Heme/Onc Flashcards

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1
Q

Acute Lymphocytic Leukemia (ALL)

A

MC in children 3-7yo and MCC childhood cancer

highly responsive to chemotherapy

Sxs

  • lymphadenopathy
  • Bone pain
  • bleeding
  • fever

Dx:

  • bone marrow > 20% blasts

Tx

  • chemotx - Imatinib (+philly) or methotrexate (CNS dz)
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2
Q

Chronic Lymphocytic Leukemia (CLL)

A

Middle age pt - peak at 50yo

MCC leukemia in adults

asymptomatic - incidental finding

Sxs:

  • fatigue
  • lymphadenopathy
  • splenomegaly

Dx:

  • Smudge cells on perip smear, mature lymphocytes

Tx:

chemo or allogenic stem cells

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3
Q

Acute Myeloid Leukemia (AML)

A

MC Adults >50yos

Sxs:

  • gingival hyperplasia
  • splenomegaly
  • fatigue
  • Pancytopenia
  • leukocytosis >100,000

Dx:

  • bone marrow with Auer Rods
  • >20% blasts

Tx

  • Bone marrow transplant + combo chemo
  • Tumor lysis syndrome - 48-72 hrs after tx, incr in uric, K, phostphate and decr in Ca –> treat with allopurinol and IV fluids
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4
Q

Chronic Myeloid Leukemia (CML)

A

>50yo, AA descent has poorer prognosis

Sxs:

  • Usu asymptomatic until blast crisis (acute leukemia)
  • strikingly increased WBC > 100,000
  • Hyperuricemic

Dx:

    • Philadelphia chromosome
  • Incr LDH, uric acid

Tx

  • Imatinib
  • Dasatinib
  • Nilotinib
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5
Q

Hodgkin’s Lymphoma

A

Bimodal - peaks in 20s then 50s

a/w EBV

Sxs:

  • B constitutional sxs - cyclical fever, wt loss, chills, nt swts up/down 2 wks period
  • painless enlarged posterior cervical chain
  • MC supraclavicular LNs (Virchow’s Nodes)

Dx

  • excisional bx - Reed Sternberg Cells (owl eyes)

Tx

  • 5 year prog - excellent
  • Combo chemo
    • Adriamycin
    • Bleomycin
    • Vinblastine
    • Dacarbazine
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6
Q

Non-Hodgkin’s Lymphoma

A

Diffuse large B cells, patients > 50yo

increased IMC risk

Sxs

  • painless peripheral lympadenopathy (axillary, abd, pelvic, inguinal, femora)
  • B const sxs not common
  • Non-contiguous extranodal spread - GI and skin most common

Dx

  • Excisional bx
  • Staging with CT

Tx

  • Variable cure rates with chemo
  • R-CHOP
    • Rituximab
    • Cyclophosphamide
    • Hydroxydaunomycin
    • Oncovine
    • Prednisone
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7
Q

Iron Deficiency Anemia

A

MC anemia in US, MCC menstrual bleeding, GI bleeding

Sxs:

  • fatigue/weakness
  • palps
  • SOB
  • Pallor
  • glossitis
  • angular chelitis
  • pica
  • koilonychia - spoon
  • Splenomegaly

Dx:

  • Low MCV, low MCH (hypochromic)
  • High TIBC, low ferritin (low Fe stores)** < 15ng/mL
    • Females - Hgb < 12/ Hct < 37%
    • Males - <13.5 / < 39%
  • target cells

Tx

  • PO Fe TID 325mg
  • 6 wks to correct
  • 6 mos to restore Fe stores
  • Recheck 13mos x 1 year
  • Vit C for absorption
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8
Q

Sickle Cell Anemia

A

AA, fam hx, B-globin mutation - hemolytic anemia

sxs:

  • hemolysis
  • poor healing
  • pain/swelling hands and feet,
  • Acute chest syndrome

Dx:

  • Hemoglobin electrophoresis - Hemoglobin S
  • Hct 15-30%
  • Sickle RBCs, Howell-Jolly bodies, target cells
  • Regular slit exam for retinopathy

Tx

  • Hydroxyurea
  • vigorous H2O
  • Morphine
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9
Q

