HemeOnc Flashcards
osteolytic lesions, osteopeniaanemia, hypercalcemia, renal insufficiency
Multiple Myeloma: CRAB: Calcium, Renal, Anemi, Bone lytic lesions/Back pain
Man p/w arge anterior mediastinal mass, elevated B-HCG, LDH, AFP
Nonseminomatous germ cell tumor
Pt p/w Thrombocytopenia (bleeding) with leukocytosis, positive myeloperoxidase test; dx, complications?
AML; leukostasis w hyperviscosity retinopathy, TIA/stroke, tumor lysis syndrome, gingival hyperplasia, CNS, infection, DIC (APL/M3)
Philadelphia chromosome (9,22 translocation: BCR-ABL)
chronic myelogenous leukemia (CML), or B-ALL (poor prognosis)
Abn blood smear with basophilia
CML, myeloproliferative disease; rx Imatinib inhibits BCR-ABL tyrosine kinase
Increased WBS w/ leukocyte ALP
Leukemoid reaction (ie to infection)
Disorders a/w ALL?
Down’s syndrome, Fanconi’s anemia, and ataxia-telangiectasia
64 yom p/w increased neutrophils, metamyelocytes, basophils, splenomegaly, low LAP, dx, rx?
CML w/ Philadelphia chromosome (t[9;22], bcr-abl); may transform to AML or ALL (“blast crisis”), rx imatinib
65 yo p/w high WBC, lymphadenopathy, mostly asymptomatic, smudge cells in peripheral blood smear, autoimmune hemolytic anemia, may have splenomegaly; dx/rx?
CLL chronic lymphocytic leukemia CD20+, CD5+ B-cell neoplasm; rx Rituximab (anti-CD20), chlorambucil, prednisone; Thrombocytopenia poor prognosis; Risk of Richter transformation to large B-cell lymphoma, infections via hypogammaglobulinemia
CRAB: hyperCalcemia, Renal insufficiency, Anemia, Bone lytic lesions/Back pain
Multiple Myeloma: Monoclonal M protein spike (IgG, IgA); Ig light chains in urine, Bence Jones protein, Rouleaux formation
sensorimotor deficits, hepatosplenomegaly, amenorrhea, DM, hypothyroidism, adrenal insufficiency, skin hyperpigmentation, digital clubbing, anemia, hyperuricemia, hypercalcemia
POEMS syndrome, sx a/w osteosclerotic multiple myeloma: Polyneuropathy, Organomegaly, Endocrinopathy, Multiple myeloma, and Skin changes
P/ p/w lethargy, weakness, post Chemo, hyperuricemia, hyperkalemia, hypocalcemia, hyperphosphatemia, normal cr; dx? Complications?
Tumor lysis syndrome, can progress to urate induced kidney failure, hyperkalemia heart arrhythmia; Rx Rasburicase,IVF
Chemo for intermediatehigh-grade lymphomas.
CHOP protocol (Cyclophosphamide, doxorubicin [Hydroxy-daunomycin], Oncovin [vincristine], and Prednisone)
Indolent course in elder w/ painless “waxing and waning” lymphadenopathy; dx? Rx?
Follicular Lymphoma, t(14;18) heavy-chain Ig (14) bcl-2 (18); Rituximab (anti-cd20)
African p/w Jaw lesion, “Starry sky” on smear, may involve Peyer’s patches in Abdomen (p/w intussusception); cyto? dx?
Burkitt’s Lymphoma (a/w EBV) w/ t(8;14) c-myc (8) heavy-chain Ig (14)
Amyloid cardiomyopathy a/w?
Plasma cell dyscrasias such as multiple myeloma (w/ bone pain and hypercalcemia, bence jones proteins)
Pt p/w hyperviscosity (Raynaud’s phenomenon, visual disturbances, anemia, lymphadenopathy, HSM), platelet dysfunction, dx, rx?
Waldenström’s macroglobulinemia, M spike of IgM; rx Plasmapheresis for viscocity, Chemo w/ alkylating plus prednisone
Epidermal cluster of atypical lymphocytes (Pautrier’s abscess); Skin lesions may progress from patch to plaque to cutaneous tumor, and subcutaneous deposits
mycosis fungoides (MF), cutaneous T-lymphocyte lymphoma. Early dz (patch or plaque) rx topical nitrogen mustards
Mycosis Fungoides (MF) with erythroderma, lymphadenopathy, and the presence of atypical T lymphocytes in the blood.
