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Auer rods(myeloperoxidase+)
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Acute myelogenous leukemia(AML)
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t(15;17)
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acute promyelocytic leumia(M3-AML)
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AML precursor
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Myelodysplastic Syndrome (Therapy induced MDS has worse prognosis)
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t(8;14) c-myc activation, ki-67, CD10+; starry sky appearance
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Burkitt’s lymphoma
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t(11;14) upregulation of cyclin D;CD5+
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mantle cell lymphoma
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t(14;18) bcl-2 activation (inhibits apoptosis) ; CD10+
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follicular lymphoma
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massive splenomegaly + dry tap + tartrate-resistant acid phosphatase
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Hairy cell leukemia
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h. pylori , marginal zone, lymphoepithelial lesions
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Mucosa Associated Lymphoid Tissue Lymphoma ( MALT)
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marrow fibrosis + splenomegaly + dry tap - teardrop cells
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myelofibrosis
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HTLV-1+ lytic bone lesions & Rash
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Adult T-cell lymphoma
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CD 19 & 20(common B-cell markers) + CD5, CD23 and monoclonal light chain
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Chronic lymphocytic leukemia
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JAK-2, itchy, hyperviscosity--> dizziness, SOB,hypertension
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polycythemia vera (Rx = phlebotomy)
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p53; cancers <35 y.o
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Li-Fraumeni Syndrome(A. dominant)
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PTEN: breast, thyroid,macrocephaly
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Cowden Syndrome(A. dominant)
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STK11: CRC, GI, breast,pancreatic, prostate
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Peutz-Jehgers( A. dominant)
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MLH/MSH mutations: Colon cancer, endometrial cancer
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Lynch syndrome ( A. dominant)
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RS cells, “B” symptoms, EBV, CD30+ mediastinal lymphadenopathy, CD15+
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Hodgkin’s lymphoma
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RS cells, nodules, collagen
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Nodular Sclerosing HL
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CD30+, CD15+, “owl’s eyes”
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reed sternberg cells
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popcorn cells; CD30-,CD15- , CD20+
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Nodular lymphocyte predominant HL
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hyper IgM → hyperviscosity
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waldenstrom’s macroglobulinemia
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M-spike of IgG(or IgA); lytic bone lesions, bence jones proteins; UPEP or SPEP; Old black man.
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Multiple Myeloma(think CRAB) Rx = autologous stem cell transplant; thalidomide
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defect in lysosomal regulator trafficking gene(LYST) = impaired phagosome-lysosome fusion
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chediak-higashi syndrome( a. recessive)
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lack of NADPH oxidase --> absent respiratory burst, ↓ reactive O2 species
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Chronic granulomatous disease
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hyposegmented neutrophils
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pelger-huet anomaly
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GvHD targets
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Skin, liver, gut (via T cell)
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Cancers very sensitive to radiation
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germ cell tumor, lymphoma
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first stage with absent nucleoli in RBC
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basophilic erythroblast
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Hb: high O2 affinity
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low CO2, high pH, low H+, low BPG
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x-linked; glutathione, hemolytic anemia; resistance to malaria; fava beans; heinz bodies + bite cells
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G6PDH deficiency
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G6PDH variant with shortest T1/2
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Mediterranean
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E6V; hydrophobic pocket; carriers are resistant to malaria
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Sickle cell anemia (a. recessive)
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leading cause of death in sickling diseases
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acute chest syndrome
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SCA + Parvovirus
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Aplastic crisis
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autosplenectomy
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Sickle cell
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skeletal abnormalities
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SCA, thalassemias
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low pH, ↑HgbS, HgbC, hypoxia, RBC lysis, high MCHC, dehydration
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factors contributing to sickling
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hypersegmented neutrophils, glossitis, ↑homocysteine,↑Methyl malonic acid, ileum, vegans, CNS
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B12 deficiency(megaloblastic anemia);
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hypersegmented neutrophils, glossitis, ↑homocysteine, jejunum
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folate deficiency(megaloblastic anemia)
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microcytic hypochromic anemia + atrophic glossitis + esophageal web
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Plummer-Vinson Syndrome
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↓EPO , ↑hepcidin, high serum ferritin, reduced serum iron and TIBC
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anemia of chronic disease
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point mutations
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B-thalassemia(a. recessive)
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gene deletions
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A-thalassemia (a. recessive)
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hypoplastic thenar eminence, microcephaly, pure red cell aplasia
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Diamond Blackfan anemia
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short stature, hypo/hyper pigmented macules, pancytopenia, defect in DNA repair
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Fanconi anemia ( a. recessive)
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Rh antibodies
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IgG(cross placenta)
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Rx: cryoprecipitate
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Indication:DIC, dillutional coagulopathy, factor VIII deficiency
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Rx: washed components(removes most plasma)
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indication: prevent anti-IgA anaphylaxis, prevent allergic rxns
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most common transfusion associated reaction; from cytokines in blood or WBC antibodies in recipient
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febrile non-hemolytic rxn.
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incompatible RBCs; fever, hypotension, shock, organ damage
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acute hemolytic reaction
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<90% pulse ox, pulmonary edema, acute respiratory distress
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Transfusion related acute lung injury (#1 TA death)
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fluid overload , resembles congestive heart failure
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Transfusion associated circulatory overload
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fever, rash, jaundice, diarrhea, pancytopenia
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Transfusion associated GVHD (85-90% mortality)
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Rx: irradiation of transfusion products
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indication: prevent TA-GVHD in immunodeficient patients
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Rx: leukoreduction
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indication: prevent CMV , febrile non-hemolytic rxn.
