Hematology/Oncology Flashcards
Causes of ITP
1) Idiopathic
2) HIV
3) SLE
4) CLL
5) Hep C
6) H. pyloir
Rule out HIV before starting treatment
Test used to measure acquired platelet dysfunction with a normal platelet count
Platelet Function Analyzer-100
A rapid screening test, has replaced bleeding time testing
Treatment for MDS to prevent progression to AML in -5q subtype
Lenalidomide
TKIs for treatment of CML (t9:22) can cause what EKG finding?
Prolonged QT, monitor with serial EKGs
Nonneoplastic causes of eosinophilia
CHINA C: Collagen vascular disease H: Helminthic infection I: Idiopathic N: Neoplasia (lymphoma, myeloproliferative) A: Allergy/Atopy/Asthma
Hypereosiniophilic syndrome
Moderate eosinophilia and end-organ damage commonly affecting: skin, lungs, GI, CV (restrictive CM )
R/o secondary causes of HES
What population can you not use a d-dimer in reliably to exclude VTE?
pregnant patients
Emergent reversal agent for vit K antagonists
Four-factor prothrombin complex concentrate (4f-PCC)
Contains II, VII, IX, and X as a lyophilized powder, quickly administered and effective >90% of the time
Plasma takes longer and is not indicated in major bleeding episodes 2/2 Vit K antagonists
Timeframe for delayed hemolytic transfusion reaction (DHTR)
7 to 14 days
Usually occurs as anamnestic antibody response to previous erythrocyte (non-ABO) antigen sensitization
Obtain DAT, LDH, Bili, and reticulocyte count to evaluate further
Cause of pure red cell aplasia in SCD?
Parvovirus B19
Can cause transient aplastic crisis
Triggers of G6PD
1) Chloroquine
2) Sulfonamides
3) Rasburicase
4) Dapsone
5) Phenazopyridine
Features of Waldenstrom macroglobulinemia
Indolent B-cell lymphoma
- elevated M spike (> 3 g/dL M protein)
- elevated gamma gap (>4)
- “B symptoms”
- peripheral neuopathy (antimyelin-associated glycoprotein)
- hyperviscosity symptoms (blurred vision, HA, vertigo)
- HSM, lymphadenopathy, anemia
- Send serum protein electrophoresis
- BMB with lymphoplasmacytic infiltrate (>10% clonal lymphoplasmacytic cells, differentiating it from MGUS)
Testing indicated after diagnosis of smoldering multiple myeloma
Whole-body MRI to look for lytic lesions
Smoldering MM has >10% plasma clonal cells in BM, >3 g/dL of M protein level, with no other MM-related signs or symptoms (normal Ca, renal function).
Reversal agent for dabigatran
Idarucizumab
Indicated for reversal for severe/life threatening bleeding, emergency surgery
Pre-operative hemoglobin goal for SCD patients (low to moderate risk surgery)
10 g/dL
Simple transfusion is equivalent to exchange transfusion pre-operatively, and is preferred given less risk and cost
Negative PERC, is d-dimer or imaging required?
No.
Do not order imaging or d-dimer
PERC criteria age <50 HR <100 O2 >95% on RA - No hemoptysis - No estrogen use - No previous DVT/PE - No unilateral leg swelling - No surgery or trauma requiring hospitalization within the last 4 weeks
Features of PNH
Membrane-bound protein loss leads to sensitivity to complement-mediated lysis (defective PIGA gene)
1) Pancytopenia
2) Intravascular hemolysis
3) Hemoglobinuria
4) Nonspecific abdominal pain
5) Thrombotic complications - Portal venous system (budd-chiari), sagittal veins in CNS
6) Increased risk of transformation to MDS or Acute Leukemia
Eval with flow cytometry (CD55/CD59)
ALL survivors (childhood) are at increased risk for what as adults?
Metabolic Syndrome
Folate vs. cobalamin deficiency
Folate - short lifespan (poorly stored), associated with diets low in green, leafy vegetables. Associate with alcoholism.
Features of cobalamin deficiency
Cobalamin - long lifespan (efficiently stored). Not usually associated with dietary inadequacy and NOT associated with alcoholism.
Pancytopenia, macrocytic erythrocytes, hypersegmented neutrophils
Can see intramedullary hemolysis
Features of MDS
Older patient with progressive fatigue, 2+ cytopenias (including macrocytic anema)
- Peripheral smear showing dysplastic hypogranular neutrophils and nucleated erythrocytes
Obtain bone marrow to diagnose
Treatment for atypical HUS
Eculizumab (monoclonal Ab against terminal component of complement cascade)
Features of atypical HUS:
- congenital syndrome 2/2 overwhelming complement activation
- not preceded by diarrheal illness
Features of cold agglutinin disease
Profound anemia
Intravascular and extravascular hemolysis
Positive direct antiglobulin (IgM) to C3 with agglutinated erythrovytes on PVS
Profoundly Elevated MCV
Associations
- waldenstrom
- MDS
- non-hodkin lymphoma
- MGUS
- mycoplasma, EBV
Tx
- keep warm (transfusions at body temp)
- rituximab
Warm vs cold hemolytic anemia
Warm
- RBCs agglutinate at body temp
- IgG
- Causes: Viral, connective tissue disease, CLL
- DAT positive IgG or C3
- Tx: steroids (first line), ritux/splenectomy
Cold
- RBCs agglutinate at room temp
- IgM
- Causes: EBV, Mycoplasma, CLL, lymphoma
- DAT positive C3, negative for IgG
- Tx: Keep warm, rituximab
- peripheral blood smear will show erythrocyte agglutination with marked elevation in MCV
Features of acquired hemophilia A
aPTT that does not correct with mixing
- low factor VIII levels
- Association with hematologic malignancies (CLL)
- Usually >60 y/o, women > men
Tx: recombinant activated factor VIII
- consider desmopressin for mild hemophilia A with bleeding or for minor procedures
Treatment for high risk prostate cancer
GNRH agonist and radiation therapy
High risk: Gleason of 8-10, extraprostratic extension
Treatment for locally advanced head and neck cancer
Radiation plus cisplatin or cetuximab
Avoid cisplatin if concern for renal toxicity
What marker does a seminoma NEVER produce?
