Heme and Onc Flashcards

1
Q

ITP definition

A

Autoimmune disorder where IgG autoantibody binds to PLTs. PLTs cycled through the spleen where they are destroyed

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2
Q

ITP causes/risk factors

A

Drugs: cimetidine, gold products, heparin, quinine, sulfonamides, thiazides

SLE

CLL

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3
Q

ITP presentation and Dx

A

s/s: bleeding, purpura, petechiae, AFEBRILE, No splenomegaly

Dx of exclusion
-low PLT, large PLTs
-ANA r/o SLE
-monoclonal antibody immobilization of platelets antigens (MAIPA) IgG antibodies (not always needed)
-BMBx to r/o other heme malignancies

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4
Q

ITP treatment

A

Start w/ watch and wait unless severe bleeding

-Pred 1-2 mg/kg/day
-HD IVIG (fast response but temporary)
-splenectomy if pred failure
-only transfuse PLT if emergency situation
-WinRho if Rh+ person born to Rh- mom
-chemo if failure w/ pred and splenectomy

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5
Q

HIT definition

A

IgG autoantibody reacts w/ PLT factor 4 on PLT surface causing arterial and venous thrombosis

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6
Q

HIT risk factors

A

Hospitalized pts

Unfractionated heparin (can happen w/ LMWH)

Surgical pts

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7
Q

HIT presentation and Dx

A

s/s: symptoms of thrombus and depend on location

-PLT C serotonin release assay (sens and spec but time consuming)
-Heparin-induced PLT aggregation assay (HIPAA): easier than C
-Heparin PF4 ELISA (definitive Dx)

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8
Q

HIT Tx

A

Stop heparin

Heme consult

Direct thrombin inhibitors if need to anticoag
-Lepirudin (renal adjust)
-Argatroban (liver adjust)

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9
Q

DIC definition

A

Acquired coag disorder from activation of coag and fibrinolytic systems.

Excess thrombin to form r/I fibrinogen consumption, irreversible PLT aggregation and activation of fibrinolytic system

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10
Q

DIC risk factors

A

Infection (GN sepsis)

Malignant neoplasms

Liver disease

Massive trauma/shock

Extensive burns
OB complications like stillborn

ABO incompat. RBC

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11
Q

DIC presentation and Dx

A

Bleeding complications

Acute DIC
-HOTN, tachycardia, edema, bruising, GIB, hematuria, skin necrosis, VTE

Thrombosis
-digital ischemia, dorsal gangrene

Dx of labs and clinical presentation
-D dimer to sens FDP
-Pan Cx for sepsis
-Factor 8 deficiency
-PT/PTT
-CBC

Acute: PLT < 150, PT/PTT 50-70% prolonged, low fibrinogen and factor V/VII

Subacute: PT/PTT wnl, fibrinogen wnl, low PLT, elevated D-dimer

Chronic/compensated: FDP > 45, + D-dimer w > 1:8 dilution

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12
Q

DIC Tx

A

Correct underlying cause
Heme referral

Replace RBC, PLT 30-50k, fibrinogen > 150, FFP q 30 min
-1 unit cryo = 6-8 mg improvement in fibrinogen

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13
Q

IDA definition

A

Iron stores in the body inadequate to preserve homeostasis

Microcytic, hypochromic

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14
Q

IDA risk factors

A

GI/GU blood loss

Uterine bleeding

GI surgery w/o adequate supplementation

Repeated pregnancies

Inadequate dietary intake

LT ASA use

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15
Q

IDA presentation and Dx

A

Fatigue, DOE, dizzy, exercise intolerance, HA, pica, pallor, periph paresthesia, tachy, palps, systolic murmur, red smooth tongue, spoon shaped brittle nails, cheilosis

Low: H&H, MCV, MCHC, RBC, iron, transferrin, retic count

High: TIBC, RDW, PLT (sometimes – non Dx)

GOLD STANDARD IS BMBX if Dx uncertain: low or absent iron stores (Prussian blue negative on report)

Others: stool guiac, endoscopy, clotting studies

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16
Q

IDA Tx

A

Oral replacement – ferrous sulfate 300-325 PO TID before meals
-can use polysaccharide Fe complex to reduce AE