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cytokine accumulation,bacterial sepsis
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platelets
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target cells
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Fe def, thalassemia
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spherocytes
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HS, WAIHA
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pappenheimer bodies
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Fe overload
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tear drop cells
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myelophthisis
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decreased haptoglobin
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intravascular hemolysis
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proteins: spectrin,ankyrin ; ↑MCHC, spherocytes, + osmotic fragility test
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hereditary spherocytosis
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GPI-anchor/decay accelerating factor(CD55) = complement mediated damage, hemosiderinuria, thrombosis
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paroxysmal nocturnal hemoglobinuria (mutation in hematopoietic stem cell)
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IgG, extravascular, a-methyldopa, spherocytes; CLL, SLE, DAT test + IgG; splenic destruction
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warm Ab hemolytic anemia
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IgM , mycoplasma, mono (M’s), DAT for C3 is +, - for IgG, hepatic destruction
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Cold agglutinin hemolytic anemia
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IgG to P-antigens on RBCs; hemolysin mediated, intravascular
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paroxysmal cold hemoglobinuria
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schistocytes; DIC, TTP, HUS
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microangiopathic hemolytic anemia
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B12 absorption
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ileum
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Folate absorption
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jejunum
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Iron absorption
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duodenum
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PF4, PDGF,VEGF, fibrinogen, Factor V, vWF, plasminogen, Ig’s
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alpha granules
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lack of alpha granules, ↑bleeding time
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gray platelet syndrome
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serotonin, Ca2+, Mg2+, ADP,ATP
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delta(dense) granules
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lack of dense granules, ↑bleeding time
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storage pool disease (auto d.)
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lack of dense granules + albinism
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hermansky-pudlak syndrome (auto r.)
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synthesizes Factor V, VIII, vWF
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megakaryocyte
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platelet receptor for fibrinogen
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Gp IIb/IIIa
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deficiency of Gp IIb/IIIa, ↑bleeding time, only ristoceten aggr. is normal
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Glanzmann’s thrombasthenia(auto r.)
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vWF receptor on platelets(adhesion)
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Gp 1b/IX/V
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deficiency in Gp1b, ↑bleeding time, ristocetin aggr. absent, Giant platelets
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Bernard Soulier Syndrome
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prostacyclin, ADPases, heparin, thrombomulin
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produced in endothelial cells
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most common coagulation disorder
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VonWillebrand’s disease
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inhibition of ADP preventing PY12(Gi) signaling and expression of GpIIb/IIIa on platelet surface
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Plavix or Clopidogrel
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warfarin skin necrosis
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Protein C/S deficiency
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neutralizes Xa, and Xa/VIIa/TF
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tissue factor pathway inhibitor
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inactivates Va, VIIIa; inhibits TAFI activation
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activated Protein C
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SerPin, inhibits IIa & Xa, requires heparin as a cofactor
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antithrombin III
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D-dimers
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DIC
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plasma, PL, Ca2+, activating surface
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aPTT
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plasma + TF + Ca2+
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PT
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plasma + thrombin + calcium
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TT
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inhibits epoxide reductase = no activated Vitamin K
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Warfarin/Coumadin
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promotes release of vWF from endothelial cells
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DDAVP(desmopressin)
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antibodies to heparin-PF4--> activate platelets-->thrombosis with thrombocytopenia
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heparin induced thrombocytopenia
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thrombin inhibitors; indicated for heparin induced thrombocytopenia
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refludin, argatroban
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anti-Xa
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Arixtra, Rivaroxaban
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Low molecular weight heparins
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Lovenox, fragmin ( target Xa)
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GpIIb/IIIa IgG antibodies, follows viral infection, seen in children
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Acute idiopathic thrombocytopenic purpura
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most common cause of thrombocytopenia in adults (GpIIb/IIa Abs)
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Chronic ITP
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ITP & DAT(+) autoimmune hemolytic anemia
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Evans syndrome
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microang. hemolytic anemia
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TTP, HUS
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decreased ADAMTS13(typically acquired autoantibody)
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TTP (Rx= plasmapherisis)
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Pentad: CNS, fever, thrombocytopenia, renal failure, MAHA
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TTP
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familial TTP -congenital ADAMTS13 def
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Upshaw Schulman Syndrome
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DOHLE bodies, macrothrombocytopenia
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May Hegglin anomaly (auto r.)
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microthrombocytopenia, eczema, immunodeficiency
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Wiskott-Aldrich syndrome(x-linked)
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RENAL injury, MAHA, diarrhea, thrombocytopenia, E. coli O157:H7
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Shiga-toxin associated HUS (**do not transfuse platelets)
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uncontrolled activated of the alternate pathway of compliment; ESRD
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Atypical HUS (Rx = Eculizumab - Mab against C5 complement factor)
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absorption of vitamin k
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ileum
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most common hereditary thrombosis syndrome
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Factor V Leiden (cannot be degraded by protein C)
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requires 4 days(2 half lives) to get therapuetic doses
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warfarin ( effects determined by Factor X activity)
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autosomal recessive
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Glanzmann thrombasthenia, Bernard-Soulier syndrome
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X-linked
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hemophilia A, hemophilia B
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Autosomal Dominant
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FV Leiden, Prothrombin 20210A, anithrombin deficiency, Protein C/S deficiency, vW disease
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hemarthrosis
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hemophilias
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leading cause of inpatient hospital deaths
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pulmonary embolisms
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