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
Q

Sickle cell definition

A

Normocytic normochromic

Genetically transmitted uncompensated hemolytic anemia w/ shortened RBC survival, increased RBC production is insufficient to balance the increased rate of destruction

26
Q

Sickle cell risk factors

A

Blacks, Mediterranean, Asian, Caribbean and Central/South America

Malarial protective

HbSS die earlier than HbSC

27
Q

Sickle cell presentation and Dx

A

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
Q

Sickle cell Tx

A

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
Q

Pernicious anemia definition

A

Lack of IF in the gut r/I malabsorption of B12

Macrocytic, normochromic anemia

30
Q

Pernicious anemia risk factors

A

Autosomal recessive (N Europe)

Atrophic gastritis

Antibodies to IF

Other autoimmune

31
Q

Pernicious anemia presentation and Dx

A

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
Q

Pernicious anemia Tx

A

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
Q

Folic acid anemia definition

A

Macrocytic, normochromic anemia

Decreased RBC production and hemoglobin from lack of adequate folic acid

34
Q

Folic acid anemia risk factors

A

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
Q

Folic acid anemia presentation and Dx

A

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
Q

Folic acid anemia Tx

A

Oral folate 1-5 mg/day x 4 months then 1 mg/day

Supplement w/ enriched foods

Monitor serum folate

37
Q

Hodgkins definition

A

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
Q

Hodgkin risk factors

A

-Male > female; except in nodular sclerosing type
-Caucasian
-Young (15-34) and older > 50
-Benzene exposure, EBV

Can cause ALL and 2nd amyloid

39
Q

Hodgkin presentation and Dx

A

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
Q

Hodgkin Tx

A

XRT (can cause NHL)

Chemo

Auto transplant

MABs

41
Q

MM definition

A

Neoplastic proliferation of plasma cells that produce monoclonal immunoglobulins.

Normal antibody production 10 g/day

B cell neoplasm

42
Q

MM risk factors

A

-Male gender
-African American
-Age > 60
-Increased BMI
-Agent orange or pesticide exposure
-1st degree relative with MM

43
Q

MM presentation and Dx

A

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
Q

MM Tx

A

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
Q

NHL definition

A

aberant B and T cells inappropriately proliferate in a uncontrolled manner

46
Q

NHL risk factors

A

-Male gender
-Caucasian descent
-Age > 60
-EBV
-Human T Lymphotropic Virus
-HIV/AIDS
-Previous XRT or chemotherapy
-Organ transplant
-Autoimmune disorders

47
Q

NHL presentation and Dx

A

painless LAD
night sweats
fever
wt loss
fatigue
bruising/bleeding
infections
weakness/pain

LN biopsy incision or excisional

BMBx if needed

CT/PET

48
Q

NHL Tx

A

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