Hematology/Oncology Flashcards
Treatment for aplastic anemia
Immunosuppression w/ antithymocyte globulin, cyclosporine, and prednisone
Younger than 50? =» Allogeneic hematopoietic stem cell transplantation
Achrocordon
Skin tag
Patient w/ ESRD who suddenly quits responding to their EPO shots
Check for anti-EPO antibodies
Infectious causes of neutropenia
Typhoid, TB, Brucellosis
HIV, hepatitis, parvovirus, EBV
Histoplasmosis
Malaria, lesihmaniasis
Ehrlichiosis, RMSF
Leukemia that can be associated w/ the clinical features of Felty Syndrome
Large Granular Lymphocyte Leukemia
Patients w/ poor MDS prognostic factors
Severe pancytopenia
Hgb<8, Plt <50, ANC <800
Unique adverse effects of TKIs (like imatinib)
QT prolongation
Fluid retention
Heme/onc side effect of testosterone supplements
Polycythemia; should be monitored at initiation, 3 and 6 months, then annually
Management of polycythemia vera (4 things)
- Phelobotomy w/ goal Hcrt <45%
- Low dose ASA
- Hydroxyurea in patients w/ high risk of thrombosis (pts >60 years old or hx of thrombosis)
- Ruxolitinib (JAK1/2 inhibitor) `
Patients w/ extreme ET are at increased risk for what?
Hemorrhage; thought to cause acquired vWF dysfunction
Levels > 1.5 million
Level >1 million w/ hx of thrombosis or age >60 =» Treat w/ hydroxyurea
Hypereosinophilic syndrome (HES)
Sustained eosinophil counts >1500/uL w/ end-organ damage; could be lungs, skin, heart, GI tract.
Tx: Glucocorticoids
Bad mutation w/ AML but still has normal cytogenetics
FLT3-ITD; needs transplant for cure
AML treatment
Induction therapy: Antracyclines (daunorubicin) + cytarabine =» bone marrow ablation
Consolidation chemotherapy follows
Other TKI used for Philadelphia Chromosom
Dasatinib
ALL Treatment
Variable but includes:
Vincristine + Anthracyclines (doxo/daunorubacin)+ Corticosteroids + L-asparaginase
***Needs CNS prophylaxis as well w/ Oral mercaptopurine and MTX weekly for up to 2 years
Prognosis remains poor due to 11% recurrence of cancer at 30 years; also develop cardiac dysfunction, metabolic syndrome compared to age-matched individuals after treatment
L-asaparinase ADRs
Allergic reactions common
Hypofibrinogenemia
Hypertriglyceridemia
POEMS
Polyneuropathy (sensorimotor) Organomegaly (liver, spleen) Endocrinopathy (adrenals, gonads, trichosis) Monoclonal protein Skin changes (hyperpigmentation)
Initial eval of plasma cell disorders
CBC, CMP B2-microglobulin SPEP Serum/urine immunofixation Serum free light chain testing (should be abnormal k-l ratio) Skeletal survery
Patient w/ MGUS + kidney disease
“Monoclonal gammopathy of renal significance”
Patients must be treated like MM to prevent renal failure
Rough estimate of risk for smoldering MM to become full blown MM
10% year; other poor factors include M protein >3g/dL, BM plasma cells >10%, Serum FLC ration <0.125
Mainstay of MM therapy if possible
Induction chemotherapy followed by:
AUTOLOGOUS BM transplant
Medication to give all patients w/ MM
Bisphosphonates; improves survival and prevents skeletal events
Treatment of Waldenstrom’s Macroglobulinemia
Rituximab + Chemotherapy (cyclophosphamide or bortezomib) + glucocorticoids
Ibrutinib ? A bruton’s TK inhibitor now approved too
Treatment of metastatic basal cell carcinoma
Oral hedgehog inhibitors
Vismodegib, sonidegib
Management of superficial basal cell carcinoma
Topical chemo w/ 5-fluorouracil or imiquimod
Reason every-other-day dosing is better for iron
Every day dosing =» Increased hepcidin production Decreased ferroportin=» decreased absorption
Iron goals in patients on EPO therapy
Ferritin > 100
Transferrin saturation >20%
Test to order for suspected folate deficiency
Homocysteine level; should be elevated
Supplement for patients w/ elevated hemolysis and hereditary spherocytosis
Folate
L-glutamine in sickle cell disease
Precursor of NAD =» decreases number of pain crises
Crizanlizumab
P-selectin inhibitor that reduces SCD pain crises by preventing cellular adhesion
Medications associated with increased number of SCD pain crises
PDE inhibitors like viagra
Moyamoya disease
Irregular perforating vascular networks near stenotic vessels in SCD in the regions corresponding to lenticulostriate and thalamoperforate arteries
=» Cerebral bleeding in 3rd and 4th decades of life
-Can cause early cognitive dysfunction likely 2/2 to chronic anemia
Post CVA management in SCD patients
Monthly transfusion
Post treatment f/up for rectal/colon cancer
6 month interval physical exam and CEA
CT scan every year; colonoscopy after one year, then in 3 years, then q5years
Do this for at least 5 years
*Need rectal sigmoidoscopy q3-6months for rectal cancer patients for 2-3 years after surgery
What do you check all colon cancers for?