Sézary syndrome
Parathyroid, Pituitary (prolactin or GH) and Pancreatic endocrine tumors (ZE, insulinomas, VIPomas, glucagonomas). P/w kidney stones, stomach ulcers
MEN1’
Medullary thyroid carcinoma (secretes calcitonin), Pheochromocytoma, Parathyroid hyperplasia (adenoma)
MEN2A (RET gene)
Medullary thyroid carcinoma (secretes calcitonin), Pheochromocytoma, Oral/intestinal ganglioneuromatosis (mucosal neuromas). a/w marfanoid habitus.
MEN2B
Abdominal distension and a firm, irregular mass B that can cross the midline, increased Homovanillic acid (HVA); dx?
Neuroblastoma; a/w N-myc oncogene.
Signs of Metastatic Neuroblastoma
BM: pancytopenia; periorbital bones: ecchymoses and proptosis; Low-grade fever, fatigue, and failure to thrive
Child p/w painless abd mass, HTN respiratory distress, Horner’s syndrome, cord compression, elevated Urinary catecholamines . Dx?
Primary neuroblastoma
2yof p/w huge, palpable flank mass and/or hematuria; rx?
Wilms tumor (nephroblastoma); LOF WT1 or WT2 Ch 11; rx nephrectomy and chemo. If metastasis add rad
Syndrome a/w Wilm’s tumor?
BW sx and WAGR (Wilms tumor, Aniridia, Genitourinary malformation, and mental Retardation)
Li-Fraumeni syndrome
Mutation in one copy of the p53 tumor suppressor gene. AD. Predisposes to sarcomas, CNS tumors, and carcinomas, chemo t-AML
Cavernous hemangiomas in skin, mucosa, organs; b/l renal cell cx; hemangioblastoma, high vascularity w hyperchromatic nuclei in retina, brain stem, cerebellum H; pheochromocytomas. Dx?
Von Hippel-Lindau syndrome; AD mutated VHL T.S. chr 3, activates angiogenic growth factors
Café-au-lait spots, Lisch nodules (pigmented iris hamartomas), neurofibromas in skin, optic gliomas, pheochromocytomas.
Neurofibromatosis I; Mutated NF1 tumor suppressor (neurofibromin, a negative regulator of Ras) chr 17. Skin tumors of NF-1 are derived from neural crest cells.
Alcoholic w/ hx pancreatitis, p/w wt loss, abd pain, jaundice, dark urine? Additional PE finding? Dx?
Courvisier’s sign: palpable nontender gallbladder via common bile duct obstruction by pancreatic head adenocarcinoma
Cancer related Anorexia/cachexia, rx?
Progesterone analogue (megesterol acetate) or corticosteroids
Painful abdomen, Port wine–colored urine, Polyneuropathy, Psychological disturbances, Precipitated by drugs, alcohol, and starvation
Acute intermittent porphyria (5Ps) w increased Porphobilinogen, δ-ALA; rx glucose and heme, which inhibit ALA synthase.
Microcytic anemia, GI and kidney dz, headache, memory loss, demyelination, increased Protoporphyrin, δ-ALA
Lead poisoning affecting Ferrochelatase and ALA dehydratase
Sick pt p/w leukopenia, low retic, anemia, hypotensive, vague hx of thyroid disorder, dx?
Agranulocytosis from thyroid meds PTU or Methimazole
55 yom p/w pancytopenia, fatigue, bruising, recurrent infection, no lymphadenopathy, HSM, low platelets/Hgb/RBC; dxx,rx?
Hairy Cell Leukemia; smear cells w/ cytoplasmic projections, TRAP positive, CD11,CD22; rx Cladribine (2-CDA) adenosine deaminase inhibitor
Platelets rx?
Stop significant bleeding (thrombocytopenia, qualitative platelet defects)
Fresh frozen plasma rx?
increases coagulation factors, rx for DIC, cirrhosis, warfarin overdose, exchange transfusion in TTP/HUS
Cryoprecipitate (Contains fibrinogen, factor VIII, XIII, vWF, fibronectin) rx?
