HemeOnc Flashcards

1
Q

osteolytic lesions, osteopeniaanemia, hypercalcemia, renal insufficiency

A

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

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2
Q

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

A

Nonseminomatous germ cell tumor

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3
Q

Pt p/w Thrombocytopenia (bleeding) with leukocytosis, positive myeloperoxidase test; dx, complications?

A

AML; leukostasis w hyperviscosity retinopathy, TIA/stroke, tumor lysis syndrome, gingival hyperplasia, CNS, infection, DIC (APL/M3)

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4
Q

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

A

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

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5
Q

Abn blood smear with basophilia

A

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

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6
Q

Increased WBS w/ leukocyte ALP

A

Leukemoid reaction (ie to infection)

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7
Q

Disorders a/w ALL?

A

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

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8
Q

64 yom p/w increased neutrophils, metamyelocytes, basophils, splenomegaly, low LAP, dx, rx?

A

CML w/ Philadelphia chromosome (t[9;22], bcr-abl); may transform to AML or ALL (“blast crisis”), rx imatinib

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9
Q

65 yo p/w high WBC, lymphadenopathy, mostly asymptomatic, smudge cells in peripheral blood smear, autoimmune hemolytic anemia, may have splenomegaly; dx/rx?

A

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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10
Q

CRAB: hyperCalcemia, Renal insufficiency, Anemia, Bone lytic lesions/Back pain

A

Multiple Myeloma: Monoclonal M protein spike (IgG, IgA); Ig light chains in urine, Bence Jones protein, Rouleaux formation

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11
Q

sensorimotor deficits, hepatosplenomegaly, amenorrhea, DM, hypothyroidism, adrenal insufficiency, skin hyperpigmentation, digital clubbing, anemia, hyperuricemia, hypercalcemia

A

POEMS syndrome, sx a/w osteosclerotic multiple myeloma: Polyneuropathy, Organomegaly, Endocrinopathy, Multiple myeloma, and Skin changes

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12
Q

P/ p/w lethargy, weakness, post Chemo, hyperuricemia, hyperkalemia, hypocalcemia, hyperphosphatemia, normal cr; dx? Complications?

A

Tumor lysis syndrome, can progress to urate induced kidney failure, hyperkalemia heart arrhythmia; Rx Rasburicase,IVF

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13
Q

Chemo for intermediatehigh-grade lymphomas.

A

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

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14
Q

Indolent course in elder w/ painless “waxing and waning” lymphadenopathy; dx? Rx?

A

Follicular Lymphoma, t(14;18) heavy-chain Ig (14) bcl-2 (18); Rituximab (anti-cd20)

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15
Q

African p/w Jaw lesion, “Starry sky” on smear, may involve Peyer’s patches in Abdomen (p/w intussusception); cyto? dx?

A

Burkitt’s Lymphoma (a/w EBV) w/ t(8;14) c-myc (8) heavy-chain Ig (14)

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16
Q

Amyloid cardiomyopathy a/w?

A

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

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17
Q

Pt p/w hyperviscosity (Raynaud’s phenomenon, visual disturbances, anemia, lymphadenopathy, HSM), platelet dysfunction, dx, rx?

A

Waldenström’s macroglobulinemia, M spike of IgM; rx Plasmapheresis for viscocity, Chemo w/ alkylating plus prednisone

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18
Q

Epidermal cluster of atypical lymphocytes (Pautrier’s abscess); Skin lesions may progress from patch to plaque to cutaneous tumor, and subcutaneous deposits

A

mycosis fungoides (MF), cutaneous T-lymphocyte lymphoma. Early dz (patch or plaque) rx topical nitrogen mustards

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19
Q

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

A

Sézary syndrome

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20
Q

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

A

MEN1’

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21
Q

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

A

MEN2A (RET gene)

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22
Q

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

A

MEN2B

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23
Q

Abdominal distension and a firm, irregular mass B that can cross the midline, increased Homovanillic acid (HVA); dx?