Microsatellite instability; if present, do NOT treat w/ adjuvant chemotherapy after surgery
Molecular testing to order on metastatic colon cancer
KRAS, NRAS, BRAF, MSIs
Role of bevacizumab in colon cancer treatment
When given concurrently w/ FOLFOX, therapy is potentiated and a modest increased in PFS is gained
Immunotherapy for KRAS, NRAS, BRAF pos colon cancers
Panitumumab or cetuximab; block EGFR
-May only have activity in left sided colon tumors
Major ADR: Acneiform rash
***Actually good because if you don’t have the rash, it may not be working
Anal cancer chemo regimen
Mitomycin + 5-FU
Chemotherapy for pancreatic cancer
Post-resection ? =» gemcitabine + capecitabine (better in RCTs when combined)
No surgery? =» FOLFIRINOX
Molecular testing for gastroesophageal cancer
HER-2; add to chemotherapy if positive for modest but statistically significant survival benefit
Origins of gastrointestinal NETs and pancreatic NETs
GI = Endocrine cells Pancreatic = Langerhan's cells
Management for poorly differentiated, highly aggressive NETs
Etoposide + Cisplatin
-Similar for lung Small Cell Carcinoma
F/up for indolent GI NET
CT scan q3months; can space to 6 months after approximately one year
Treatment for NET w/ somatostatin receptor
Octreotide
-Can also consider hepatic arterial embolization, radiofrequency ablation, or surgical debulking if symptoms of carinoid are debilitating
Treatment of GIST tumor
Low risk => Surgery alone
High risk =» Surgery + adjuvant imatinib for 3 years
Surgical candidacy eval of non-small cell carcinoma
FEV1 and DLCO measurement
- If one variable is unfavorable, an expected post-surgical level is calculated
- If candidates can get surgery, 5 year survival for Stage I = 60-70% and Stage II = 40% w/ radiation therapy or stereotactic radiation if tumor was small
Sleeve resection (pulmonary)
Performed on lung cancer patients w/ proximal mass; involves resecting lung and part of bronchus
Postsurgical therapy for Stage I-III lung cancers
Cisplatin + one of the followgin: gemcitabine (SCC), docetaxel, pemetrexed (adenocarcinoma), vinorelbine
Resectable T4 non-small cell lung cancer characteristic
No invasion to surrounding vital structures
No mediastinal involvement
No CONTRAlateral spread
-*Occasionally, w/ single contralateral mass can be resected under assumption this is new, independent secondary tumor
In some cases, single ipsilateral mediastinal node positive and nonbulky disease can have surgery
-Basically treated as Stage III disease
Metastatic annotation to the contralateral lung in non-small cell lung cancer
M1a
M1b = farther met
Molecular testing for non-squamous, non-small cell lung cancer
EGFR (w/ transolacations for ALK, ROS)
- If identified, patients need early tx w/ erlotinib
- If w/ TRANSLOCATION, crizotinib
Standard chemo for metastatic lung cancer
Carboplatin (more favorable ADR proflie) + bevacizumab
AND ONE OF THE FOLLOWING:
pemetrexed (if adenocarcinoma)
or
gemcitabine (if squamous cell carcinoma)
New immunotherapy for lung cancer
Pembrolizumab (PD-L1 inhibitor)
-Superior as 1ST LINE THERAPY if expression of PD-L1 >50%
PERC (there are 8 of them)
Age <50 HR <100 O2 Sat <95% No hemoptysis No estrogen use No previous DVT/PE No unilateral leg swelling No surgery or trauma requiring hospitalization within the last 4 weeks
Treatment of an acquired factor VIII deficiency
Activated factor VII
Standard therapy for nonresectable non small cell carcinoma
Platinum + taxol + daily radiation for 6 weeks
Then darvolumab for one year
Indication for pemetrexed in NsCc lung cancer
Adenocarcinoma
Gemcitabine for squamous
Treatment of diarrhea 2/2 to immunotherapy
< 4 episodes : Symptomatic
4-6 episodes/day; Antimotility agents, prednisone 1mg/kg/day
> 6 episodes daily: Admit, stool studies, prednisone 2mg/kg/day, GI for scoping
-Infliximab if all else fails
Patient on immunotherapy w/ headache, dizziness, nausea
Suspect hypophysitis
Test ACTH, TFTs, cort stim, MRI brain
May require steroids and thyroid replacement
Difference between carboplatin and cisplatin for NSCC
Cisplatin = CURATIVE INTENT
Erdheim-Chest disease
Non-Langherhan’s histocytosis malignancy characterized by osseous involvement w/ bilateral symmetric osteosclerosis of the long bones, cardiac imaging/conduction abnormalities (requires MRI imaging of ENTIRE aorta) potential diabetes insipidus, and periocular infiltration manifesting as proptosis
Malignant cells negative for S100 or CD1a (unlike in Langerhans)
