HemeOnc Flashcards by Mani Yavi

osteolytic lesions, osteopeniaanemia, hypercalcemia, renal insufficiency

Multiple Myeloma: CRAB: Calcium, Renal, Anemi, Bone lytic lesions/Back pain

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Man p/w arge anterior mediastinal mass, elevated B-HCG, LDH, AFP

Nonseminomatous germ cell tumor

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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)

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Philadelphia chromosome (9,22 translocation: BCR-ABL)

chronic myelogenous leukemia (CML), or B-ALL (poor prognosis)

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Abn blood smear with basophilia

CML, myeloproliferative disease; rx Imatinib inhibits BCR-ABL tyrosine kinase

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Increased WBS w/ leukocyte ALP

Leukemoid reaction (ie to infection)

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Disorders a/w ALL?

Down’s syndrome, Fanconi’s anemia, and ataxia-telangiectasia

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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

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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

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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

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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

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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

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Chemo for intermediatehigh-grade lymphomas.

CHOP protocol (Cyclophosphamide, doxorubicin [Hydroxy-daunomycin], Oncovin [vincristine], and Prednisone)

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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)

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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)

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Amyloid cardiomyopathy a/w?

Plasma cell dyscrasias such as multiple myeloma (w/ bone pain and hypercalcemia, bence jones proteins)

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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

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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

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Mycosis Fungoides (MF) with erythroderma, lymphadenopathy, and the presence of atypical T lymphocytes in the blood.

Sézary syndrome

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Parathyroid, Pituitary (prolactin or GH) and Pancreatic endocrine tumors (ZE, insulinomas, VIPomas, glucagonomas). P/w kidney stones, stomach ulcers

MEN1’

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Medullary thyroid carcinoma (secretes calcitonin), Pheochromocytoma, Parathyroid hyperplasia (adenoma)

MEN2A (RET gene)

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Medullary thyroid carcinoma (secretes calcitonin), Pheochromocytoma, Oral/intestinal ganglioneuromatosis (mucosal neuromas). a/w marfanoid habitus.

MEN2B

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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)