A

Neuroblastoma; a/w N-myc oncogene.

24
Q

Signs of Metastatic Neuroblastoma

A

BM: pancytopenia; periorbital bones: ecchymoses and proptosis; Low-grade fever, fatigue, and failure to thrive

25
Q

Child p/w painless abd mass, HTN respiratory distress, Horner’s syndrome, cord compression, elevated Urinary catecholamines . Dx?

A

Primary neuroblastoma

26
Q

2yof p/w huge, palpable flank mass and/or hematuria; rx?

A

Wilms tumor (nephroblastoma); LOF WT1 or WT2 Ch 11; rx nephrectomy and chemo. If metastasis add rad

27
Q

Syndrome a/w Wilm’s tumor?

A

BW sx and WAGR (Wilms tumor, Aniridia, Genitourinary malformation, and mental Retardation)

28
Q

Li-Fraumeni syndrome

A

Mutation in one copy of the p53 tumor suppressor gene. AD. Predisposes to sarcomas, CNS tumors, and carcinomas, chemo t-AML

29
Q

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?

A

Von Hippel-Lindau syndrome; AD mutated VHL T.S. chr 3, activates angiogenic growth factors

30
Q

Café-au-lait spots, Lisch nodules (pigmented iris hamartomas), neurofibromas in skin, optic gliomas, pheochromocytomas.

A

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.

31
Q

Alcoholic w/ hx pancreatitis, p/w wt loss, abd pain, jaundice, dark urine? Additional PE finding? Dx?

A

Courvisier’s sign: palpable nontender gallbladder via common bile duct obstruction by pancreatic head adenocarcinoma

32
Q

Cancer related Anorexia/cachexia, rx?

A

Progesterone analogue (megesterol acetate) or corticosteroids

33
Q

Painful abdomen, Port wine–colored urine, Polyneuropathy, Psychological disturbances, Precipitated by drugs, alcohol, and starvation

A

Acute intermittent porphyria (5Ps) w increased Porphobilinogen, δ-ALA; rx glucose and heme, which inhibit ALA synthase.

34
Q

Microcytic anemia, GI and kidney dz, headache, memory loss, demyelination, increased Protoporphyrin, δ-ALA

A

Lead poisoning affecting Ferrochelatase and ALA dehydratase

35
Q

Sick pt p/w leukopenia, low retic, anemia, hypotensive, vague hx of thyroid disorder, dx?

A

Agranulocytosis from thyroid meds PTU or Methimazole

36
Q

55 yom p/w pancytopenia, fatigue, bruising, recurrent infection, no lymphadenopathy, HSM, low platelets/Hgb/RBC; dxx,rx?

A

Hairy Cell Leukemia; smear cells w/ cytoplasmic projections, TRAP positive, CD11,CD22; rx Cladribine (2-CDA) adenosine deaminase inhibitor

37
Q

Platelets rx?

A

Stop significant bleeding (thrombocytopenia, qualitative platelet defects)

38
Q

Fresh frozen plasma rx?

A

increases coagulation factors, rx for DIC, cirrhosis, warfarin overdose, exchange transfusion in TTP/HUS

39
Q

Cryoprecipitate (Contains fibrinogen, factor VIII, XIII, vWF, fibronectin) rx?

A

Treat coagulation factor deficiencies involving fibrinogen and factor VIII

40
Q

Most common inherited coagulation disorder (thrombophilia)?

A

Factor V Leiden, mutation impairing degredation by protein C, leads to activation of thrombin

41
Q

thrombocytopenia, microangiopathic hemolytic anemia, mental status changes, fever, renal failure, schistocytes, increased BT; dx,rx?

A

Thrombotic thrombocytopenic purpura, Inhibition or deficiency of ADAMTS 13; rx exchange transfusion and steroids.

42
Q

Cytopenias, fatigue (anemia), hematuria (hemolysis) w/ increased LDH low haptoglobin, thrombocytopenia, hepatic vein thrombosis (or cerebral vein), indirect hyperbilirubinemia; dx?