IV iron if GI absorption issues or noncompliance
-Iron dextran IV or IM. HIGH RISK ANAPLYLAXIS
-Ferrlecit IV: lower risk anaphylaxis

Check CBC after 2 weeks for increased retic count

If no response after 6-8 weeks: chec compliance, absorption, GI source or possible incorrect Dx

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17
Q

Thalassemia definition

A

Genetically inherited disorders r/I defective production of globin portion of hemoglobin

Microcytic hypochromic

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18
Q

Thalassemia risk factors

A

Mostly Mediterranean population (Beta). African (Beta), ME, Indian and Asian (Alpha) also

Malarial protective

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19
Q

Thalassemia presentation and Dx

A

s/s: pallor, fatigue, dark urine, poor growth and development. HSM, cards failure/dilation, jaundice, Cooley’s anemia facies (osteo w/ cortical thinning), patho fx of long bones and spine

Low: H&H, MCV, MCHC, Alpha or B HGB chains on hemoglobin electrophoresis

Normal: TIBC, ferritin, RBC

High: indirect bili

Wrights’ stain, osmotic fragility, genetic testing (mostly just prenatal d/t cost)

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20
Q

Thalassemia Tx

A

Mild to mod disease usually just watch and wait

-RBC transfusion (use judiciously)
-Iron chelation therapy (can have iron overload)
-splenectomy if counts low
-BMT for severe

21
Q

Anemia of chronic disease definition

A

Normochromic, normocytic

Inability of reticuloendothelial cells to recirculate iron w/ tissue iron stores normal or increased