Tx: BRAF + =» Vemurafenib
BRAF - =>> INF-a
Cytarabine MOA and ADRs
Pyrimidine analog that inserts itself into DNA and inhibits DNA polymerase
ADRs: BM suppression, fever, myalgia, bone pain, GI toxicity, pancreatitis, tumor lysis
Idarubicin MOA and ADRs
Inserts itself into DNA double helix? also inhibits DNA topoisomerase III inhibiting DNA repairs
ADRs: BM suppression, CARDIOMYOPATHY, hyperuricemia, skin necrosis if extravasation occurs
Differentiation Syndrome
Second severe manifestation of APML that consists of high fever, peripheral edema, pulmonary infiltrates, hypoxia, hypotension, renal/hepatic dysfunction, and serositis; more likely in patients w/ WBC >10,000
Tx: Stop ATRA + ATO; start dexamethasone 10mg IV BID
Diamond-Blackfan anemia
Erythroid aplasia characterized by: Macrocytic anemia in childhood No reticulocyte response Decreased RBC precursors in BM Triphalangeal thumbs Increased risk of malignancy and endocrine dysfnxn
Most common gene mutation w/ Diamond Blackfan anemia
RBS 19; affects ribosome synthesis
*Disease has incomplete penetrance meaning, although it may seem to skip familial generations, it is an inherited condition
Abnormalities associated w/ Diamond-Blackfan other than triphalangeal thumbs
High arched or cleft palate Flat nose bridge Hypertelorism Cardiac/renal abnormalities ***Cardiac and renal imaging required in workup
Therapy for Diamond-Blackfan anemia
Steroids at 2mg/kg/day versus Leukoreduced RBC transfusions
Ivosidenib
IDH-1 inhibitor; this leads to increased levels in cancer cells causing decreased blast production and increased hematopoetic differentiation
Indications: Treatment of AML in patients >75 years of age w/ comorbidities excluding them from chemotherapy OR refractory AML
ADRs: DIFFERENTIATION SYNDROME, Guillan-Barre, QT prolongation, edema, transaminitis,
Low-risk management therapies of MDS
Anemia: EPO for 3 months if EPO levels <500
Thrombocytopenia: Plt <20000 or <50 w/ bleeding =»TPO-RA (avoid if >5% blasts in marrow)
5q deletion: Linalidomide
Ringed Sideroblasts: Luspatercept
IDH: Ivodesenib
Immunosuppressive therapy for MDS
Azacitidine or Decitabine: DNA methylating agents as they INHIBIT DNA methyltransferase
Neither one superior to the other
Fostamatinib
Therapy for chronic ITP resistant to rituxan, ivig, and splenectomy
Acts as a small molecule spleen tyrosine kinase inhibitor affecting the differentiation and survival of antibodies via igG Fc receptor signaling
Enzalutamide
Medication for castration resistant prostrate cancer associated with ischemic heart disease, PRESS, lower seizure threshold
Stauffer syndrome
Hepatic dysfunction in the absence of liver Mets with RCC
RCC also can cause anemia, thrombocytosis, hypercalcemia, AA anylodosis, and polymyalgia rheumatica
Stage IV breast cancer therapy that recurs after previous adjuvant aromatase therapy
Letrozole + Palbociclib
-Cdk4 and Cdk 6 inhibitor
Abiraterone
CYP17-inhibitor Medication used in metastatic castrate-resistant prostate cancer; blocks androgen synthesis in tumor tissues, tested, and adrenal glands. *must administer with prednisone due to adrenal suppression
Muir-Torre Syndrome
AD inherited MMR mutation in genes of MLH1- MSH-2, or MSH-6; same genes as HNPCC
Associated w/ one sebaceous gland malignancy and one visceral malignancy
Treatment for metastatic melanoma that has progressed on PDL-1 inhibitor therapy
BRAF + MEK inhibitor therapy
MEKs include: , Trametinib, Cobemitinib
-Potentially reduced toxicity of BRAF inhibitors when use in combination
Complete Thrombophilia Lab Test Profile
Activated protein C (APC) resistance (FVL test) Prothrombin G20210A genetic teset Lupus anticoagulant anticardiolipin antibody anti-B2 microglobulin Antithrombin activity Protein C, S activity Fibrinogen levels (elevation is bad) PAI-1
Acquired antithrombin deficiency
Occurs in liver disease and nephrotic syndromes BUT can also occur w/ OCPs, prolonged heparin, asparaginase therapy, and DIC
**If patient gets a clot while on heparin, may need to think of this
One mechanism of action for clots in chronic inflammation
Increased C4bBP binds protein S, thereby lowering free protein S that cannot form with activated protein C and complex to break down Factor Va and VIIIa
Capecitabine
MoA: prodrug of 5-FU that is a pyrimidine antimetabolite that inhibits thimidylate synthetase which blocks the methylation of deoxyuridylic acid to thimidylic acid