Treat coagulation factor deficiencies involving fibrinogen and factor VIII
Most common inherited coagulation disorder (thrombophilia)?
Factor V Leiden, mutation impairing degredation by protein C, leads to activation of thrombin
thrombocytopenia, microangiopathic hemolytic anemia, mental status changes, fever, renal failure, schistocytes, increased BT; dx,rx?
Thrombotic thrombocytopenic purpura, Inhibition or deficiency of ADAMTS 13; rx exchange transfusion and steroids.
Cytopenias, fatigue (anemia), hematuria (hemolysis) w/ increased LDH low haptoglobin, thrombocytopenia, hepatic vein thrombosis (or cerebral vein), indirect hyperbilirubinemia; dx?
Nocturnal hemolytic anemia w/ lack of CD55/59 on flow cytometry; rx eculizumab, iron/folate supplements
Sudden onset dark urine, jaundice, fatigue, anemia, increased LDH and unconjugated bilirubin, spherocytes on smear, recent pneumonia; dx? Etilogy?
Immune Hemolytic Anemia; COOMBS positive; via Mycoplasma, Chlamydia, Penicillin, cephaosporin, sulfa, NSAIDs
NSAID platelet dysfunction
inhibit COX1, decreased thromboxane A2 causes increased blead time
Petechia, Echymosis, increaed Bleed time, nomal Pt, slightly increased PTT, normal platelet count, FHx; dx/rx?
Von Willebrand dz, AD; Diagnosed by ristocetin cofactor assay (decreased agglutination is diagnostic). Rx: DDAVP (desmopressin)
Labs: schistocytes, increased fibrin split products (D-dimers), decreased fibrinogen, decreased factors V and VIII. Dx/etilogy?
DIC via Sepsis (gram-negative), Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion (STOP Making New Thrombi).
microhemorrhage: mucous membrane bleeding, epistaxis, petechiae, purpura, increased bleeding time, normal PC, PT, PTT, normal ristocetin; dx?
Glanzmann w/ GpIIb/IIIa defect (platelet aggregation)
microhemorrhage w/ increased bleeding time, decreased PC, normal PT, PTT, nomal blood smear, megakaryocytes on bone marrow biopsy. Dx?
ITP Immune thrombocytopenia, anti-GpIIb/IIIa ab, splenic macs consume platelet/antibody complex. May be triggered by viral illness.
Pt p/w fatigue, paresthesias, macrocytic anemia w/ hypersegmented neutrophils; dx steps?
Likely B12 deficiency, check B12 levels, if decreased, check for anti-intrinsic factor, anti-parietal cell abs, if so then Pernicious anemia
Back pain, hemoglobinuria a few days after oxidant stress, RBCs with Heinz bodies and bite cells
G6PD def, XR
Shcistocytes, increased LDH and indirect bilirubin, low haptoglobin; dx?
Intravascular hemolytic anemia (HUS, DIC, TTP, prosthetic valves)
triad of microangiopathic anemia (increased LDH), thrombocytopenia, and acute renal failure (BUN:Cr<15)
HUS via O157:H7, EHEC Shiga-like toxin; rx supportive w/ plasmapheresis, steroids, dialysis if necessary
Diarrhea, cutaneous flushing, asthmatic wheezing, and right-sided valvular disease. Increased 5-HIAA in urine, niacin deficiency (pellagra); dx, rx?
Carcinoid tumor (serotonin secreting); rx resection, octreotide
Pt p/w fever, HSM, fatigue, post cervical lymph adenopathy, tonsillar exudates, palatal petechia; dx, a/w?
Infectious Mononucleaolus, coombs positive cold IgM agglutin Immune Hemolytic Anemia and Thrombocytopenia
Pt p/w jaundice, hemolytic anemia, negative Coombs, increased MCHC, splenomegaly, positive murphy’s sign, RUQ abd pain; dx/rx?
Hereditary Spherocytosis (AD ankirin/spectrin def), w/ cholecystitis of pigmented gallstones; rx cholecystectomy, folate, splenectomy
Thrombocytopenia with thrombosis of arteries and/or veins post heparin use; dx?
HIT Heparin induced thrombocytopenia, Heparin + PF4 + IgG + platelet complex activates cytokines (thrombosis) and splenic macrophages (thrombocytopenia)