AFP
If biopsy is c/w seminoma, but AFP elevated, consider the tumor to be nonseminomatous
What are the 4 major categories of lung cancer?
Central lesions: Squamous (hypercalcemia and cavitary) and small cell (aggressive, Lambert-Eaton, SIADH)
Peripheral: Adenocarcinoma and Large cell
Advanced ovarian cancer with germline BRCA1/BCRA2 mutation(s) can be treated with what new therapy?
Olaparib (adenosine diphosphate [ADP]-ribose polymerase (PARP) inhibitor)
PARP inhibition leads to ds-DNA breaks that are repaired in BRCA-compentent tumors using recombination, which is not possible in BRCA-mutated cancers, thus leading to cell death (synthetic lethality)
Postmenopausal LCIS treatment options for prevention of breast CA
Exemestane/Anastrozole if postmenopausal nd history of VTE
Tamoxifen or raloxifene (SERMs) can be used pre or postmenopausal
Adjuvant therapy for DCIS following lumpectomry and radiation in postmenopausal women
Anastrozole
Chemotherapy is not used in this population, as the risk for distant metastasis in DCIS is ~1%
Testing in nonsquamous metastatic NSCLC
Molecular alterations: EGFR, ALK, ROS1
EGFR benefits from erlotinib
ALK/ROS1 benefit from crizotinib
EGFR mutation is rarely seen in squamous cell carcinoma associated with a smoking history, do not test for this routinely
Treatment option for progressive, metastatic mismatch repair-deficient CRC (MLH1 aka Lynch Syndrome)
Immune checkpoint inhibitor (pembrolizumab)
Epidermal Growth Factor Receptor inhibitors (panitumumab or cetuximab) can be considered if patients do not have KRAS or RAS gene mutations
Genetic markers for CRC
Molecular analysis for KRAS, NRAS and BRAF genes
If metastatic disease limited to the liver: consider surgical resection (three or less lesions)
Limited role for hepatic arterial embolization, as CRC metastases are not vascular-dependent
Treatment for metastatic NSCLC
First line: platinum-based chemotherapy
Patients who respond should continue pemetrexed maintenance chemotherapy or switch to docetaxel maintenance chemotherapy. This assumes no driver mutations were found
Radiation does not have a curative/treatment role in metastatic disease aside from palliation
Treatment for early-stage laryngeal cancer
Radiation therapy alone
Early-stage laryngeal cancer is most appropriately treated with radiation alone because it is highly effective in that area, has low morbidity, and is associated with superior voice quality compared with patients who are treated with surgery.
Treatment for GIST
High-risk GISTs (high % of mitotic features, tumor size, stomach/proximal intestinal location) should be treated with surgery and oral imatinib for 3 years
GISTs often carry a c-KIT proto-oncogene activating mutation. Imatinib blocks c-KIT tyrosine kinase phosphorylation.
GISTs are not responsive to radiation therapy
Treatment for stage II and III NSCLC with negative margins
Adjuvant cisplatin-based chemotherapy
Treatment for hormone-receptor positive breast cancer in a young woman who is premenopausal after surgery, radiation and adjuvant chemotherapy
Ovarian suppression plus aromatase inhibitor
Shown to be superior to tamoxifen (which is used for low risk cancers in young, premenopausal women)
Treatment for ovarian cancer grades IC to IV
Surgery, platinum-based adjuvant chemotherapy
Maintenance intravenous and intraperitoneal chemotherapy is indicated if small (<1 cm) residual disease is present after initial round of adjuvant chemotherapy
Treatment for hairy cell leukemia
Cladribine or pentostatin
Flow cytometry will show positivity to CD20, CD11c, CD25, and CD103. Despite this, hairy cell leukemia does not respond to rituximab
Hairy “Clar-Ri”
“Clar” = claridibine
“Ri” = Ritximab
Follicular lymphoma can transform to what disease?
Agressive non-hodgkin lymphoma (DLBCL)
Look for change in disease pattern, newly elevated LDH, or new areas on PET
Obtain biopsy to rule out transformation
Recommendations for BRCA1/2 management with regard to BSO
BRCA1: BSO between 35-40 and after childbearing completed
BRCA2: BSO between 40 and 45 (risk of ovarian cancer before age 50 is <1%)
Diagnosis of CLL
Smudge cells on peripheral smear
Flow cytometry makes diagnosis, BMB not necessary
Treatment strategy for GE disease`
Neoadjuvant chemotherapy and radiation followed by surgical resection
Treatment for metastatic prostate cancer that is castrate-sensitive
Antiandrogen therapy: Leuprolide + flutamide/bicalutamide (androgen receptor blocker)
Followed by continuation of antiandrogen therapy with docetaxel
Complication of essential thrombocytosis
Acquired von Willebrand Disease
Treatment for recurrence-prevention in early-stage, HR positive, high-risk breast cancer in women who remain premenopausal after chemotherapy
Leuprolide (ovarian suppression) plus an aromatase inhibitor
Tests to stage colorectal cancer
CT C/A/P with contrast
CEA level
Do NOT pick PET/CT