Anemia of Chronic Dz

A

Eti - inflammation, cancer, RA,

MCC - chronic renal failure - impaired EPO production

Dx:

  • Normal or microcytic MCV
  • low TIBC and Fe
  • High Ferritin stores (nl to 3x)
  • low Serum EPO

Tx

  • IV EPO
  • Tx underlying dz
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10
Q

Hemolytic Anemia - G6PD

A

hemolytic anemia - premature breakdown of RBCs

Oxidant sensitivity - after infection or medication causing oxidative stress

  • Fava beans
  • sulfonamides
  • antimalarials
  • analgesics - phenacetine - acetylsalicyclic acid

AA male (x lined), S Asian, middle eastern

Dx - Bite cells, Heinz body on smear

Tx - avoid drugs,

acute - blood transfusion

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11
Q

Hemolytic Anemia - Thalassemia

A

Genetic - microcytic hypochromic

only 2 B globin genes instead of 4 - change stability of Hgb = hemolysis

Beta Thalassemia Major

  • Most severe, Mediterrean, FTT
  • HgB A2 and F

Beta Thalassemia trait

  • mild anemia, misdx as Fe def
  • Hbg A2

Alpha Thalassemia

  • Chinese and SE Asians
  • HgB H, hemoglobin Bart’s (Hydrops fetalis), Hgb A

Dx - periph smear - Target cells and basophilic stippling

Tx

  • acute - blood transfusion
  • Asymp - PO Fe repletion
  • Poor prognosis - most die In 20-30s from Infx, liver or heart failure
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12
Q

Folate Deficiency

A

Cofactor in DNA synthesis

eti - etoh or malnourished

contributes to neural tube bifida

Dx:

  • low folate
  • incr MCV - looks like B12 but no neuro sxs
  • Serum folic acid - low
  • Hypersegmented PMNs
  • elevated homocysteine

Tx - PO Folic acid 1-5mg/d

green veggies

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13
Q

Vitamin B 12 deficiency

A

Cobalamine - autoimmune destruction of gastric parietal cells –> atrophic gastritis -> no Intrinsic Factor = no Vit B12 absorption

RF - gastric bypass sx, celiac dz

Sxs:

  • Weakness
  • gums bleeding
  • sore tongue - Glossitis
  • peripheral neuropathy
  • Depression
  • Dementia

Dx:

  • MCV > 100
  • Hypersegmented neutrophils
  • low B12, high homocysteine
  • MMA elevated (not in folate)

Tx

  • lifelong B12 1-2mg PO daily
  • years to replete stores
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14
Q

Aplastic Anemia

A

All three cell lines decreased - LOW WBC, RBC, Plts

Normal MCV and low RTC

eti - chemicals, drugs, radiation (ACE-I, sulfa, phenytoin, chemo, XRT)

Sxs:

  • severe pallor
  • weakness
  • petechiae
  • ecchymosis
  • mucosal bleeding
  • severe infx

Dx: - pancytopenia

BM bx

Tx - stop causative agent, RBC transfusion, BMT

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15
Q

Clotting Factors Disorder

Von Willebrand’s Disease (vWD)

A

MC genetic bleeding disorder, Autosomal Dom

plts can’t adhere to vessel wall = bleeding doesn’t stop as quickly

Sxs/ Dx

  • Hx of bleeding post cut or incr menstrual bleeding
  • petechia, small amt of superficial bleeding
  • PT and PTT nl (extrinsic)
  • low vW Factor
  • low Factor 8

Tx:

  • Desmopressin/DDAVP
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16
Q

Clotting Factors Disorder

Hemophilias A and B

A

hereditary bleeding disorder - two forms

  • Hemophilia A - Factor VIII/8 Deficiency
  • Hemophilia B - Factor IX/9 Deficiency (Christmas Disease)

Sxs:

  • Hemarthrosis - bleeding into joint space
  • Bruising and bleeding easily

Dx:

  • Incr PTT
  • normal PT and plts
  • Low Factor 8 or 9 on assay

Tx - replace factor 8 or 9

17
Q

Hypercoaguable State:

Eti

A

Platelets - too many or overactive

  • TTP
  • HIT
  • HUS
  • HELLP

Vascular injury - plaques, trauma, or burns

Clotting factors - anti clotting factors

  • Protein C
  • Protein S
  • Antithrombin III deficient

Stasis and Surgery

18
Q

Hypercoaguable State

Factor V Leiden

A

MCC hypercoaguable state, Autosomal dominant

amplifies production of thrombin causing more clot formation

Mutated factor V resistant to breakdown by Protein C

  • increased risk of DVT and PE in young pts

Dx

  • activated protein C resistance assay
  • normal PT/PTT

Tx

  • LMWH bridge to warfarin
  • long term antithrombic tx not rec’d
19
Q

Hypercoaguable State

Protein C and S Deficiency

A

Protein C - cleaves/inhibits factor V and VIII

Protein S - inhibits Factor Va and VIIIa, cofactor of Protein C –> reduces thrombin generation

Vitamin K dependent anticoag liver protein - stimulates fibrinolysis and clot lysis (inactivates factor V and VIII)

  • increase DVT and PE risk

Dx

  • Protein C/S functional assay
  • decreased C and S activity levels

Tx

  • Heparin and PO anticoag for life
20
Q

Hypercoaguable State

Antithrombin III Deficiency

A

Recurrent venous thrombosis and PE

Repetitive IU fetal death

AT III - natural anticoag; inhibits thrombin (IIa), and Xa, and other proteases

a/w VTA - 1st ep 20-30

Tx acute thrombotic event w/ high dose IV hep, PO Anticaog indefinitely

21
Q

Hypercoaguable State

Antiphospholipid Antibody Syndrome

A

Autoimmune, often a/w SLE

AutoABs attack platelet membranes, activates endothelial cells + plts - thrombosis

sxs

  • thromboses and recurrent spont abortions

Dx:

  • lupus anticoag
  • anticardiolpin
  • Prolonged PTT

Tx

  • high dose IV hep w/ thrombic event, PO Anticoag indef
22
Q

Polycythemia Vera (primary)

A

Malignancy of BM - increased production of RBCs (can affect WBC and plts)

sxs

  • Pruritis after hot baths
  • Swelling
  • burning pain
  • rubor on hands and feet

Dx:

  • 4 H’s
    • Hypervolemia - incr RBC, Hct > 30
    • Histaminemia - incr histamine d/t release frm mast cells
    • Hyperviscosity - incr Hct = incr viscosity
    • Hyperuricemia
  • Serum EPO lvls reduced
  • ele Vit B12 levels
  • Positive JAK2 tyrosine kinase mutation
  • BM bx

Tx

  • repeated phlebotomy until Hct < 42%
23
Q

Thrombocytopenia

Idiopathic Thrombocytopenic Purpura

A

Autoimmune rx to platelets usu after viral illness (1-2 wks)

Chronic in adults, self limited in children

A/w HIV, HCV, SLE, CLL

Dx of exclusion, +Direct Coombs Test

Tx

  • Children supportive care - IVIG for refractory causes
  • Adults - prednisone
24
Q

Thrombotic Thrombocytopenic Purpura (TTP)

Thrombocytopenia

A

low Platelets + anemia + schistocytes on smear

Acute febrile disease w/ multiorgan thrombosis

Eti - after drugs - quinidine, cyclosporine, pregnancy

inhibition of ADAMTS13

Sxs:

  • Purpura + FAT RN
    • Fever
    • Anemia
    • Thrombocytopenia
    • Renal failure
    • Neurological symptoms

Dx:

  • CBC - low plts
  • Schistocytes (RBC fragments)
  • neg Coombs test

Tx - steroids, plasmapheresis

25
Q

Hemolytic Uremic Syndrome (HUS)

Thrombocytopenia

A

Low platelets + anemia + renal failure (a/w E.coli O157:H7 and diarrheal illness in a child

post infection - E coli or shigella

Children

Severe kidney problems

26
Q

Disseminated Intravascular Coagulation

Thrombocytopenia

A

Abnormal activation of coagulation sequence

  • microthrombi throughout microcirculation
  • consumption of plts, fibrin, coag factors
  • Bleeding and thrombosis occurs simultaneously

Dx:

  • decr Plts
  • Incr bleeding time, PT, PTT
    • D Dimer (fibrin degradation product)

Tx:

  • cryoprecepitate
  • FFP
  • plt transfusion if < 30,000
  • Heparin tx cause