A

Nocturnal hemolytic anemia w/ lack of CD55/59 on flow cytometry; rx eculizumab, iron/folate supplements

43
Q

Sudden onset dark urine, jaundice, fatigue, anemia, increased LDH and unconjugated bilirubin, spherocytes on smear, recent pneumonia; dx? Etilogy?

A

Immune Hemolytic Anemia; COOMBS positive; via Mycoplasma, Chlamydia, Penicillin, cephaosporin, sulfa, NSAIDs

44
Q

NSAID platelet dysfunction

A

inhibit COX1, decreased thromboxane A2 causes increased blead time

45
Q

Petechia, Echymosis, increaed Bleed time, nomal Pt, slightly increased PTT, normal platelet count, FHx; dx/rx?

A

Von Willebrand dz, AD; Diagnosed by ristocetin cofactor assay (decreased agglutination is diagnostic). Rx: DDAVP (desmopressin)

46
Q

Labs: schistocytes, increased fibrin split products (D-dimers), decreased fibrinogen, decreased factors V and VIII. Dx/etilogy?

A

DIC via Sepsis (gram-negative), Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion (STOP Making New Thrombi).

47
Q

microhemorrhage: mucous membrane bleeding, epistaxis, petechiae, purpura, increased bleeding time, normal PC, PT, PTT, normal ristocetin; dx?

A

Glanzmann w/ GpIIb/IIIa defect (platelet aggregation)

48
Q

microhemorrhage w/ increased bleeding time, decreased PC, normal PT, PTT, nomal blood smear, megakaryocytes on bone marrow biopsy. Dx?

A

ITP Immune thrombocytopenia, anti-GpIIb/IIIa ab, splenic macs consume platelet/antibody complex. May be triggered by viral illness.

49
Q

Pt p/w fatigue, paresthesias, macrocytic anemia w/ hypersegmented neutrophils; dx steps?

A

Likely B12 deficiency, check B12 levels, if decreased, check for anti-intrinsic factor, anti-parietal cell abs, if so then Pernicious anemia

50
Q

Back pain, hemoglobinuria a few days after oxidant stress, RBCs with Heinz bodies and bite cells

A

G6PD def, XR

51
Q

Shcistocytes, increased LDH and indirect bilirubin, low haptoglobin; dx?

A

Intravascular hemolytic anemia (HUS, DIC, TTP, prosthetic valves)

52
Q

triad of microangiopathic anemia (increased LDH), thrombocytopenia, and acute renal failure (BUN:Cr<15)

A

HUS via O157:H7, EHEC Shiga-like toxin; rx supportive w/ plasmapheresis, steroids, dialysis if necessary

53
Q

Diarrhea, cutaneous flushing, asthmatic wheezing, and right-sided valvular disease. Increased 5-HIAA in urine, niacin deficiency (pellagra); dx, rx?

A

Carcinoid tumor (serotonin secreting); rx resection, octreotide

54
Q

Pt p/w fever, HSM, fatigue, post cervical lymph adenopathy, tonsillar exudates, palatal petechia; dx, a/w?

A

Infectious Mononucleaolus, coombs positive cold IgM agglutin Immune Hemolytic Anemia and Thrombocytopenia

55
Q

Pt p/w jaundice, hemolytic anemia, negative Coombs, increased MCHC, splenomegaly, positive murphy’s sign, RUQ abd pain; dx/rx?

A

Hereditary Spherocytosis (AD ankirin/spectrin def), w/ cholecystitis of pigmented gallstones; rx cholecystectomy, folate, splenectomy

56
Q

Thrombocytopenia with thrombosis of arteries and/or veins post heparin use; dx?

A

HIT Heparin induced thrombocytopenia, Heparin + PF4 + IgG + platelet complex activates cytokines (thrombosis) and splenic macrophages (thrombocytopenia)