Unknown cause – decreased erythrocyte life span

22
Q

Anemia of chronic disease risk factors

A

Renal failure

23
Q

Anemia of chronic disease presentation and Dx

A

Fatigue, wt loss, SOB, syncope, anorexia

Depends on underlying disorder

Low: H&H, iron, TIBC
High: ferritin, WBC, PLT
Normal: MCV, MCHC

24
Q

Anemia of chronic disease Tx

A

ID and treat underlying disease

Recombinant human erythropoietin in CRF and autoimmune

25
Sickle cell definition
Normocytic normochromic Genetically transmitted uncompensated hemolytic anemia w/ shortened RBC survival, increased RBC production is insufficient to balance the increased rate of destruction
26
Sickle cell risk factors
Blacks, Mediterranean, Asian, Caribbean and Central/South America Malarial protective HbSS die earlier than HbSC
27
Sickle cell presentation and Dx
Generalized joint/bone pain, fatigue, Abd pain, NV, swollen joints, priapism, depression. Delayed growth and development, HSM, tachycardia/pnea, fever, cardiomegaly, retinopathy, ulcers to LL -Confirmatory: Hgb electrophoresis -periph smear = sickled cells High: indirect bili, transaminase, alk phos, factor VIII, thrombin, PLTs Low: H&H, plasma protein C and S, serum Cr
28
Sickle cell Tx
Acute management -IV fluids, O2, pain control w/ NSAIDS or narcs -exchange transfusion Hospitalize if -severe pain, sepsis, CNS disorder, pregnancy related complications, priapism, ACS Preventative -folic acid 1 mg daily -LT transfusion if at stroke risk -pneumococcal vaccine -HepB vaccine -annual eye exam and retinal eval -genetic counseling if considering pregnancy -Hydrae to prevent recurrent pain episodes
29
Pernicious anemia definition
Lack of IF in the gut r/I malabsorption of B12 Macrocytic, normochromic anemia
30
Pernicious anemia risk factors
Autosomal recessive (N Europe) Atrophic gastritis Antibodies to IF Other autoimmune
31
Pernicious anemia presentation and Dx
NEURO ARE HALLMARK FINDINGS -tingling hands/feet, depression, unsteady balance, poor memory, trouble concentrating -fatigue, bleeding gums, N w/ wt loss, sore tongue, jaundice, dyspnea, HA -diminished reflex, + Babinski, paresthesia, ataxia, low sense of smell, dementia, HSM, tinnitus, tachycardia/CHF LOW: H&H, retic, PLT/WBC if severe HIGH: ferritin, LDH, folate Serum B12 < 100 = symptomatic 170-240 borderline >240 normal Others: anti-IF and parietal cell antibodies, gastric analysis for achlorhydria GOLD STANDARD: (+) Schillings test
32
Pernicious anemia Tx
B12 100-1,000 mcg SQ daily x 7 days, then weekly x 1 mo -then monthly Monitor for HOK during first week replacement Serum B12 levels Neuro consult if severe s/s
33
Folic acid anemia definition
Macrocytic, normochromic anemia Decreased RBC production and hemoglobin from lack of adequate folic acid
34
Folic acid anemia risk factors
Dietary: alcohol abuse, anorexia, restricted diets, elderly Malabsorption: celiac, short bowel, bypass Inadequate conversion folate  tetrahydrofolate Increased demand/depleted reserves Drug induced: MTX, phenytoin
35
Folic acid anemia presentation and Dx
Fatigue, pallor, mucosa/tongue pain, s/s malnutrition, glossitis, NV, hyperpigmented skin, infertility, orthostasis Less likely to have neuro Serum folate < 4 mcg/L RBC folate < 150 ng/ml (BETTER INDICATOR) MCV > 115
36
Folic acid anemia Tx
Oral folate 1-5 mg/day x 4 months then 1 mg/day Supplement w/ enriched foods Monitor serum folate
37
Hodgkins definition
Defect of cell mediated immunity Contagious spread to nearby nodes Nuclear factor kappa light chain is a transcription factor that is turned on by EBV
38
Hodgkin risk factors
-Male > female; except in nodular sclerosing type -Caucasian -Young (15-34) and older > 50 -Benzene exposure, EBV Can cause ALL and 2nd amyloid
39
Hodgkin presentation and Dx
Painless cervical LAD; can also see supraclavicular and anterior mediastinal -fever, itching, splenomegaly, nephrotic syndrome, jaundice d/t obstruction Core needle bx or excisional (+) Reed-Sternberg -Immunohistochemistry: CD15 and 30 -CBC: neutrophilia, eosinophilia -LHD = high is poor prognosis CXR: mediastinal mass CT scan for staging PET for initial stage
40
Hodgkin Tx
XRT (can cause NHL) Chemo Auto transplant MABs
41
MM definition
Neoplastic proliferation of plasma cells that produce monoclonal immunoglobulins. Normal antibody production 10 g/day B cell neoplasm
42
MM risk factors
-Male gender -African American -Age > 60 -Increased BMI -Agent orange or pesticide exposure -1st degree relative with MM
43
MM presentation and Dx
Hypercalcemia = lethargy, confusion, weakness Elevated Cr – tubular damage, bacterial pyelo, nephro stones, proteinuria wo HTN Elevated ESR (RBC clumping) Heme -normocytic normochromic anemia -Rouleaux formation (clumping rbcs) -increased bleed time -Hyperviscosity syndrome = SOB, HA, fatigue Bone Fx (stimulation of osteoclasts) – lytic lesions -X-ray to eval Radiculopathy – vertebral fx and compression r/I nerve root impingement Neuropathy – amyloid deposit infiltration around nerves Bacterial infections Serum M protein > 3 g/dl Urine and serum Electrophoresis = M spike -Benze Jones protein = light chains in urine Immunofixation = immunoelectrophoresis to determine Kappa vs Lamba and GAM
44
MM Tx
Chemotherapy Targeted therapy with proteasome inhibitors, monoclonal antibodies and BCL2 inhibitors Immunotherapy with immunomodulators and CAR-T cellular therapy Radiation Stem cell transplant Corticosteroids
45
NHL definition
aberant B and T cells inappropriately proliferate in a uncontrolled manner
46
NHL risk factors
-Male gender -Caucasian descent -Age > 60 -EBV -Human T Lymphotropic Virus -HIV/AIDS -Previous XRT or chemotherapy -Organ transplant -Autoimmune disorders
47
NHL presentation and Dx
painless LAD night sweats fever wt loss fatigue bruising/bleeding infections weakness/pain LN biopsy incision or excisional BMBx if needed CT/PET
48
NHL Tx
Watch and wait for slow growing lymphomas Radiation Chemotherapy IV and/or IT. Immunotherapy with immunomodulators and CAR-T cell therapy Targeted therapies with monoclonal antibodies, proteasome inhibitors, kinase inhibitors, BCL-2 inhibitors. Plasmapheresis to remove antibodies Surgery Stem cell transplant