Miscellaneous 2 Flashcards
Del 5q- prognostic significance in MDS
low risk
Del 7q- prognostic significance in MDS
adverse
Trisomy 8 prognostic significance in MDS
Intermediate risk
del20q- prognostic significance in MDS
adverse
What is G4 CRS?
multiple pressors and/or positive pressure ventilation
Management of G4 CRS
Toci + solumedrol 1 g/day
(solumedrol instead of dex)
Management of severe CRS per boards
- toci + steroids concurrently (dex for G3, solumedrol for G4)
What does POEMS stand for
Polyneuropathy
Organomegaly
Endocrinopathy
Monoclonal (M) protein spike
Skin changes
Transplant criteria for AL amyloidosis
-Transplant eligible = 1) Age <70
* 2) SBP >90
- 3) NYHA <3
- 4) No more than 2 organs involved (liver, heart, kidney, or autonomic nerve)
- 5) Troponin T level <0.06 (high sensitivity troponin T <75 ng/ml)
- 6) NTproBNP <5 ng/L
- 7) ECOG 0-1
- 8) CrCl ?30 mL/minute
of cycles of induction for transplant eligible myeloma pts
4 (can give a couple more if they don’t have VGPR but answer choice will state 4)
Firstline for Castleman’s
Siltuximab
posaconazole issue w/ HMA+venetoclax
- can increase serum concentration of venetoclax through CYP3A4 inhibition so need to dose reduce venetoclax if using
Management of AML patient in CR1 after induction who’s transplant ineligible after induction
maintenance with PO azacitadine
Induction for AML with myelodysplasia-related changes
Induction:
Liposomal cytarabine and daunorubicin (vyxeos)
Brentuximab mechanism
Targets CD30
Management of T-Dxd with ILD - continue vs. hold
G1:
steroids
hold until resolved to G0
continuation:
If resolved in 28 days or less, maintain dose
If resolved in greater than 28 days, dose reduce
If >G2, permanent discontinuation
Grade I ILD w/ T-Dxd
Asymptomatic
Most common CHIP mutations
DNMT3A, TET2, ASXL1
What defines CCUS?
1) Idiopathic, clinically meaningful cytopenia
2) >2 percent variant allele frequency (VAF) of a leukemia-associated gene
3) No other evidence of a hematologic malignancy
Lowest and highest risk serologic status for CMV transmission in transplant
- highest = Donor+, recipient-
- lowest = Donor-, recipient-
Grading of NET’s
G1 = <3
intermediate = 3-20
high = >20
Translocation that can be seen in DLBCL
- t(14;18)
What is T4 disease in CRC
- invasion of visceral peritoneum or invasion/adherence to adjacent organs/structures
What is high risk for stage III CRC?
T4 or N2
What is N2 disease in CRC
4 or more lymph nodes
T3 in RCC
- extension into major veins or perinephric tissues
Most common SE of crizotinib
acneiform rash
HLADR15 - 1) seen in what conditions 2) predictive of what
- aplastic anemia + hypoplastic MDS + PNH
- predicts response to immunosuppressive treatment (ATG + cyclosprine)
What should increase in PTT after protein C concentrate make you think?
FVL
Components of managing factor 8 inhibitor
- suppress the antibody with rituxan or steroids
- treat underlying precipitant
Catheter associated VTE managemetn
- keep catheter in if still functional and anticoagulate
Adjuvant management of CRC patient w/ ESRD
5-Fu
T cell leukemia/lymphoma immunophenotype
CD2+, CD3+, CD4+, CD5+, CD7-, CD8-.
Smear finding with HTLV-1 -associated T cell leukemia/lymphoma
Flower cells
*see photo online
Mechanism of promacta in AA
TPO receptor expression in hematopoietic stem cells
luspatercept mechanism
Reduces aberrant Smad2/3 signaling by binding (“trapping”) transforming growth factor (TGF) beta superfamily ligands
What defines CHIP?
Patient gets a bone marrow biopsy and a clonal myeloid mutation is found on sequencing
*DNMT3A, TET2, ASXL1 most commonly
What defines ICUS?
- cytopenia (s) unexplained after extensive workup
- NO evidence of clonal hematopoiesis (if present, then it is CCUS)
Studies needed for hypereosinophilia workup
- PDGFR
- FGFR1
- TCR gene rearrangement
Ferroportin function + change in AICD
- iron transport protein
- decreased
sequence of correcting B12 and folate
- Replete B12 first (folate repletion with severe B12 deficiency can worsen B12 neurologic deficits)
When hydrea needs to be held in SCD per package instructions
- plt count <80k
- ANC <2000
- hgb <4.5
management of single bone lesion in langerhan’s
Biopsy/curretage then observe
management of multiple bone lesion in langerhan’s
Single agent cytarabine or cladribine
Management of CNS disease in Langerhan’s
IF mutant, BRAF targeted therapy
Preferred systemic therapy for Langerhan’s with multiorgan system involvement
- single agent cytarabine
- single agent cladribine
How many cycles of HiDAC in favorable risk AML
3-4
Second line for HCL w/ short PFS benefit from firstline (<2 years)
1) alternative purine analog + rituximab
2) vemurafenib +/- rituximab (classic HCL BRAF is uniformly positive)
3) Single agent rituximab
T-PLL immunophenotype
Strong CD52 expression
CD3+
Deauville score 5 vs 4
4= uptake moderately greater than liver (so less than 2x)
5 = at least 2x max SUV of liver
Ratio of PRBC to platelet to FFP recommended in massive transfusion
1:1:1
Folic acid is modified in a cell by means of which process
methylation
Venetoclax mechanism
BH3-mimetic
- blocks the anti-apoptotic B-cell lymphoma-2 (Bcl-2) protein, leading to programmed cell death of CLL cells.
HS managment
- IF chronic hemolysis and splenomegaly, splenectomy
- IF more benign course, transfusions and supportive care
Hemoglobin M disease inheritance
Autosomal dominant
Cyb5R deficiency inheritance
autosomal recessive
Basophilic stippling on smear
*see photo
granules that are dispersed through the cytoplasm of erythrocytes in a peripheral blood smear
basophilic stippling differential
- thalassemia
- hemolysis
- MDS
- alcoholism
- lead poisoning
PNH patient with liver failure - what is the cause?
Budd chiari from portal vein thrombosis
When you can use IVIG to treat acquired Von Willebrand
SLE, plasma cell dyscrasias, some lymphoproliferative disorders (*these cause acquired von willebrand from anti-VWF autoantibodies)
Next step for mild VWS deficiency diagnosis
- check blood type (patients with blood type O have 25-30% lower VWF antigen at baseline) to ensure not misdiagnosis