Miscellaneous 2 Flashcards by Ned Jones

Del 5q- prognostic significance in MDS

low risk

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Del 7q- prognostic significance in MDS

adverse

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Trisomy 8 prognostic significance in MDS

Intermediate risk

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del20q- prognostic significance in MDS

adverse

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What is G4 CRS?

multiple pressors and/or positive pressure ventilation

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Management of G4 CRS

Toci + solumedrol 1 g/day
(solumedrol instead of dex)

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Management of severe CRS per boards

toci + steroids concurrently (dex for G3, solumedrol for G4)

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What does POEMS stand for

Polyneuropathy
Organomegaly
Endocrinopathy
Monoclonal (M) protein spike
Skin changes

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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

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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)

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Firstline for Castleman’s

Siltuximab

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posaconazole issue w/ HMA+venetoclax

can increase serum concentration of venetoclax through CYP3A4 inhibition so need to dose reduce venetoclax if using

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Management of AML patient in CR1 after induction who’s transplant ineligible after induction

maintenance with PO azacitadine

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Induction for AML with myelodysplasia-related changes

Induction:
Liposomal cytarabine and daunorubicin (vyxeos)

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Brentuximab mechanism

Targets CD30

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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

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Grade I ILD w/ T-Dxd

Asymptomatic

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Most common CHIP mutations

DNMT3A, TET2, ASXL1

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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

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Lowest and highest risk serologic status for CMV transmission in transplant

highest = Donor+, recipient-
lowest = Donor-, recipient-

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Grading of NET’s

G1 = <3
intermediate = 3-20
high = >20

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Translocation that can be seen in DLBCL

t(14;18)

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What is T4 disease in CRC

invasion of visceral peritoneum or invasion/adherence to adjacent organs/structures

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What is high risk for stage III CRC?

T4 or N2

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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